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24 The Child With Gastrointestinal Dysfunction

CAROLYN V. DAIGNEAU

http://evolve.elsevier.com/Wong/essentials/

Remember to check out your companion CD-ROM

RELATED TOPICS and ADDITIONAL RESOURCESIN TEXT

Collection of Specimens, Ch. 22

Cystic Fibrosis, Ch. 23

Genitourinary Tract Disorders/Defects, Ch. 27

Diaper Dermatitis, Ch. 30

Encopresis, Ch. 17

Family-Centered Care of the Child During Illness and Hospitalization, Ch. 21

Infection Control, Ch. 22

Intestinal Parasitic Diseases, Ch. 14

Nursing Care of the High-Risk Newborn and Family, Ch. 9

Ostomies, Ch. 22

Pain Assessment; Pain Management, Ch. 21

Preparation for Procedures, Ch. 22

Procedures Related to Alternative Feeding Techniques, Ch. 22

Procedures Related to Elimination, Ch. 22

Recommendations for Routine Immunizations: Hepatitis, Ch. 10

Shock, Ch. 25

Surgical Procedures, Ch. 22

Total Parenteral Nutrition, Ch. 22

CD COMPANION

Critical Thinking Exercises—Abdominal Pain; Constipation; Hematemesis; Home Care for the Adolescent; Ingestion of a Foreign Body

Case Studies—Appendicitis, Cleft Lip and Palate; Acute Diarrhea (Gastroenteritis); Dehydration and Diarrhea

Clinical Manifestations—Gastrointestinal Dysfunction in Children; Appendicitis; Hypertrophic Pyloric Stenosis

Nursing Care Plans—The Child With Appendicitis; The Infant With Esophageal Atresia and Tracheoesophageal Fistula; The Child With Acute Diarrhea (Gastroenteritis); The Child With Cleft Lip or Cleft Palate

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WONG'S CLINICAL MANUAL OF PEDIATRIC NURSING, 6/E

Nursing Care Plans—The Child With Hirschsprung Disease; The Child With Inflammatory Bowel Disease; The Child With Peptic Ulcer Disease; The Child With Acute Hepatitis; The Child With Esophageal Atresia and Tracheoesophageal Fistula; The Child With Hypertrophic Pyloric Stenosis; The Child With Intussusception; The Infant With an Anorectal Malformation; The Child With Celiac Disease

Community and Home Care Instructions—Caring for the Child With a Colostomy; Giving Gastrostomy Feedings; Caring for a Central Venous Catheter (for TPN)

LEARNING OBJECTIVESOn completion of this chapter the reader will be able to:

• Describe the characteristics of infants that affect their ability to adapt to fluid loss or gain.

• Formulate a plan of care for the infant with acute diarrhea.

• Compare and contrast the inflammatory diseases of the gastrointestinal tract.

• Describe the nursing care of the child with hepatitis.

• Formulate a plan for teaching parents preoperative and postoperative care of the child with a cleft lip or palate.

• Formulate a plan of care for the child with an obstructive disorder.

• Identify nutritional therapies for the child with a malabsorption syndrome.

GASTROINTESTINAL DYSFUNCTIONThe extensive surface area of the gastrointestinal (GI) tract and its digestive function represent the major means of exchange between the human organism and the environment. Inflammatory and malabsorptive disorders impair the functional integrity of the GI tract. In addition, the intestine of the infant is extremely vulnerable to infection. Acute infectious diarrhea causes significant alterations in fluid and electrolyte balance in both infants and children.

Numerous observations provide clues to specific GI problems (Box 24-1). In any disorder that involves GI losses of large amounts of fluid, dehydration poses a serious threat to life and demands immediate attention.

BOX 24-1 Clinical Manifestations of Gastrointestinal Dysfunction in Children

Failure to thrive—Weight consistently below the 3rd percentile or BMI (body mass index) below the 5th percentile or a decrease from established growth pattern.

Spitting up or regurgitation—Passive transfer of gastric contents into the esophagus or mouth

Vomiting—Forceful ejection of gastric contents; involves a complex process under central nervous system control that causes salivation, pallor, sweating, and tachycardia; usually accompanied by nausea

Projectile vomiting—Vomiting accompanied by vigorous peristaltic waves and typically associated with pyloric stenosis or pylorospasm

Nausea—Unpleasant sensation vaguely referred to the throat or abdomen with an inclination to vomit

Constipation—Delay or difficulty with the passage of stools that is present for two or more weeks; associated with symptoms that may include blood streaked stools and abdominal discomfort.

Encopresis—Involuntary overflow of incontinent stool causing soiling or incontinence secondary to fecal retention or impaction.

Diarrhea—Increase in the number of stools with an increased water content as a result of alterations of water and electrolyte transport by the gastrointestinal (GI) tract; may be acute or chronic

Hypoactive, hyperactive, or absent bowel sounds—Evidence of intestinal motility problems that may be caused by inflammation or obstruction

Abdominal distention—Protuberant contour of the abdomen that may be caused by delayed gastric emptying, accumulation of gas or stool, inflammation, or obstruction

Abdominal pain—Pain associated with the abdomen that may be localized or diffuse, acute or chronic; often caused by inflammation, obstruction, or hemorrhage

Gastrointestinal bleeding—May be from an upper or lower GI source and may be acute or chronic

Hematemesis—Vomiting of bright red blood or denatured blood that results from bleeding in the upper GI tract or from swallowed blood from the nose or oropharynx

Hematochezia—Passage of bright red blood per rectum, usually indicating lower GI tract bleeding

Melena—Passage of dark-colored, "tarry" stools resulting from denatured blood, suggesting upper GI tract bleeding or bleeding from the right colon

Jaundice—Yellow coloration of the skin and sclerae associated with liver dysfunction

Dysphagia—Difficulty swallowing caused by abnormalities in the neuromuscular function of the pharynx or upper esophageal sphincter or by disorders of the esophagus

Dysfunctional swallowing—Impaired swallowing caused by central nervous system defects or structural defects of the oral cavity, pharynx, or esophagus; can cause feeding problems or aspiration

Fever—Common manifestation of illness in children with GI disorders; usually associated with dehydration, infection, or inflammation

DEHYDRATIONDehydration is a common body fluid disturbance in infants and children and occurs whenever the total output of fluid exceeds the total intake, regardless of the cause. Dehydration may result from a number of diseases that cause insensible losses through the skin

and respiratory tract, through increased renal excretion, and through the GI tract. Although dehydration can result from lack of oral intake (especially in elevated environmental temperatures), more often it is a result of abnormal losses, such as those that occur in vomiting or diarrhea, when oral intake only partially compensates for the abnormal losses. Other significant causes of dehydration are diabetic ketoacidosis and extensive burns.

Water Balance in InfantsInfants and young children have a greater need for water and are more vulnerable to alterations in fluid and electrolyte balance. Compared with older children and adults, infants have a greater fluid intake and output relative to size. Water and electrolyte disturbances occur more frequently and more rapidly, and infants and children adjust less promptly to these alterations.

The fluid compartments in the infant vary significantly from those in the adult, primarily because of an expanded extracellular compartment. The extracellular fluid (ECF) compartment constitutes more than half the total body water at birth and has a greater relative content of extracellular sodium and chloride. The infant loses a large amount of fluid at birth and maintains a larger amount of ECF than the adult until about 2 years of age. This contributes to greater and more rapid water loss during this age period.

Fluid losses create compartment deficits that are reflected throughout the duration of dehydration. In general, approximately 60% of fluid is lost from the ECF, and the remaining 40% comes from the intracellular fluid (ICF). The amount of fluid lost from the ECF increases with acute illness and decreases with chronic loss.

Fluid losses vary with age and are divided into insensible, urinary, and fecal losses. Approximately two thirds of insensible losses occur through the skin; the remaining one third is lost through the respiratory tract. Heat and humidity, body temperature, and respiratory rate influence insensible fluid loss. Infants and children have a greater tendency to become highly febrile than do adults. Fever increases insensible water loss approximately 7 mL/kg/24 hr for each degree rise in temperature above 37.2° C (99° F). Fever and increased surface area relative

to volume are factors that contribute to greater insensible fluid losses in young patients.

Body Surface Area.The infant's relatively greater body surface area (BSA) allows larger quantities of fluid to be lost in insensible perspiration through the skin. It is estimated that the BSA of the premature neonate is five times as great, and that of the newborn is two to three times as great, as that of the older child or adult. The proportionately longer GI tract in infancy is another source of fluid loss, especially from diarrhea.

Basal Metabolic Rate.The rate of metabolism in infancy is significantly higher than in adulthood because of the larger BSA in relation to the mass of active tissue. Consequently, there is a greater production of metabolic wastes that must be excreted by the kidneys. Any condition that increases metabolism causes greater heat production, insensible fluid loss, and an increased need for water for excretion. The basal metabolic rate (BMR) in infants and children is higher to support growth.

Kidney Function.The kidneys of the infant are functionally immature at birth and are inefficient in excreting waste products of metabolism. Of particular importance for fluid balance is the inability of the infant's kidneys to concentrate or dilute urine, to conserve or excrete sodium, and to acidify urine. The infant is less able to handle large quantities of solute-free water than is the older child and infants are more likely to become dehydrated when given concentrated formulas or overhydrated when given excessive water or dilute formula.

Fluid Requirements.Infants ingest and excrete a greater amount of fluid per kilogram of body weight than do older children. Because electrolytes are excreted with water and the infant has limited ability for conservation, maintenance requirements include

both water and electrolytes. The daily exchange of ECF in the infant is greatly increased over that of older children, which leaves the infant little fluid volume reserve in dehydrated states. Fluid requirements depend on hydration status, size, environmental factors, and underlying disease (see Box 24-2 for Daily Maintenance Fluid Requirements).

BOX 24-2 Daily Maintenance Fluid Requirements

1. Calculate weight of child in kilograms:

2. Allow 100 mL/kg for first 10 kg.

3. Allow 50 mL/kg for second 10 kg.

4. Allow 20 mL/kg for remainder of weight in kilograms.

5. Divide total amount by 24 hours to obtain rate in milliliters per hour.

Types of DehydrationThe pathophysiology of dehydration is understood by recognizing that the distribution of water between the ECF and ICF spaces depends on active transport of potassium into and sodium out of cells by energy-requiring processes. Sodium is the chief solute in ECF and is the primary determinant of ECF volume. Potassium is primarily intracellular. When ECF volume is reduced in acute dehydration, the total body sodium content is almost always reduced as well, regardless of serum sodium measurements. Replacement of fluid volume should therefore be accompanied by sodium repletion. Sodium depletion in diarrhea occurs in two ways: out of the body in stool and into the ICF compartment to replace potassium to maintain electrical equilibrium.

Dehydration is classified into three categories on the basis of osmolality and depends primarily on the serum sodium concentration: (1) isotonic, (2) hypotonic, and (3) hypertonic (Fann, 1998; Ledwith, 1997).

Isotonic (isosmotic or isonatremic) dehydration, the primary form of dehydration in children, occurs in conditions in which electrolyte and water deficits are present in approximately balanced proportions. Water and salt are lost in approximately equal amounts. The observable fluid losses are not necessarily isotonic, because losses from other avenues make adjustments so that the sum of all losses, or the net loss, is isotonic. There is no osmotic force between the ICF and the ECF, so the major loss is sustained from the ECF compartment. This significantly reduces the plasma volume and the circulating blood volume, which affects the skin, muscles, and kidneys. Shock is the greatest threat to life, and the child with isotonic dehydration displays symptoms characteristic of hypovolemic shock. Plasma sodium remains within normal limits, between 130 and 150 mEq/L.

Hypotonic (hyposmotic or hyponatremic) dehydration occurs when the electrolyte deficit exceeds the water deficit, leaving the serum hypotonic. Because ICF is more concentrated than ECF in hypotonic dehydration, water moves from the ECF to the ICF to establish osmotic equilibrium. This movement further increases the ECF volume loss, and shock is a frequent finding. Because there is a greater proportional loss of ECF in hypotonic dehydration, the physical signs tend to be more severe with smaller fluid losses than with isotonic or hypertonic dehydration. Serum sodium concentration is less than 130 mEq/L.

Hypertonic (hyperosmotic or hypernatremic) dehydration results from water loss in excess of electrolyte loss and is usually caused by a proportionately larger loss of water or a larger intake of electrolytes. This type of dehydration is the most dangerous, and requires more specific fluid therapy. Hypertonic diarrhea may occur in infants who are given fluids by mouth that contain large amounts of solute, or in children who receive high-protein nasogastric tube feedings that place an excessive solute load on the kidneys. In hypertonic dehydration, fluid shifts from the lesser concentration of the ICF to the ECF. Plasma sodium concentration is greater than 150 mEq/L (Behrman, Kliegman, and Arvin, 2000).

Because the ECF volume is proportionately larger, hypertonic dehydration consists of a greater degree of water loss for the same intensity of physical signs. Shock is less apparent. However, neurologic disturbances, including alterations in

consciousness, poor ability to focus attention, lethargy, increased muscle tone with hyperreflexia, and hyperirritability to stimuli are more likely to occur. Cerebral changes are serious and may result in permanent damage.

Diagnostic EvaluationDiagnosis of the type and degree of dehydration is necessary to develop an effective plan of therapy. The degree of dehydration has been described as a percentage: 5% (mild), 10% (moderate), or 15% (severe). Water constitutes only 60% to 70% of the infant's weight. However, adipose tissue contains little water and is highly variable in individual infants and children. A more accurate means of describing dehydration is to reflect acute loss (over 48 hours or less) in milliliters per kilogram of body weight. For example, a loss of 50 mL/kg is considered to be a mild fluid loss, whereas a loss of 100 mL/kg produces severe dehydration. Weight is the most important determinant of the percent of total body fluid loss in infants and younger children. However, often the preillness weight is unknown. Other predictors of fluid loss include a changing level of consciousness (irritability to lethargy), response to stimuli, decreased skin elasticity and turgor, prolonged capillary refill, increased heart rate, and sunken eyes and fontanels. Clinical signs provide clues to the extent of dehydration (Table 24-1). Using multiple predictors increases the sensitivity of assessing the fluid deficit and early studies have shown a reasonable high degree of agreement between experienced observers in assessment of the level of dehydration. Objective signs of dehydration are present at a fluid deficit of less than 5%. Any two of the following signs—capillary refill of 2 seconds, absent tears, dry mucous membranes, and an ill general appearance—are predictors of a deficit of at least 5%. Generally, three or more clinical findings are present at a deficit of 5% to 9% and six or more findings are found with a deficit of 10% or more (Gorelick, Shaw, and Murphy, 1997). Shock, tachycardia, and very low blood pressure are common features of severe depletion of ECF volume (see Shock, Chapter 25).

TABLE 24-1 Evaluating Extent of Dehydration

Adapted from Jospe N, Forbes G: Fluids and electrolytes—clinical aspects, Pediatr Rev 17(11):395–403, 1996.

Therapeutic ManagementSee discussion on therapeutic management of diarrhea, p. 847.

Nursing ConsiderationsNursing observation and intervention are essential to the detection and therapeutic management of dehydration. A variety of circumstances cause fluid losses in infants, and changes can take place quickly. An important nursing responsibility is observation for signs of dehydration. Nursing assessment should begin with observation of general appearance and proceed to more specific observations. Conditions in which dehydration may develop quickly include diarrhea; vomiting; sweating; fever; disorders such as diabetes, renal disease, and cardiac anomalies; administration of certain drugs (such as diuretics and steroids); and trauma (major surgery, burns, and other extensive injury).

Intake and Output.Accurate measurements of fluid intake and output are vital to the assessment of dehydration. This includes oral and parenteral intake and losses from urine, stools, vomiting, fistulas, nasogastric suction, sweat, and wound drainage:

Urine—Frequency, color, consistency, and volume (when weighing diapers, approximately 1 g wet diaper weight equals 1 mL urine)

Stools—Frequency, volume, and consistency

Vomitus—Volume, frequency, and type

Sweating—Can be only estimated from frequency of clothing and linen changes

In addition to fluid intake and output, the following observations assist in assessment of dehydration:

Vital signs—Temperature (normal, elevated, or lowered depending on degree of dehydration), pulse (tachycardia), respirations (tachypnea), and blood pressure (hypotension)

Skin—Color, temperature, turgor, presence or absence of edema, and capillary refill

Mucous membranes—Moisture, color, and presence of and consistency of secretions

Body weight—Decreased in relation to degree of dehydration

Fontanel (infants)—Sunken, soft, or normal

Sensory alterations—Presence of thirst

For nursing interventions, see discussion under specific disorders.

DISORDERS OF MOTILITY

DIARRHEADiarrhea is a symptom that results from disorders involving digestive, absorptive, and secretory functions. Diarrhea is caused by abnormal intestinal water and electrolyte transport. Worldwide, there are an estimated 1.3 billion episodes of diarrhea. Approximately 24% of all deaths in children living in developing countries are related to diarrhea and dehydration. Most children

living in developed countries have mild forms of gastroenteritis. However, in the United States, approximately 220,000 children younger than age 5 are hospitalized and approximately 300 children younger than 5 years die of diarrhea and dehydration each year (Endsley and Galbraith, 1998).

Diarrheal disturbances involve the stomach and intestines (gastroenteritis), the small intestine (enteritis), the colon (colitis), or the colon and intestines (enterocolitis). Diarrhea is classified as acute or chronic.

Acute diarrhea, a leading cause of illness in children younger than 5 years of age, is defined as a sudden increase in frequency and a change in consistency of stools, often caused by an infectious agent in the GI tract. It may be associated with upper respiratory or urinary tract infections, antibiotic therapy, or laxative use. Acute diarrhea is usually self-limited (less than 14 days duration) and subsides without specific treatment if dehydration does not occur. Acute infectious diarrhea (infectious gastroenteritis) is caused by a variety of viral, bacterial, and parasitic pathogens (Table 24-2).

TABLE 24-2 Infectious Causes of Acute Diarrhea

TABLE 24-2 Infectious Causes of Acute Diarrhea—cont'd

TABLE 24-2 Infectious Causes of Acute Diarrhea—cont'd

Chronic diarrhea is defined as an increase in stool frequency and increased water content with a duration of more than 14 days. It is often caused by chronic conditions such as malabsorption syndromes, inflammatory bowel disease, immune deficiency, food allergy, lactose intolerance, or chronic nonspecific diarrhea, or as a result of inadequate management of acute diarrhea.

Intractable diarrhea of infancy is a syndrome that occurs in the first few months of life, persists for longer than 2 weeks with no recognized pathogens, and is refractory to treatment. The most common cause is acute infectious diarrhea that was not managed adequately.

Chronic nonspecific diarrhea (CNSD), also known as irritable colon or childhood and toddlers' diarrhea, is a common cause of chronic diarrhea in children 6 to 54 months of age. These children have loose stools, often with undigested food particles, and diarrhea greater than 2 weeks' duration. Children with CNSD grow normally and have no evidence of malnutrition, no blood in their

stool, and no enteric infection (Huffman, 1999). Dietary indiscretions and food sensitivities have been linked to chronic diarrhea. The excessive intake of juices and artificial sweeteners such as sorbitol, a substance found in many commercially prepared beverages and foods, may be a factor.

EtiologyMost pathogens that cause diarrhea are spread by the fecal-oral route through contaminated food or water or are spread from person to person where there is close contact (e.g., day care centers). Lack of clean water, crowding, poor hygiene, nutritional deficiency, and poor sanitation are major risk factors, especially for bacterial or parasitic pathogens. The increased frequency and severity of diarrheal disease in infants is also related to age-specific alterations in susceptibility to pathogens. For example, the immune system of infants has not been exposed to many pathogens and has not acquired protective antibodies. Worldwide infectious agents, viruses, bacteria, and parasites are the most common causes of acute gastroenteritis. In developed nations, viruses, primarily rotavirus, cause 70% to 80% of infectious diarrhea.

Rotavirus is the most important cause of serious gastroenteritis among children and a significant nosocomial (hospital acquired) pathogen. Rotavirus disease is most severe in children 3 to 24 months of age. Children younger than 3 months of age have some protection from the disease because of maternally acquired antibodies. Approximately 25% of severe cases of rotavirus occur in older children (Coffin, 2001).

Salmonella, Shigella, and Campylobacter organisms are the most frequently isolated bacterial pathogens. Salmonella has the highest occurrence in infants; Giardia and Shigella have the highest incidence among toddlers. Shigella infection is uncommon in the United States, accounting for less than 5% of diarrheal illnesses in infants and toddlers. Campylobacter has a bimodal presentation (highest in children less than 12 months with a second rise in incidence at 15 to 19 years). Giardia and Cryptosporidium organisms are parasites. Giardia represents 15% of nondysenteric illness in the United States; Cryptosporidium is often associated with outbreaks in young children in day care centers. C. difficile, Plesiomonas, or Yersinia

are also parasites that are frequently responsible for causing diarrhea that lasts more than 10 days in a previously healthy adolescent. Traveler's diarrhea is also more common in adolescents than in other age-groups (Ramaswamy and Jacobson, 2001). (See also Intestinal Parasitic Diseases, Chapter 14).

Antibiotic administration is frequently associated with diarrhea because antibiotics alter the normal intestinal flora resulting in an overgrowth of other bacteria such as Clostridium difficile. Antibiotic-associated diarrhea can also be caused by Salmonella, Clostridium porringers type A, and Staphylococcus aureus pathogens (Jabbar and Wright, 2003).

PathophysiologyInvasion of the GI tract by pathogens results in increased intestinal secretion as a result of enterotoxins, cytotoxic mediators, or decreased intestinal absorption secondary to intestinal damage or inflammation. Enteric pathogens attach to the mucosal cells and form a cuplike pedestal on which the bacteria rests. The pathogenesis of the diarrhea depends on whether the organism remains attached to the cell surface resulting in a secretory toxin (noninvasive, toxin producing, noninflammatory type) diarrhea, or penetrates the mucosa (systemic) diarrhea. Noninflammatory diarrhea is the most common diarrheal illness, resulting from the action of enterotoxin that is released after attachment to the mucosa (Ramaswamy and Jacobson, 2001). The most serious and immediate physiologic disturbances associated with severe diarrheal disease are (1) dehydration, (2) acid-base imbalance with acidosis, and (3) shock that occurs when dehydration progresses to the point that circulatory status is seriously impaired.

Diagnostic EvaluationEvaluation of the child with acute gastroenteritis begins with a careful history that seeks to discover the possible cause of diarrhea, to assess the severity of symptoms and the risk of complications, and to elicit information about current symptoms indicating other treatable illnesses that could be causing the diarrhea. The history should include questions about recent

travel, exposure to untreated drinking or washing water sources, contact with animals or birds, day care center attendance, recent treatment with antibiotics, or recent diet changes. History questions should also explore the presence or absence of other symptoms such as the presence of fever, vomiting, frequency, and character of stools (e.g., watery, bloody), urine output, dietary habits, and recent food intake (Burkhart, 1999).

Extensive laboratory evaluation is not indicated in children who have uncomplicated diarrhea and no evidence of dehydration because most diarrheal illnesses are self-limiting. Laboratory tests are indicated for children who are severely dehydrated and receiving intravenous therapy. Watery, explosive stools suggest glucose intolerance; foul-smelling, greasy, bulky stools suggest fat malabsorption. Diarrhea that develops after the introduction of cow's milk, fruits, or cereal may be related to enzyme deficiency or protein intolerance (Savilahti, 2000). Neutrophils or red blood cells in the stool indicate bacterial gastroenteritis or inflammatory bowel disease. The presence of eosinophils suggests protein intolerance or parasitic infection. There is debate about the benefit of obtaining stool cultures in children with domestically acquired gastroenteritis. Stool cultures should be performed when blood, mucus, or polymorphonuclear leukocytes are present in the stool; when symptoms are severe; when there is a history of travel to a developing country; and when there is suspicion of a specific pathogen. Gross blood or occult blood may indicate pathogens such as Shigella, Campylobacter, or hemorrhagic Escherichia coli strains. An enzyme-linked immunosorbent assay (ELISA) may be used to confirm the presence of rotavirus or Giardia. If there is a history of recent antibiotic use, the stool should be tested for C. difficile toxin. When bacterial and viral cultures are negative and when diarrhea persists for more than a few days, stools should be examined for ova and parasites. A stool specimen with a pH of less than 6 and the presence of reducing substances may indicate carbohydrate malabsorption or secondary lactase deficiency. Stool electrolyte measurements may help to identify children with secretory diarrhea.

Urine specific gravity should be determined if dehydration is suspected. A complete blood count (CBC), serum electrolytes, creatinine, and blood urea nitrogen (BUN) should be obtained in the child who requires hospitalization. The hemoglobin,

hematocrit, creatinine, and BUN levels are usually elevated in acute diarrhea and should normalize with rehydration.

Therapeutic ManagementThe major goals in the management of acute diarrhea include (1) assessment of fluid and electrolyte imbalance, (2) rehydration, (3) maintenance fluid therapy, and (4) reintroduction of an adequate diet. Infants and children with acute diarrhea and dehydration should be treated first with oral rehydration therapy (ORT). ORT is one of the major worldwide health care advances of the past decade. It is more effective, safer, less painful, and less costly than intravenous (IV) rehydration. The American Academy of Pediatrics, World Health Organization, and Centers for Disease Control and Prevention all recommend ORT as the treatment of choice for most cases of dehydration caused by diarrhea (Nappert and others, 2000; American Academy of Pediatrics, 1996; Gastanaduy and Begue, 1999). Oral rehydration solutions (ORS) enhance and promote the reabsorption of sodium and water, and studies indicate that these solutions greatly reduce vomiting, volume loss from diarrhea, and the duration of the illness. Oral replacement solutions are available in the United States as commercially prepared solutions and are successful in treating the majority of infants with isotonic, hypotonic, or hypertonic dehydration. Guidelines for rehydration recommended by the American Academy of Pediatrics are included in Box 24-3.

BOX 24-3 Model for RehydrationRehydration solution should consist of 75 to 90 mEq of sodium (Na+) per liter.

Give 40 to 50 mL/kg of rehydration solution over 4 hours.

Replacement and maintenance solution should consist of 40 to 60 mEq of Na+ per liter.

Reevaluate the need for further rehydration; initiate maintenance therapy using maintenance formulations, with daily volumes not to exceed 150 mL/kg/day.

In children with diarrhea without significant dehydration, the maintenance phase may be initiated without the need for rehydration solution (Acra and Ghishan, 1996).

If additional fluids are needed, use low-salt fluids such as breast milk or water.

Modified from American Academy of Pediatrics, Provisional Committee on Quality Improvement, Subcommittee on Acute Gastroenteritis: Practice parameter: the management of acute gastroenteritis in young children, Pediatrics 97(3):424–435, 1996.

After rehydration, ORS may be used during maintenance fluid therapy by alternating the solution with a low-sodium fluid such as water, breast milk, lactose-free formula, or half-strength lactose-containing formula. In older children ORS can be given and a regular diet continued. Ongoing stool losses should be replaced on a 1:1 basis with ORS. If the stool volume is not known, approximately 10 mL/kg (4 to 8 ounces) of ORS should be given for each diarrhea stool.

Solutions for oral hydration are useful in most cases of dehydration, and vomiting is not a contraindication. A child who is vomiting should be given an ORS at frequent intervals and in small amounts. In young children the fluid may be given with a spoon or small syringe in 5- to 10-mL increments every 1 to 5 minutes by the caregiver. An ORS may also be given via nasogastric or gastrostomy tube infusion. Infants without clinical signs of dehydration do not need ORT. They should, however, receive the same fluids recommended for infants with signs of dehydration in the maintenance phase and for ongoing stool losses.

! NURSING ALERTDiarrhea is not managed by encouraging intake of clear fluids by mouth, such as fruit juices, carbonated soft drinks, and gelatin. These fluids usually have a high carbohydrate content, a very low electrolyte content, and a high osmolality (Lasche and Duggan, 1999). Caffeinated soda is avoided, because caffeine is a mild diuretic and may lead to increased loss of water and

sodium. Chicken or beef broth is not given, because it contains excessive sodium and inadequate carbohydrate. A BRAT diet (bananas, rice, applesauce, and toast or tea) is contraindicated for the child and especially for the infant with acute diarrhea, because this diet has little nutritional value (low in energy and protein), is high in carbohydrates, and is low in electrolytes.

Early reintroduction of nutrients is desirable and is gaining more widespread acceptance. Continued feeding or early reintroduction of a normal diet has no adverse effects and actually lessens the severity and duration of the illness and improves weight gain when compared with the gradual reintroduction of foods (Lasche and Duggan, 1999). Infants who are breast-feeding should continue to do so, and ORS should be used to replace ongoing losses in these infants.

The use of nonhuman milk for infants and children with diarrhea remains controversial. Cow's milk and cow's milk formulas are of concern because poor digestion of lactose can occur in children with infectious diarrhea. However, some studies indicate that well-hydrated infants may resume full-strength nonhuman milk feeding immediately without adverse reactions (Hugger, Harkless, and Rentschler, 1998).

Many infants and children are safely managed with a diet containing cow's milk. Some practitioners advocate the use of a lactose-free formula only if milk or regular formula is not tolerated. In older children a regular diet can generally be offered after rehydration has been achieved. In toddlers there is no contraindication to continuing soft or pureed foods. A diet of easily digestible foods such as cereals, cooked vegetables, and meats is adequate for the older child.

In cases of severe dehydration and shock, IV fluids are initiated whenever the child is unable to ingest sufficient amounts of fluid and electrolytes to (1) meet ongoing daily physiologic losses, (2) replace previous deficits, and (3) replace ongoing abnormal losses. Patients who usually require IV fluids are those with severe dehydration, those with uncontrollable vomiting, those who are unable to drink for any reason (e.g., extreme fatigue, coma), and those with severe gastric distention.

The IV solution is selected on the basis of what is known regarding the probable type and cause of the dehydration—usually a saline solution containing 5% dextrose in water. Sodium bicarbonate may be added, because acidosis is usually associated with severe dehydration. Although the initial phase of fluid replacement is rapid in both isotonic and hypotonic dehydration, it is contraindicated in hypertonic dehydration because of the risk of water intoxication, especially in the brain cells.

After the severe effects of dehydration are under control, specific diagnostic and therapeutic measures are begun to detect and treat the cause of the diarrhea. Because of the self-limiting nature of vomiting and its tendency to improve when dehydration is corrected, the use of antiemetic agents is not recommended. The use of antibiotic therapy in children with acute gastroenteritis is controversial. Antibiotics may shorten the course of some diarrheal illnesses (e.g., those caused by Shigella). However, most bacterial diarrheas are self-limiting, and the diarrhea often resolves before the causative organism can be determined. Antibiotics may prolong the carrier period for bacteria such as Salmonella. Antibiotics may be considered, however, in patients with immunosuppression, severe symptoms or persistent disease, or patients who have had transplantation (Jabbar and Wright, 2003; Burkhart, 1999) (see Intestinal Parasitic Diseases, Chapter 14).

Nursing Considerations

AssessmentThe nursing assessment of diarrhea begins with observation of the infant or child's general appearance and behavior. Physical assessment includes all the parameters described for assessment of dehydration, such as decreased urine output; decreased weight; dry mucous membranes; poor skin turgor, sunken fontanel; and pale, cool, dry skin. With severe dehydration, increased pulse and respiration, decreased blood pressure, and a prolonged capillary refill time (>2 seconds) may indicate impending shock (see Table 24-1).

A history provides information about probable etiologic agents, such as introduction of a new food, exposure to infectious

agents, travel to an area of high susceptibility, contact with foods that might be contaminated, and contact with pets known to be sources of enteric infections. An allergic, drug, and dietary history may indicate food allergies, use of laxatives or antibiotics, or sources of excess sorbitol and fructose (e.g., apple juice).

Nursing DiagnosesSeveral nursing diagnoses are identified following a thorough physical assessment. The major diagnoses appropriate for the infant or child are described in the Nursing Care Plan on pp. 849–850. Other diagnoses may be evident depending on the age, condition, and etiology of the diarrhea.

NURSING CARE PLAN: The Child With Acute Diarrhea (Gastroenteritis)NURSING DIAGNOSIS: Fluid volume deficit related to excessive gastrointestinal (GI) losses in stool or emesis

PATIENT GOAL 1: Will exhibit signs of rehydration and maintain adequate hydration

NURSING INTERVENTIONS/RATIONALES

*Administer an oral rehydration solution (ORS) for both rehydration and replacement of stool losses

Give ORS frequently in small amounts, especially if child is vomiting, because vomiting, unless severe, is not a contraindication to using ORS

*Administer and monitor intravenous fluids as prescribed for severe dehydration and vomiting

*Administer antimicrobial agents as prescribed to treat specific pathogens causing excessive GI losses

Alternate ORS with a low-sodium fluid such as water, breast milk, or formula for maintenance fluid therapy (most authorities say formula should be lactose free only if infant is not tolerating formula)

After rehydration, offer child regular diet as tolerated because studies show that early reintroduction of normal diet is beneficial in reducing number of stools and weight loss and shortening duration of illness

Maintain a strict record of intake and output (urine, stool, and emesis) to evaluate effectiveness of interventions

Monitor urine specific gravity every 8 hours or as indicated to assess hydration

Weigh child daily to assess for dehydration

Assess vital signs, skin turgor, mucous membranes, and mental status every 4 hours or as indicated to assess hydration

Discourage intake of clear fluids such as fruit juices, carbonated soft drinks, and gelatin because these fluids usually are high in carbohydrates and low in electrolytes, and have a high osmolality

Instruct family in providing appropriate therapy, monitoring intake and output, and assessing for signs of dehydration to ensure optimum results and improve compliance with the therapeutic regimen

EXPECTED OUTCOME

Child exhibits signs of adequate hydration (specify)

NURSING DIAGNOSIS: Altered nutrition: less than body requirements related to diarrheal losses, inadequate intake

PATIENT GOAL 1: Will consume nourishment adequate to maintain appropriate weight for age


After rehydration, instruct breast-feeding mother to continue feeding breast milk because this tends to reduce severity and duration of illness

Avoid giving BRAT diet (bananas, rice, applesauce, and toast or tea) because this diet is low in energy and protein, too high in carbohydrates, and low in electrolytes

Observe and record response to feedings to assess feeding tolerance

Instruct family in providing appropriate diet to gain compliance with therapeutic regimen

Explore concerns and priorities of family members to improve compliance with therapeutic regimen

EXPECTED OUTCOME

Child takes prescribed nourishment and exhibits a satisfactory weight gain

NURSING DIAGNOSIS: Risk for transmitting infection related to microorganisms invading GI tract

PATIENT (OTHERS) GOAL 1: Will not exhibit signs of GI infection


Implement standard precautions or other hospital infection control practices, including appropriate disposal of stool and laundry and appropriate handling of specimens, to reduce risk of spreading infection

Maintain careful handwashing to reduce risk of spreading infection

Apply diaper snugly to reduce likelihood of fecal spread

Use superabsorbent disposable diapers to contain feces and decrease chance of diaper dermatitis

Attempt to keep infants and small children from placing hands and objects in contaminated areas

Teach children, when possible, protective measures to prevent spread of infection, such as handwashing after using toilet

Instruct family members and visitors in isolation practices, especially handwashing, to reduce risk of spreading infection

EXPECTED OUTCOME

Infection does not spread to others

NURSING DIAGNOSIS: Impaired skin integrity related to irritation caused by frequent, loose stools

PATIENT GOAL 1: Skin will remain intact


Change diaper frequently to keep skin clean and dry

Cleanse buttocks gently with bland, nonalkaline soap and water or immerse child in a bath for gentle cleansing because diarrheal stools are highly irritating to skin

Apply ointment such as zinc oxide to protect skin from irritation (type of ointment may vary for each child and may require a trial period)

Expose slightly reddened intact skin to air whenever possible to promote healing; apply protective ointment to very irritated or excoriated skin to facilitate healing

Avoid using commercial baby wipes containing alcohol on excoriated skin because they will cause stinging

Observe buttocks and perineum for infection, such as Candida, so that appropriate therapy can be initiated

*Apply appropriate antifungal medication to treat fungal infection of skin

EXPECTED OUTCOME

Child has no evidence of skin breakdown

NURSING DIAGNOSIS: Anxiety/fear related to separation from parents, unfamiliar environment, distressing procedures

PATIENT GOAL 1: Will exhibit signs of comfort


Provide mouth care and pacifier for infants to provide comfort

Encourage family visitation and participation in care as much as the family is able to prevent stress associated with separation

Touch, hold, and talk to child as much as possible to provide comfort and relieve stress

Provide sensory stimulation and diversion appropriate for child's developmental level and condition to promote optimum growth and development

EXPECTED OUTCOMES

Child exhibits minimal signs of physical or emotional distress

Family participates in child's care as much as possible

NURSING DIAGNOSIS: Altered family processes related to situational crisis, knowledge deficit

PATIENT (FAMILY) GOAL 1: Family will understand about child's illness and its treatment and will be able to provide care


Provide information to family about child's illness and therapeutic measures to encourage compliance with therapeutic regimen, especially at home

Assist family in providing comfort and support to child

Permit family members to participate in child's care as much as they desire to meet needs of both child and family

Instruct family regarding precautions to prevent spread of infection

Arrange for posthospitalization health care for continued assessment and treatment

Refer family to a community health care agency for supervision of home care as needed

EXPECTED OUTCOME

Family demonstrates ability to care for child, especially at home

* Dependent nursing action.

PlanningThe goals for the dehydrated infant or child and for the family are as follows:

1. Infant or child will maintain adequate hydration.

2. Infant or child will maintain appropriate nutrition for age.

3. Infant or child will not spread infection (if etiologic agent) to others.

4. Family will receive appropriate support and education, especially regarding home care.

ImplementationThe management of most cases of acute diarrhea takes place in the home with education of the caregiver. Caregivers are taught to monitor for signs of dehydration (especially the number of wet diapers or voidings) and the amount of fluids taken by mouth, and to assess the frequency and amount of stool losses. Education relating to ORT, including the administration of maintenance fluids and replacement of ongoing losses, is important (see Critical Thinking Exercise). ORS should be administered in small quantities at frequent intervals. Vomiting is not a contraindication to ORT unless it is severe. Information concerning the introduction of a normal diet is essential. Parents need to know that a slightly higher stool output initially occurs with continuation of a normal diet and with ongoing replacement of stool losses. The benefits of a better nutritional outcome with fewer complications and a shorter duration of illness outweigh the potential increase in

stool frequency. Parents' concerns should be addressed to ensure adherence to the treatment plan.

CRITICAL THINKING EXERCISE: DiarrheaA mother brings her 8-month-old infant, Mary, to the primary care clinic. The mother reports that Mary has had a "cold" for about 2 days, and this morning she began to vomit and has had diarrhea for the past 8 hours. The mother states that Mary is still breast-feeding, but that she is not taking as much fluid as usual, and she is having three times as many stools as usual (the stools are watery in consistency). When the nurse practitioner examines Mary, she notes that her temperature is 100.4° F, her pulse and blood pressure are in the normal range, her mucous membranes are moist, and that she has tears when she cries. The nurse practitioner also notes that Mary's weight has not changed from what it was when she was seen in the clinic 2 weeks ago for her well-child visit. What interventions should the nurse practitioner include in her initial management of Mary?

QUESTIONS1. Evidence—Is there sufficient evidence for the nurse practitioner to draw any conclusions for her initial plan of management?

2. Assumptions—Describe some underlying assumptions about the following:

a. Clinical manifestations of various levels of dehydration

b. Management of acute diarrhea

c. Breast-feeding and the management of acute diarrhea

d. Use of antidiarrheal medications for acute diarrhea

3. What nursing interventions should the nurse practitioner implement at this time?

4. Does the evidence support the nurse practitioner's conclusion?

5. Are there any alternative perspectives that the nurse practitioner should consider?

ANSWERS1. Yes, there are sufficient data for the nurse practitioner to arrive at some conclusions.

2. a. See Table 24-1: Evaluating Extent of Dehydration, and note the criteria for mild dehydration.

b. Infants/children with mild dehydration are managed with oral rehydration therapy (ORS) and early reintroduction of an adequate diet. In cases of severe dehydration, or when infants and children have uncontrollable vomiting, intravenous fluids are used in the management of acute diarrhea.

c. Breast feeding generally can be continued in mild dehydration.

d. Antidiarrheal medications are not recommended for the treatment of acute infectious diarrhea. These medications have adverse effects such as slowed motility and can prolong the illness.

3. At the present time, Mary meets all the criteria for mild dehydration. It is highly probable that she has acute infectious diarrhea because her mother noted that she has had a "cold" for several days, she is vomiting, having diarrhea, and has an elevated temperature. The priority for nursing care at this time is to provide rehydration via ORS. ORS is an effective, safe, and cost-effective way to treat mild dehydration. The nurse practitioner should provide the mother with instructions to give Mary ORS at frequent intervals and in small amounts. The mother should also be instructed to continue with breast-feeding and normal feedings. Early reintroduction of normal nutrients is desirable in cases of mild dehydration; delayed introduction of food may be harmful and can prolong the

illness. Mary's mother should also be told to avoid the use of antidiarrheal medications.

4. Yes, the evidence supports this initial plan of management.

5. Mary's mother should be instructed to continue to monitor Mary for signs of improvement (an increase in the number of voidings or the number of wet diapers, and a decrease in vomiting). However, if Mary's condition does not improve, Mary's mother should be instructed to bring Mary back to the clinic or to the local emergency department. Mary's mother should be told to use frequent handwashing when caring for Mary to avoid transferring this infection to other members of the family.

If the child with acute diarrhea and dehydration is hospitalized, an accurate weight must be obtained, as well as careful monitoring of intake and output. The child may be placed on parenteral fluid therapy with nothing by mouth (NPO) for 12 to 48 hours. Monitoring the IV infusion is an important nursing function. The nurse must ensure that the correct fluid and electrolyte concentration is infused, that the flow rate is adjusted to deliver the desired volume in a given time, and that the IV site is maintained.

Accurate measurement of output is essential to determine if renal blood flow is sufficient to permit the addition of potassium to the IV fluids. The nurse is responsible for examination of stools and collection of specimens for laboratory examination (see Collection of Specimens, Chapter 22). Care should be taken when obtaining and transporting stools to prevent possible spread of infection. A clean tongue depressor can be used to obtain specimens for laboratory examination or as an applicator for transfer to a culture medium. Stool specimens should be transported to the laboratory in appropriate containers and media according to hospital policy.

Diarrheal stools are highly irritating to the skin, and extra care is needed to protect the skin of the diaper region from excoriation (see Diaper Dermatitis, Chapter 30). Rectal temperatures are avoided because they stimulate the bowel, increasing passage of stool.

Support for the child and family involves the same care and consideration given all hospitalized children (see Chapter 21). Parents are kept informed of the child's progress and instructed in the use of frequent and proper handwashing and the disposal of soiled diapers, clothes, and bed linen. Everyone caring for the child must be aware of "clean" areas and "dirty" areas, especially in the hospital, where the sink in the child's room is used for many purposes. Soiled diapers and linen should be discarded in receptacles close to the bedside. To remind caregivers to keep diapers and other soiled articles away from clean areas, place signs identifying "clean" (e.g., bed table) and "dirty" (e.g., sink, bathroom) areas. List the articles that may be stored in each area on these signs.

Prevention.The best intervention for diarrhea is prevention. The fecal-oral route spreads most infections, and parents need information about preventive measures such as personal hygiene, protecting the water supply from contamination, and careful food preparation.

! NURSING ALERTTo reduce the risk of bacteria transmitted via food, encourage parents to:

Quickly freeze or refrigerate all ground meat and other perishable foods.

Never thaw food on the counter or let it sit out of the refrigerator for more than 2 hours.

Wash hands, utensils, and work areas with hot, soapy water after contact with raw meat to keep bacteria from spreading.

Check ground meat with a fork to make sure no pink is showing before taking a bite.

Cook all dishes made with ground meat until brown or gray inside or to an internal temperature of 71° C (160° F).

Meticulous attention to perianal hygiene, disposal of soiled diapers, proper handwashing, and isolation of infected persons also minimizes the transmission of infection (see Infection Control, Chapter 22).

Parents need information about preventing diarrhea while traveling. They are cautioned against giving their children adult medications that are used to prevent traveler's diarrhea. Until vaccines or other prophylactic measures are proved to be safe for children, the best measure during travel to areas where water may be contaminated is to allow children to drink only bottled water and carbonated beverages (from the container through a straw supplied from home). Tap water, ice, unpasteurized dairy products, raw vegetables, unpeeled fruits, meats, and seafood should also be avoided.

EvaluationThe effectiveness of nursing interventions is determined by continued reassessment according to the following observational guidelines:

1. Monitor fluid losses with careful intake and output measurements and daily weights.

2. Monitor food intake, especially calories.

3. Observe for evidence of complications from underlying disease (specify) or therapy.

4. Observe and interview family to determine extent and effectiveness of care.

The expected outcomes are described in the Nursing Care Plan on pp. 849–850.

CONSTIPATIONConstipation is an alteration in the frequency, consistency, or ease of passing stool. Parents often define constipation as 3 or more days without the passage of stool (Castiglia, 2001). It may also be defined as painful bowel movements, which are often blood streaked, or include the retention of stool, with or without soiling,

even with a stool frequency of more than three stools per week (Loening-Baucke, 1995). The frequency of bowel movements, however, is not considered a diagnostic criterion because it varies widely among children. Having extremely long intervals between defecation is termed obstipation. Constipation with fecal soiling is referred to as encopresis.

Constipation may arise secondary to a variety of organic disorders or in association with a wide range of systemic disorders. Structural disorders of the intestine, such as strictures, ectopic anus, and Hirschsprung disease, may be associated with constipation. Systemic disorders associated with constipation include hypothyroidism, hypercalcemia resulting from hyperparathyroidism or vitamin D excess, and chronic lead poisoning. Constipation may be associated with drugs such as antacids, diuretics, antiepileptics, antihistamines, opioids, and iron supplementation. Spinal cord lesions may be associated with loss of rectal tone and sensation. Affected children are prone to chronic fecal retention and overflow incontinence.

The majority of children have idiopathic or functional constipation, because no underlying cause can be identified. Chronic constipation may occur as result of environmental, psychosocial factors, or a combination of both. Transient illness, withholding and avoidance secondary to painful or negative experiences with stooling, and dietary intake with decreased fluid and fiber all play a role in the etiology of constipation.

Newborn PeriodNormally the newborn infant passes a first meconium stool within 24 to 36 hours of birth. Any infant who does not do so should be assessed for evidence of intestinal atresia or stenosis, Hirschsprung disease (congenital aganglionic megacolon), hypothyroidism, meconium plugs, or meconium ileus. Meconium plugs are caused by meconium that has reduced water content and are usually evacuated after digital examination but may require irrigations with a hypertonic solution or contrast medium.

Meconium ileus, the initial manifestation of cystic fibrosis, is the luminal obstruction of the distal small intestine by abnormal meconium. Treatment is the same as for a meconium plug; early

surgical intervention may be needed to evacuate the small intestine.

InfancyThe onset of constipation frequently occurs during infancy, and may result from organic causes such as Hirschsprung disease, hypothyroidism, and strictures. It is important to differentiate these conditions from functional constipation. Constipation in infancy is often related to dietary practices. It is less common in breast-fed infants, who have softer stools than bottle-fed infants. Breast-fed infants may also have decreased stools because of more complete use of breast milk with little residue. When constipation occurs with a change from human milk or modified cow's milk to whole cow's milk, simple measures such as adding or increasing the amount of cereal, vegetables, and fruit in the diet of the infant usually corrects the problem. When a bottle-fed infant passes a hard stool that results in an anal fissure, stool-withholding behaviors may develop in response to pain on defecation (see Critical Thinking Exercise).

CRITICAL THINKING EXERCISE: ConstipationHarry, an 8 month-old infant, is seen by the pediatric nurse practitioner for his well-child visit. Harry's mother states that he usually has one hard stool every 4 to 5 days, which causes discomfort when the stool is passed. He has also had one episode of diarrhea and two episodes of ribbonlike stools. Abdominal distention and vomiting have not accompanied the constipation, and Harry's growth has been normal. Currently, his diet consists of cow's milk formula only. Harry's mother reports that the infrequent passage of hard stools began approximately 6 weeks ago when she stopped breast-feeding. Which interventions should the nurse practitioner include in the initial management of Harry's problem?

QUESTIONS1. Evidence—Is there sufficient evidence for the nurse practitioner to draw any conclusions about the management of Harry's problem?


a. Causes of constipation in infants

b. Factors associated with functional constipation in infants

c. Management of functional constipation in infants

3. What interventions should the nurse practitioner implement at this time?

4. Does the evidence support these interventions?

5. Are there alternative perspectives that the nurse practitioner should consider? What are they?

ANSWERS1. Yes, there are sufficient data to arrive at some conclusions for an initial plan of management.

2. a. Constipation in infancy can be caused by medical conditions such as Hirschsprung disease, hypothyroidism or strictures, or it can be simple functional constipation.

b. In infancy, changes in dietary practices such as a change from human milk to cow's milk may precipitate functional constipation.

c. Functional constipation is usually treated by dietary modifications such as increasing the amount of carbohydrate, fruit or vegetables in the infant's diet.

3. Initially, the nurse practitioner can tell Harry's mother that functional constipation may occur with changes in the diet (e.g., the change from breast-feeding 6 weeks ago to bottle feeding of cow's milk). The nurse practitioner can recommend that Harry's mother slowly introduce cereal and prune juice into Harry's diet. Cereal and one or two offerings of fruit juice each day may help to prevent further constipation. Often, simple measures such as the introduction of solid foods or

other dietary modifications help to remedy functional constipation.

4. The initial data seem to point to the conclusion that Harry has functional constipation. However, the one episode of diarrhea and the two episodes of passage of ribbonlike stools do not usually occur with functional constipation

5. The nurse practitioner should remember that constipation can be caused by medical conditions such as Hirschsprung disease; therefore, a referral to a gastroenterologist for further evaluation is also warranted at this time.

ChildhoodMost constipation in early childhood is due to environmental changes or normal development when a child begins to attain control over bodily functions. A child who has experienced discomfort during bowel movements may deliberately try to withhold stool. Over time, the rectum accommodates to the accumulation of stool, and the urge to defecate passes. When the bowel contents are ultimately evacuated, the accumulated feces are passed with pain, thus reinforcing the desire to withhold stool.

Constipation in school-age children may represent an ongoing problem or a first time event. The onset of constipation at this age is often the result of environmental changes, stresses and changes in toileting patterns. A common cause of new-onset constipation at school entry is fear of using the school bathrooms, which are noted for their lack of privacy. Early and hurried departure for school immediately after breakfast may also impede bathroom use.

The management of simple constipation consists of a plan to promote regular bowel movements. Often this is as simple as changing the diet to provide more fiber and fluids, eliminating foods known to be constipating, and establishing a bowel routine that allows for regular passage of stool. Stool-softening agents such as docusate or lactulose may also be helpful. If other symptoms such as vomiting, abdominal distention, or pain and evidence of growth failure are associated with the constipation, the condition should be investigated further.


Constipation tends to be self-perpetuating. A child who has difficulty or discomfort when attempting to evacuate the bowels has a tendency to retain the bowel contents and this may initiate a vicious circle. Nursing assessment begins with an accurate history of bowel habits, diet, events associated with the onset of constipation, drugs or other substances that the child may be taking, and the consistency, color, frequency, and other characteristics of the stool. If there is no evidence of a pathologic condition, the major task is to educate the parents regarding normal stool patterns and to participate in the education and treatment of the child.

Dietary modifications are essential in preventing constipation. During infancy, simply increasing the carbohydrate (sugar or corn syrup) in the infant's formula will often relieve the problem. During childhood the diet should contain increased amounts of fiber and fluid. Parents benefit from guidance in selecting foods that facilitate bowel movements (Box 24-4). They need reassurance concerning the benign nature of the condition. It is also important to discuss their attitudes and expectations regarding toilet habits.

BOX 24-4 High-Fiber FoodsBREAD, GRAINS

Whole-grain bread or rolls

Whole-grain cereals

Bran

Pancakes, waffles, and muffins with fruit or bran

Unrefined (brown) rice

VEGETABLES

Raw vegetables, especially broccoli, cabbage, carrots, cauliflower, celery, lettuce, and spinach

Cooked vegetables, such as those listed above, and asparagus, beans, Brussels sprouts, corn, potatoes, rhubarb, squash, string beans, and turnips

FRUITS

Raw fruits, especially those with skins or seeds, other than ripe banana or avocado

Raisins, prunes, or other dried fruits

MISCELLANEOUS

Nuts, seeds, legumes, popcorn

High-fiber snack bars

When constipation persists despite dietary intervention, more aggressive management may be necessary. It is important to differentiate an acute episode of constipation from chronic functional constipation, which can result from chronic stool withholding behavior. As the rectal vault becomes distended over time, further complications such as fecal impaction and encopresis may develop (see Chapter 17).

HIRSCHSPRUNG DISEASEHirschsprung disease (congenital aganglionic megacolon) is a mechanical obstruction caused by inadequate motility of part of the intestine. It accounts for about one fourth of all cases of neonatal obstruction, although it may not be diagnosed until later in infancy or childhood. The incidence is 1 in 5000 live births. It is four times more common in males than in females, and may follow a familial pattern in a small number of cases. Hirschsprung disease is usually an isolated birth defect but it has been associated with other syndromes including Down syndrome. Depending on its presentation, it may be an acute, life-threatening, or chronic condition (DiLorenzo, 2001).

PathophysiologyThe term congenital aganglionic megacolon describes the primary defect, which is the absence of ganglion cells in one or more segments of the colon. In Hirschsprung disease, there is an abnormal migration of the precursor ganglion cells that derive from the neural crest in the developing brain. The result is an impaired colonization of ganglion cells in the distal portion of the GI tract resulting in aganglionosis. In about 75% of cases the disease is

limited to the rectosigmoid area with the aganglionic segment beginning at the internal anal sphincter and extending proximally blending into the normal colon. Lack of enervation produces the functional defect that results in absence of propulsive movements (peristalsis). Stool accumulates with distention of the bowel proximal to the defect (megacolon). The internal sphincter fails to relax because the ganglion segment is missing the inhibitory neurotransmitter, nitric oxide. The result is an obstruction because the evacuation of stool, gas and liquids are prevented (Fig. 24-1). Intestinal distention and ischemia may also occur as a result of distention of the bowel wall, which contributes to the development of enterocolitis (inflammation of the small bowel and colon). Enterocolitis is the leading cause of death in children with Hirschsprung disease (DiLorenzo, 2001).FIG. 24-1 Hirschsprung disease.

Diagnostic Evaluation

Most children with Hirschsprung disease are diagnosed in the first few months of life. Clinical manifestations vary according to the age when symptoms are recognized and the presence of complications, such as enterocolitis (Box 24-5). In infants the findings include a distended abdomen, a contracted anal sphincter, and small-caliber empty rectum. In older children, a careful history is helpful. Radiographs, an unprepped barium enema, and anorectal manometric examinations assist in the differential diagnosis, which is confirmed by a full-thickness rectal biopsy demonstrating the absence of ganglion cells in the myenteric and submucosal plexus.

BOX 24-5 Clinical Manifestations of Hirschsprung DiseaseNEWBORN PERIOD

Failure to pass meconium within 24 to 48 hours after birth

Refusal to feed

Bilious vomiting

Abdominal distention

INFANCY

Failure to thrive

Constipation


Episodes of diarrhea and vomiting

Signs of enterocolitis

Explosive, watery diarrhea

Fever

Appears significantly ill

CHILDHOOD (Symptoms appear more chronic)

Constipation

Ribbonlike, foul-smelling stools


Visible peristalsis

Easily palpable fecal mass

Undernourished anemic appearance

Therapeutic ManagementTreatment is primarily surgical to remove the aganglionic portion of the bowel to relieve obstruction and restore normal bowel motility and function of the internal anal sphincter. If the bowel is not significantly distended this is accomplished in one surgery. However, in most cases two stages are required. First, a temporary ostomy is created proximal to the aganglionic segment to relieve obstruction and allow the normally enervated and dilated bowel to return to its normal size. Second, complete corrective surgery is performed usually when the child weighs approximately 9 kg (20 pounds). The various surgical procedures that can be performed are the Swenson, Duhamel, Boley, and Soave procedures. The Soave endorectal pull-through procedure, one of the most frequently used procedures, consists of pulling the end of the normal bowel through the muscular sleeve of the rectum, from which the aganglionic mucosa has been removed. The ostomy is usually closed at the time of the pull-through procedure.

Prognosis.Most children with Hirschsprung disease require surgery rather than medical therapy. Once the patient is stabilized with fluid and electrolyte replacement, if needed, the temporary colostomy is performed and has a high rate of success. After the later pull-through procedure, anal stricture and incontinence are potential complications that may occur, requiring further therapy, including dilation or bowel-retraining therapy.

Nursing ConsiderationsThe nursing concerns depend on the child's age and the type of treatment. If the disorder is diagnosed during the neonatal period,

the main objectives are (1) to help the parents adjust to a congenital defect in their child, (2) to foster infant-parent bonding, (3) to prepare them for the medical/surgical intervention, and (4) to assist them in colostomy care after discharge.

Preoperative Care.The child's preoperative care depends on the age and clinical condition. A child who is malnourished may not be able to withstand surgery until the physical status improves. Often this involves symptomatic treatment with enemas; a low-fiber, high-calorie, and high-protein diet, and in severe situations the use of total parenteral nutrition (TPN).

Physical preoperative preparation includes the same measures that are common to any surgery (see Surgical Procedures, Chapter 22). In the newborn, whose bowel is sterile, no additional preparation is necessary. However, in other children, preparation for the pull-through procedure involves emptying the bowel with repeated saline enemas and decreasing bacterial flora with systemic antibiotics and colonic irrigations using antibiotic solution. Oral antibiotics may also be prescribed.

Enterocolitis is the most serious complication of Hirschsprung disease. Emergency preoperative care includes frequent monitoring of vital signs and blood pressure for signs of shock; monitoring fluid and electrolyte replacements, as well as plasma or other blood derivatives; and observing for symptoms of bowel perforation, such as fever, increasing abdominal distention, vomiting, increased tenderness, irritability, dyspnea, and cyanosis.

Because progressive distention of the abdomen is a serious sign, the nurse measures abdominal circumference with a paper tape measure, usually at the level of the umbilicus or at the widest part of the abdomen. The point of measurement is marked with a pen to ensure reliability of subsequent measurements. Abdominal measurement can be obtained with the vital sign measurements and is recorded in serial order so that any change is obvious. To reduce stress to the acutely ill child when frequent measurements of abdominal circumference are needed, the tape measure can be left in place beneath the child rather than removed each time.

The child's age dictates the type and extent of psychologic preparation. Because a colostomy is usually performed, the child who is of at least preschool age is told about the procedure in concrete terms, with the use of visual aids (see Chapter 22). It is important to time explanations appropriately to prevent the anxiety and confusion that could result from too much information. It is also important to stress to parents and older children that the colostomy for Hirschsprung disease is temporary, unless so much bowel is involved that a permanent ileostomy must be performed. In most instances the extent of bowel resection is known before surgery, although the nurse should be aware of those instances when doubt exists concerning repair. The nurse should remember that although a temporary colostomy is favorable in terms of future health and adjustment, it requires additional surgery, which may be very stressful to parents and children.

Postoperative Care.Postoperative care is the same as that for any child or infant with abdominal surgery (see Surgical Procedures, Chapter 22). When a colostomy is part of the corrective procedure, stomal care is a major nursing task (see Ostomies, Chapter 22). To prevent contamination of the abdominal wound with urine in the infant, the diaper should be pinned below the dressing. Sometimes a Foley catheter is used in the immediate postoperative period to divert the flow of urine away from the abdomen.

Discharge Care.After surgery, parents need instruction concerning colostomy care. Even a preschooler can be included in the care by handing articles to the parent, rolling up the colostomy pouch after it is emptied, or applying barrier preparations to the surrounding skin. Although the diagnosis of Hirschsprung disease is less frequent in school-age children or adolescents, children this age can often be involved in colostomy care to the point of total responsibility.

Some institutions and communities have enterostomal therapists who provide expert assistance in planning home care. If families require financial assistance and psychologic support, referral to a social worker, home health care agency, or community health nurse provides continuity of care.

VOMITINGVomiting is the forceful ejection of gastric contents through the mouth. It is a well-defined, complex, coordinated process that is under central nervous system control and is often accompanied by nausea and retching. Vomiting may be divided into two categories, nonbilious and bilious. Some small intestinal reflux is common in all vomiting. In nonbilious vomiting, the majority of bile drains into the more distal portions of the intestine. If an obstruction is present, nonbilious vomiting suggests a more proximal obstruction. Bilious vomiting implies a disorder of motility or distal physical blockage. Causes of nonbilious vomiting include infectious, inflammatory, metabolic/endocrinologic, neurologic, psychologic, and obstructive lesions. Causes of bilious vomiting include intestinal atresia and stenosis, malrotation with or without volvulus, ileus, intussusceptions, intestinal duplication, mass lesions, incarcerated inguinal hernia, and appendicitis. Vomiting may also be associated with other processes including acute infectious diseases, increased intracranial pressure (ICP), toxic ingestions, food intolerances and allergies, mechanical obstruction of the GI tract, metabolic disorders, and psychogenic problems. Vomiting is common in childhood, is usually self-limited, and requires no specific treatment. However, complications may occur, including dehydration and electrolyte disturbances, malnutrition, aspiration, and Mallory-Weiss syndrome (small tears in the distal esophageal mucosa).

Therapeutic ManagementManagement is directed toward detection and treatment of the cause of the vomiting and prevention of complications from the loss of fluid. Fluids are administered in the same manner and in a similar electrolyte composition to those administered for diarrhea. Although most children respond to these measures, antiemetic drugs may be needed. Antiemetics such as ondansetron (Zofran), and trimethobenzamide (Tigan) block receptors in the chemoreceptor trigger zone; others such as metoclopramide (Reglan) enhance gastroduodenal peristalsis, or promethazine (Phenergan) compete for H1-receptor sites. For children who are prone to motion sickness, it is helpful to administer an appropriate dose of dimenhydrinate (Dramamine) before a trip.


The major focus of nursing care is observation and reporting of vomiting behavior and associated symptoms and the implementation of measures to reduce the vomiting. Accurate assessment of the type of vomiting, the appearance of the vomitus, and the child's behavior in association with the vomiting helps to establish a diagnosis.

Nursing interventions are determined by the cause of the vomiting. When the vomiting is a manifestation of improper feeding methods, establishing proper techniques through teaching and example will usually correct the situation. If vomiting is believed to be an indication of obstruction, food is usually withheld or special feeding techniques are implemented. In situations in which vomiting is related to concurrent infection, dietary indiscretion, or emotional factors, efforts are directed toward maintaining hydration or preventing dehydration.

The thirst mechanism is the most sensitive guide to fluid needs, and ad libitum administration of a glucose-electrolyte solution to an alert child will restore water and electrolytes satisfactorily. It is important to include carbohydrate to spare body protein and avoid ketosis resulting from exhaustion of glycogen stores. Small, frequent feedings of fluids or foods are preferred. After vomiting has stopped, more liberal amounts of fluids are offered, followed by gradual resumption of the regular diet.

The vomiting infant or child is positioned on the side or semireclining to prevent aspiration and observed for evidence of dehydration. It is important to emphasize the need for the child to brush the teeth or rinse the mouth after vomiting to dilute hydrochloric acid that comes in contact with the teeth. A flavored mouthwash or tooth brushing will freshen the mouth. Careful monitoring of fluid and electrolyte status is necessary to prevent an electrolyte disturbance.

GASTROESOPHAGEAL REFLUXGastroesophageal reflux (GER) is defined as the transfer of gastric contents into the esophagus. Approximately 1 in 300 to 1 in 1000 children have gastroesophageal reflux. It is important to differentiate GER from gastroesophageal reflux disease (GERD). GERD represents symptoms or tissue damage that result from GER. However, GER may occur without reflux disease (GERD), and

conversely GERD may occur without regurgitation (Orenstein, 1999). GER becomes a disease when complications such as failure to thrive, bleeding, or dysphagia develop. GERD is associated with respiratory symptoms, including apnea, bronchospasm, laryngospasm, and pneumonia (Zeiter and Hyams, 1999).

The causes of GER are related to dysfunction of the lower esophageal sphincter (LES), delay in gastric emptying, poor clearance of esophageal acid, and the susceptibility of esophageal mucosa to acid injury (Zeiter and Hyams, 1999). In the past, GER was thought to be the result of decreased LES tone. However, it now appears that transient relaxation of the lower esophageal sphincter (TRLES) is the mechanism the leads to GER. Factors that cause LES pressure to vary include gastric distention, increased abdominal pressure caused by coughing, central nervous system (CNS) disease, delayed gastric emptying, hiatal hernia, and gastrostomy placement.

Infants and children who are especially prone to GER include premature infants, infants with bronchopulmonary dysplasia, and children who have had tracheoesophageal or esophageal atresia repair, neurologic disorders, scoliosis, asthma, cystic fibrosis, or cerebral palsy.

Reflux of stomach contents into the esophagus predisposes the infant or child to aspiration and the development of respiratory symptoms, particularly pneumonia. Other concerns include the association of life-threatening apnea with GER, repeated irritation of the esophageal lining with gastric acid which can lead to esophagitis and subsequent bleeding. Bleeding produces anemia, hematemesis, or melena (blood in stools). Heartburn is also a frequent symptom in children who are able to describe it (see also Box 24-6).

BOX 24-6 Clinical Manifestations and Complications of Gastroesophageal RefluxSYMPTOMS IN INFANTS

Spitting up, regurgitation, vomiting (may be forceful)

Excessive crying, irritability, arching of the back, stiffening

Weight loss, failure to thrive

Respiratory problems (cough, wheeze, stridor, gagging, choking with feedings)

Hematemesis

Apnea or apparent life-threatening event (ALTE)

SYMPTOMS IN CHILDREN

Heartburn

Abdominal pain

Noncardiac chest pain

Chronic cough

Dysphagia

Nocturnal asthma

Recurrent pneumonia

COMPLICATIONS

Esophagitis

Esophageal stricture

Laryngitis

Recurrent pneumonia

Anemia

Barrett's esophagus

Adapted from Rudolph CD and others: Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: Recommendations of the North American Society for Pediatric Gastroenterology and Nutrition, J Pediatr Gastroenterol Nutr 32(suppl 2):S1–S31, 2001.


The history and physical examination is usually sufficiently reliable to establish the diagnosis of GER. However, the upper GI series is helpful to evaluate the presence of anatomic abnormalities (e.g., pyloric stenosis, malrotation, annular pancreas, hiatal hernia, esophageal stricture). Esophageal pH monitoring establishes the presence of acid reflux. Endoscopy may be helpful to assess the presence and severity of esophagitis, strictures, and to exclude other disorders such as Crohn disease. Scintigraphy detects radioactive substances in the esophagus after a feeding of the compound and assesses gastric emptying.

Therapeutic ManagementTherapeutic management of GER depends on its severity. No therapy is needed for the infant who is thriving and has no respiratory complications. Some children require small, frequent feedings of thickened formula and positioning therapy, which helps to minimize the symptoms until the child grows and a normal physiologic barrier to reflux develops.

Although more study continues on this topic, there is evidence to support a 1- to 2-week trial of a hypoallergenic formula in formula-fed infants. Controversies surround thickened feedings. Milk-thickening agents do not improve reflux index scores, but this therapy has been shown to decrease the number of episodes of vomiting and to increase the caloric density of the formula. Feedings thickened with 1 teaspoon to 1 tablespoon of rice cereal per ounce of formula may be recommended. This may benefit infants who are underweight as a result of GERD. Constant nasogastric feedings may be necessary for the infant with severe reflux and failure to thrive.

Several studies have examined the effectiveness of positioning therapy for infants. Esophageal pH monitoring has demonstrated that infants have significantly less GER in the prone position than in the supine position. Despite the potential benefit of the prone position in relationship to GERD, because of its association with sudden infant death syndrome (SIDS), the American Academy of Pediatrics recommends the non-prone positioning for sleep (see Chapter 11). The prone position should only be considered in cases where the risk of death from the complications of GER outweighs the risk of SIDS. If the prone position is used, parents need to be cautioned to avoid soft bedding, which can increase the risk of SIDS.

In children older than a year, there is a benefit to the left side position during sleep and the elevation of the head of the bed.

Pharmacologic therapy may be used as an adjunct therapy to treat infants and children with persistent symptoms of GER. H2 antagonists, such as cimetidine (Tagamet), ranitidine (Zantac), or famotidine (Pepcid), have proved effective in reducing the amount of acid present in gastric contents and may prevent esophagitis. Proton pump inhibitors such as esomeprazole (Nexium), lansoprazole (Prevacid), omeprazole (Prilosec), pantoprazole (Protonix), and rabeprazole (Aciphex) are very effective in blocking acid production. Current investigations are ongoing to examine the effectiveness and potential side effects of these drugs in infants and children. Metoclopramide (Reglan) has been found to increase resting LES pressure mildly and to increase rates of gastric emptying. However, side effects, including restlessness, drowsiness, and extrapyramidal reaction, may occur, and in some patients, metoclopramide actually increases the number of reflux episodes. Bethanechol has been shown to increase LES pressure, but has not been proved to decrease reflux by pH probe studies. Bethanechol has a side effect of respiratory symptoms such as wheezing. In practice, metoclopramide and bethanechol have not been shown to be effective in treating GERD in children (Rudolph and others, 2001).

Cisapride (Propulsid), a drug used to promote gastric emptying, was taken off the market in 2000 because of the risk of serious cardiac arrhythmias and death associated with its use. However, the drug is available through an investigational limited-access program.

Surgical management of GER is reserved for children with severe complications such as recurrent aspiration pneumonia, apnea, severe esophagitis, or failure to thrive, and for children who have failed to respond to medical therapy. The Nissen fundoplication (Fig. 24-2) is the most common surgical procedure. This surgery involves passage of the gastric fundus behind the esophagus to encircle the distal esophagus. The most recent surgical advance is the introduction of the laparoscopic Nissen fundoplication (Rothenberg, 1998; Trover and others, 1998). Complications following fundoplication include breakdown of the wrap, small bowel obstruction, gas-bloat syndrome, infection, and retching and dumping syndrome (Rudolph and others, 2001).FIG. 24-2

Nissen fundoplication sutures passing through esophageal musculature. (Redrawn from Campbell A, Ferrara B: AORN J 57:671–679, 1993.)

Prognosis.The majority of infants with GER have a mild problem that generally improves by 12 to 18 months of age and requires only conservative lifestyle changes or medical therapy. If GER is severe and remains unsuccessfully treated, multiple complications can occur. Esophageal strictures caused by persistent esophagitis with scarring is the most significant complication. Recurrent respiratory distress with aspiration pneumonia, another serious complication, is an indication for surgery. Failure to thrive caused by GER is generally managed with medical therapy and nutritional support.

Nursing ConsiderationsNursing care is directed at (1) identifying children with symptoms; (2) educating parents regarding home care, including feeding, positioning, and medications; and (3) if appropriate, providing care for the child undergoing surgical repair (see Surgical Procedures, Chapter 22). Early in the treatment program, parents should be reassured that most infants and children outgrow GER, and often conservative lifestyle changes are sufficient. Parents need support and reassurance to implement lifestyle changes. Although it is not known if lifestyle changes bring additional benefit to patients receiving pharmacologic interventions, some changes may be helpful. Older children and adolescents need to know that caffeine, chocolate, and spicy foods may weaken the LES and aggravate symptoms. Exposure to tobacco and alcohol are also associated with GER. Obesity increases abdominal pressure, and weight management may reduce GER symptoms. To help parents cope with the inconvenience of dealing with a child, who spits up frequently, simple measures such as using bibs and protective cloths during and after feedings are beneficial. When medical management is necessary, parents need information about the medications and their potential side effects. Prokinetic medications must be given before feedings. Medications for acid control must be given regularly and timed to provide coverage two or three times a day as ordered.

INFLAMMATORY DISORDERS

ACUTE APPENDICITISAppendicitis, inflammation of the vermiform appendix (blind sac at the end of the cecum), is the most common cause of emergency abdominal surgery in childhood. In the Unites States, 60,000 to 80,000 cases are diagnosed each year. The average age of children with appendicitis is 10 years with boys and girls equally affected before puberty. Despite emphasis on early surgical intervention, the mortality of acute appendicitis in children is high. Death rates from nonperforated appendicitis are 0.1% and 5% for perforated appendicitis. Death is usually the result of complications associated with a delayed diagnosis. At the time of initial presentation, about one third of all cases involve an already perforated appendix. Abdominal pain is a common complaint in children, and perforation of

the appendix can occur within approximately 48 hours of the initial complaint of pain. Early recognition is essential.

EtiologyThe cause of appendicitis is obstruction of the lumen of the appendix usually by hardened fecal material (fecalith). Swollen lymphoid tissue, frequently occurring after a viral infection, can also obstruct the appendix. Another rare cause of obstruction is a parasite such as Enterobius vermicularis or pinworms which can obstruct the appendiceal lumen.

PathophysiologyWith acute obstruction, the outflow of mucus secretions is blocked and pressure builds within the lumen, resulting in compression of blood vessels. The resulting ischemia is followed by ulceration of the epithelial lining and bacterial invasion. Subsequent necrosis causes perforation or rupture with fecal and bacterial contamination of the peritoneal cavity. The resulting inflammation spreads rapidly throughout the abdomen (peritonitis), especially in young children, who are unable to localize infection. Progressive peritoneal inflammation results in functional intestinal obstruction of the small bowel (ileus) because intense GI reflexes severely inhibit bowel motility. Because the peritoneum represents a major portion of total body surface, the loss of ECF to the peritoneal cavity leads to electrolyte imbalance and hypovolemic shock.

Diagnostic EvaluationDiagnosis is not always straightforward. Numerous infections and inflammatory processes have similar features to that of appendicitis. Fever, vomiting, abdominal pain, and an elevated blood count are associated with appendicitis, but are also seen in inflammatory bowel disease, pelvic inflammatory disease, gastroenteritis, urinary tract infection, right lower lobe pneumonia, constipation, mesenteric adenitis, Meckel diverticulum, and intussusception. Prolonged symptoms and delayed diagnosis often occur in preschool-age children and the risk of perforation is greatest in this age-group because of their inability to verbalize their complaints.

The diagnosis is based primarily on the history and physical examination (Box 24-7). Pain, the cardinal feature, is initially generalized (usually periumbilical); however, it usually descends to the lower right quadrant. The most intense site of pain may be at McBurney point, located at a point midway between the anterior superior iliac crest and the umbilicus. Rebound tenderness is not a reliable sign and is extremely painful to the child. Referred pain, elicited by light percussion around the perimeter of the abdomen, indicates the presence of peritoneal irritation. Movement, such as riding over bumps in an automobile or gurney, aggravates the pain. In addition to pain, significant clinical manifestations include fever, a change in behavior, anorexia, and vomiting.

BOX 24-7 Clinical Manifestations of Appendicitis

Right lower quadrant abdominal pain

Fever

Rigid abdomen

Decreased or absent bowel sounds

Vomiting (typically follows onset of pain)

Constipation or diarrhea may be present

Anorexia

Tachycardia, rapid shallow breathing

Pallor

Lethargy

Irritability

Stooped posture

Laboratory studies usually include a complete blood count, urinalysis (to rule out a urinary tract infection), and in adolescent females, a serum human chorionic gonadotropin (to rule out an ectopic pregnancy). A white blood cell (WBC) count greater than

10,000/mm3 is common, but not necessarily specific for appendicitis. However, a normal white blood count and a temperature less than 100.5° F may be helpful to exclude appendicitis. An elevated percentage of bands (often referred to as "a shift to the left"), may indicate an inflammatory process. Recently, some primary care providers are adding the C-reactive protein (CRP) to the laboratory studies. CRP is an acute phase reactant that rises within 12 hours of the onset of infection. However, the CRP test has low specificity, and any infectious process can cause an elevated CRP.

Ultrasonography (US) and a computed topography (CT) scan are helpful differentiating pediatric abdominal pain from other causes. Findings such as visualization of the appendix and the presence of fluid around the appendix are important sonographic signs (Irish and others, 1998).

! NURSING ALERTSigns of peritonitis in addition to fever include sudden relief from pain after perforation, subsequent increase in pain (usually diffuse and accompanied by rigid guarding of the abdomen), progressive abdominal distention, tachycardia, rapid shallow breathing, pallor, chills, and irritability.

Therapeutic ManagementTreatment of appendicitis before perforation includes rehydration, antibiotics, and surgical removal of the appendix (appendectomy). The operation is usually performed through a right lower quadrant incision (open appendectomy). Laparoscopic surgery is now commonly used to treat nonperforated acute appendicitis (Holcomb, 2001). Recovery is rapid, and, if no complications occur, the hospital stay is short.

Ruptured Appendix.Management of the child diagnosed with peritonitis caused by a ruptured appendix often begins preoperatively with IV administration of fluid and electrolytes, systemic antibiotics, and nasogastric suction. Postoperative management includes IV fluids,

continued administration of antibiotics, and nasogastric suction for abdominal decompression until intestinal activity returns. The child with peritonitis is given antibiotics, including ampicillin, gentamicin, and clindamycin, for 7 to 10 days.

In some instances the wound is closed following irrigation of the peritoneal cavity. Many surgeons, however, leave the wound open (delayed closure) to prevent wound infection. A Penrose drain may be used to permit transperitoneal drainage. When delayed closure is used, wound irrigations and wet-to-dry dressings are a routine part of postoperative care.

Prognosis.Complications are uncommon following a simple appendectomy. The mortality rate from perforating appendicitis has improved from nearly certain death a century ago to 1% or less at present (Strahlman, 2001). The most common complications include wound infection and intraabdominal abscess. Early recognition of the illness is essential to prevent complications.

Nursing ConsiderationsBecause abdominal pain is the most common childhood complaint with appendicitis, it is important to assess the severity of pain (see Pain Assessment, Chapter 21). One of the most reliable estimates is the degree of change in behavior. For example, a child who stays home from school and voluntarily lies down or refuses to play is much more likely to have considerable discomfort than the child who is absent from school but plays contentedly at home. The younger, nonverbal child will assume a rigid, motionless, side-lying posture with the knees flexed on the abdomen, and there is decreased range of motion of the right hip. Older children may exhibit all of these behaviors while complaining of abdominal pain. They can always indicate a point at which the pain is worse than at any other location.

! NURSING ALERTIn any instance when severe abdominal pain is expected, be aware of the danger of administering laxatives or enemas or applying heat to the area. Such measures

stimulate bowel motility and increase the risk of perforation.

Postoperative Care.Postoperative care for the nonperforated appendix is the same as for most abdominal procedures. Care of the child with a ruptured appendix and peritonitis involves more complex care, and the course of recovery is considerably longer (usually 7 to 10 days of hospitalization). The child is maintained on IV fluids, allowed nothing by mouth, and kept on low continuous gastric decompression until there is evidence of intestinal activity. Listening for bowel sounds and observing for other signs of bowel activity (e.g., passage of stool) are part of the routine assessment. Management of IV therapy is the same as for any child receiving fluids and parenteral antibiotics. A drain is often placed in the wound during surgery, and frequent dressing changes with meticulous skin care are essential to prevent excoriation of the area surrounding the surgical site. Wound care includes irrigation with antibacterial solution.

Management of pain from the incision and repeated dressing changes and irrigations are an essential part of the child's care. Psychologic care of the child and parents is similar to that used in other emergency situations (see Emergency Admission, Chapter 21). Parents and older children need to express their feelings and concerns regarding the events surrounding the illness and hospitalization. The nurse can provide education and psychosocial support to promote adequate coping and alleviation of anxiety for both the child and the family. (See Nursing Care Plan The Child with Appendicitis.)

NURSING CARE PLAN: The Child With AppendicitisPREOPERATIVE CARE

NURSING DIAGNOSIS: Pain related to inflamed appendix

PATIENT GOAL 1: Will experience no pain or reduction of pain to level acceptable to child


See Pain Assessment; Pain Management, Chapter 21

Allow position of comfort (usually with legs flexed) because it may vary among children

Provide small pillow for splinting of abdomen

*Administer analgesia to provide pain relief

EXPECTED OUTCOME

Child rests quietly, reports or exhibits no evidence of discomfort

NURSING DIAGNOSIS: Risk for fluid volume deficit related to decreased intake and losses secondary to loss of appetite, vomiting

PATIENT GOAL 1: Will receive fluids for adequate hydration


Maintain NPO to minimize losses through vomiting and minimize abdominal distention

Maintain integrity of infusion site for intravenous (IV) fluids and electrolytes

*Administer IV fluids and electrolytes as prescribed

Monitor intake and output to assess hydration

EXPECTED OUTCOMES

Child receives sufficient fluids to replace losses

Child exhibits signs of adequate hydration (specify)

NURSING DIAGNOSIS: Risk for infection related to possibility of rupture

PATIENT GOAL 1: Will experience minimized risk of infection


Closely monitor vital signs, especially for increased heart rate and temperature and rapid, shallow breathing, to detect ruptured appendix

Observe for other signs of peritonitis (e.g., sudden relief of pain [sometimes] at time of perforation, followed by increased, diffuse pain and rigid guarding of the abdomen, abdominal distention, bloating, belching [from accumulation of air], pallor, chills, and irritability) for appropriate treatment to be initiated

Avoid administering laxatives or enemas, because these measures stimulate bowel motility and increase risk of perforation

Monitor white blood cell (WBC) count as indicator of infection

EXPECTED OUTCOMES

Child remains free of symptoms of peritonitis

Signs of peritonitis are recognized early (specify)

POSTOPERATIVE CARE

See Postoperative Care in Nursing Care Plan: The Child Undergoing Surgery, Chapter 22

RUPTURED APPENDIX

NURSING DIAGNOSIS: Risk for spread of infection related to presence of infective organisms in abdomen

PATIENT GOAL 1: Will experience minimized risk of spread of infection


Provide wound care and dressing changes as prescribed to prevent infection

Monitor vital signs and WBC count to assess presence of infection

*Administer antibiotics as prescribed

EXPECTED OUTCOME

Child demonstrates resolution of peritonitis, as evidenced by lack of fever, clean wound, normal WBC count

NURSING DIAGNOSIS: Risk for injury related to absence of bowel motility

PATIENT GOAL 1: Will not experience abdominal distention, vomiting


Maintain NPO in early postoperative period to prevent abdominal distention and vomiting

Maintain nasogastric tube decompression until bowel motility returns

Assess abdomen for distention, tenderness, presence of bowel sounds to assess presence of peristalsis

Monitor passage of flatus and stool as indicator of bowel motility

EXPECTED OUTCOME

Child does not exhibit signs of discomfort; abdomen remains soft and nondistended; child does not vomit

NURSING DIAGNOSIS: Altered family processes related to illness and hospitalization of child

PATIENT (FAMILY) GOAL 1: Will receive adequate support


Encourage expression of feelings and concerns to enhance coping

Encourage child to discuss hospital admission and treatments to clarify misconceptions

EXPECTED OUTCOMES

Child and family express feelings and concerns

Child and family demonstrate understanding of hospitalization and treatments

See Nursing Care Plan: The Child in the Hospital, Chapter 21

See Nursing Care Plan: The Family of the Ill or Hospitalized Child, Chapter 21


MECKEL DIVERTICULUMMeckel diverticulum is a remnant of the fetal omphalomesenteric duct that connects the yolk sac with the primitive midgut during fetal life. Normally this structure is obliterated by the seventh to eighth week of gestation, when the placenta replaces the yolk sac as the source of nutrition for the fetus. Failure of obliteration may result in an omphalomesenteric fistula (a fibrous band connecting the small intestine to the umbilicus, known as Meckel diverticulum).

Meckel diverticulum is a true diverticulum because it arises from the antimesenteric border of the small intestine, and all layers of the intestinal wall are present. The diverticulum is usually found within 100 cm (40 inches) of the ileocecal valve and averages 1 to 10 cm (2.625 to 4 inches) in length (Schwartz, 1999).

Meckel diverticulum is the most common congenital malformation of the GI tract and is present in 1% to 4% of the population (Schwartz, 1999). It is twice as common in males as in females, and complications are more frequent in males. Most symptomatic cases are seen in childhood. Patients requiring surgery are generally less than 10 years of age and about 50% are less than 2 years of age (Schwartz, 1999).

PathophysiologyThe symptomatic complications of this condition are bleeding, obstruction, or inflammation; bleeding is the most common problem in children. Gastric mucosa is the most common ectopic tissue found in Meckel diverticulum. Bleeding is caused by peptic ulceration or perforation because of the unbuffered acidic secretion. Several other mechanisms can cause obstruction. Intussusception may be led by the diverticulum. Obstruction may also be caused by entanglement of the small intestine around a fibrous cord, trapping

of a loop of intestine under the band, incarceration within a hernia sac, or volvulus of the intestinal segment containing the diverticulum. Diverticulitis occurs when peptic ulceration or obstruction leads to inflammation.

Diagnostic EvaluationDiagnosis is usually based on the history, physical examination, and a specialized radiographic study. The most common clinical presentation in children includes painless rectal bleeding, abdominal pain, or signs of intestinal obstruction (Box 24-8). Bleeding, which may be mild or profuse, often appears as dark red or "currant jelly" stools; bleeding may be significant enough to cause hypotension. The more common obstructive symptoms in children are volvulus and intussusception. The Meckel scan, a radionucleotide scintigraphy, detects the presence of gastric mucosa with an overall diagnostic accuracy of 90%. Abdominal radiographs, barium enema, and arteriography are not successful diagnostic tools. Blood studies are performed to screen for bleeding disorders and anemia (Swartz. 1999)

BOX 24-8 Clinical Manifestations of Meckel DiverticulumABDOMINAL PAIN

Similar to appendicitis

May be vague and recurrent

BLOODY STOOLS*

Painless

Bright or dark red with mucus ("currant jelly" stool)

In infants, bleeding may be accompanied by pain

Sometimes

Severe anemia

Shock* Often a presenting sign.

Therapeutic ManagementThe standard treatment is surgical removal of the diverticulum. When severe hemorrhage increases the surgical risk, interventions to correct hypovolemic shock, such as blood replacement, IV fluids, and oxygen, may be necessary. Antibiotics may be used preoperatively to control infection. If intestinal obstruction has occurred, appropriate preoperative measures are used to reverse electrolyte imbalances and prevent abdominal distention.

Prognosis.If this condition is diagnosed and treated early, full recovery is likely. The mortality rate of untreated Meckel diverticulum ranges from 2.5% to 15%. Complications of untreated Meckel diverticulum include GI hemorrhage and bowel obstruction.

Nursing ConsiderationsNursing objectives are similar to those for any child undergoing surgery (see Chapter 22). Because the onset of this condition is often rapid, parents require psychologic support. The massive intestinal bleeding that can accompany a Meckel diverticulum is traumatic to both the child and the parents and may significantly affect their emotional reaction to hospitalization and surgery.

Specific preoperative considerations with intestinal bleeding include (1) frequent monitoring of vital signs and blood pressure for shock, (2) keeping the child on bed rest, and (3) recording the approximate amount of blood lost in stools. In the absence of frank hemorrhage, the nurse tests the stools for occult blood. Postoperatively, the child requires IV fluids, and a nasogastric tube for the decompression and evacuation of gastric contents.

INFLAMMATORY BOWEL DISEASEInflammatory bowel disease (IBD) is a term that is used for two forms of chronic intestinal inflammation—ulcerative colitis (UC) and Crohn disease (CD). Although UC and CD have similar epidemiologic, immunologic, and clinical features, they are two distinct conditions with very important differences (Table 24-3).

TABLE 24-3 Clinical Manifestations of Inflammatory Bowel Diseases

GI symptoms, extraintestinal and systemic inflammatory responses, and exacerbations and remissions without complete resolution characterize these diseases. Growth failure, particularly common in CD, is an important problem unique to the pediatric population. CD is more disabling, has more serious complications, and has less effective medical and surgical treatment than UC. Because UC is confined to the colon, theoretically it may be cured with a colectomy. Over the past 30 years, the incidence of CD has risen, whereas the incidence of UC in children has remained stable. The incidence of UC in children has been estimated as 3.5 new cases per 100,000 per year; the incidence of CD is 3.11 per 100,000 (Jackson and Grand, 1999)

EtiologyDespite decades of research, the etiology of IBD is not completely understood and there is no known cure. There is evidence to indicate a multifactorial etiology. It is proposed that IBD is the result of one or more environmental influences, such as infectious organisms, dietary habits, and environmental toxins that promote disease in genetically susceptible individuals. Research is focused on theories of defective immunoregulation of the inflammatory response to bacteria or viruses in the GI tract in individuals with a

genetic predisposition. A familial tendency is apparent in approximately 20% to 25% of cases. Individuals from higher socioeconomic levels and more whites are affected, and the condition occurs more frequently among Jews living in Europe and North America, and among people living in urban settings. Males and females are affected equally (Leichtner, Jackson, and Grand, 1996). A primary role for psychologic factors has not been supported, although psychologic problems may occur as a result of IBD and psychologic problems may intensify symptoms and influence the course of the disease.

Pathophysiology: Ulcerative ColitisThe inflammation is limited to the colon and rectum, with the distal colon and rectum the most severely affected. Inflammation affects the mucosa and submucosa and involves continuous segments along the length of the bowel with varying degrees of ulceration, bleeding, and edema. The presentation may be mild, moderate, or severe, depending on the extent of mucosal inflammation and systemic symptoms. Most cases include bloody diarrhea or occult fecal blood, abdominal pain that is most intense during defecation, and varying degrees of systemic manifestations and growth abnormalities (Leichtner, Jackson, and Grand, 1996). Thickening of the bowel wall and fibrosis are unusual, but long-standing disease can result in shortening of the colon and strictures. Extraintestinal manifestations are less common in UC than in CD.

Pathophysiology: Crohn DiseaseThe chronic inflammatory process of CD involves any part of the GI tract from the mouth to the anus but most often affects the terminal ileum. The disease involves all layers of the bowel wall (transmural) in a discontinuous fashion, meaning that between areas of intact mucosa, there are areas of affected mucosa (skip lesions). The most common symptoms are abdominal pain with cramps, diarrhea, and weight loss. Other manifestations include fever; anorexia; rectal bleeding; and perineal discomfort, including anal fissures or fistulas. The presence of perianal disease is a strong indication for CD. Mild GI symptoms, poor growth, and extraintestinal manifestations may be present for several years before overt GI symptoms occur. The inflammation may result in ulcerations, fibrosis, and adhesions, stiffening of the bowel wall, stricture formation, and fistulas to other loops of bowel, bladder, vagina, or skin. Extraintestinal

manifestations, including erythema nodosum, large joint arthritis, uveitis, mouth ulcers, liver disease, and renal calculi are common.

Diagnostic EvaluationThe diagnosis of UC and CD is derived from the history, physical examination, laboratory evaluation, and other diagnostic procedures. Because the diseases have similar symptoms, it is difficult to distinguish CD from UC. UC is confined to the large bowel and affects only the inner lining (the mucosa and submucosa). CD involves all layers of the bowel (transmural). Laboratory tests include a CBC to evaluate anemia and an erythrocyte sedimentation rate or CRP to assess the systemic reaction to the inflammatory process. Levels of total protein, albumin, iron, zinc, magnesium, vitamin B12, and fat-soluble vitamins may be low in children with CD. Stools are examined for the presence of blood, leukocytes, and infectious organisms. A serologic panel is often used in combination with clinical findings to diagnose IBD and to differentiate between CD and UC. In IBD, autoantibodies called antineutrophil cytoplasm antibodies (ANCA) may be detected in the blood. The perinuclear antineutrophil cytoplasm antibody (pANCA) is associated with UC. Approximately 60% to 70% of patients with UC and 15% of those with CD are pANCA-positive. Anti-Saccharomyces cerevisiae antibodies (ASCA) have been found in 67% to 92% of individuals with CD (Baron, 2002).

In patients with CD, an upper GI series with small bowel follow-through reveals images that demonstrate narrowing or modularity of the small bowel. The terminal ileum may be narrowed or rigid with partial obstruction. In about one third of the patients, lesions pierce the walls of the small intestine and colon, creating tracts called fistulas between the intestine and adjacent structures such as the bladder, anus, vagina, or skin. Fistulas may become infected causing discharge of pus and mucous. A CT scan is helpful in evaluating abscesses, fistulas, and bowel wall thickening. Endoscopy, direct visualization of the surface of the gastrointestinal tract with biopsies, is necessary to confirm the diagnosis and to assess the extent of inflammation and to evaluate for strictures. Endoscopy may be both upper and lower colonoscopy depending on the clinical presentation of the child.

Therapeutic Management

The goals of therapy are to (1) control the inflammatory process to reduce or eliminate the symptoms, (2) obtain long-term remission, (3) promote normal growth and development, and (4) allow as normal a lifestyle as possible. Treatment is individualized and managed according to the type and the severity of the disease, its location, and the response to therapy.

Medical TreatmentDrugs that mediate and control inflammation (corticosteroids, aminosalicylates, sulfasalazine, immunosuppressives, and biologic therapies) are used to treat IBD. Corticosteroids, such as prednisone and prednisolone, are used in short bursts to suppress the inflammatory response in moderate to severe IBD. These drugs inhibit the production of adhesion molecules, cytokines, and leukotrienes. Although these drugs reduce the acute symptoms of IBD, they have side effects that relate to long-term use, including growth suppression (adrenal suppression), weight gain, and decreased bone density (Baron, 2002). High doses of IV corticosteroids may be administered in acute episodes and tapered according to clinical response. Aminosalicylates are useful in decreasing the frequency of recurrences in mild cases of IBD. Sulfasalazine decreases inflammation by inhibiting prostaglandin synthesis. Because it is only active in the colon, it is not effective in the treatment of small bowel disease. Sulfasalazine may decrease the absorption of folic acid; therefore, daily supplements of folic acid are needed in long-term therapy. Side effects of this drug include headache, nausea, vomiting, neutropenia, and oligospermia. The side effects are caused primarily by the sulfapyridine component of the drug, so alternative nonabsorbable salicylate drugs such as olsalazine and mesalamine may be prescribed. Mesalamine comes in a variety of formulations that are active in different parts of the bowel. Asacol is active in the terminal ileum and the colon. Pentasa targets the jejunum and Rowasa is a topical preparation administered rectally by enema to relieve inflammation of the distal colon and rectum.

Immunomodulatory medications are used when the symptoms of IBD persist despite the use of steroids or when the patient cannot be weaned from corticosteroids (e.g., when reducing the dose of steroids results in return of symptoms such as diarrhea, pain, and bleeding). Azathioprine and its metabolite 6-MP block the synthesis of purine, thus inhibiting the ability of DNA and ribonucleic

acid (RNA) to hinder lymphocyte function, especially that of T cells. Side effects include infection, pancreatitis, hepatitis, bone marrow toxicity, arthralgia, and malignancy. Other immunomodulatory medications include methotrexate, cyclosporin, and mycophenolate mofetil. Patients on these medications require regular monitoring of their CBC and differential to assess for changes that reflect suppression of the immune system.

Antibiotics, such as metronidazole and ciprofloxacin, may be used as an adjunctive therapy to treat complications such as perianal disease or small bowel bacterial overgrowth. Side effects of this drug are peripheral neuropathy, nausea, and a metallic taste.

Biologic therapies act to regulate inflammatory and anti-inflammatory cytokines. Tumor necrosis factor-alpha (TNF-α) is believed to influence active inflammation. Infliximab (Remicade) is an antibody to TNF-α. This drug is given via IV infusions 6 to 12 weeks apart. Approximately 5% of patients have acute allergic reactions to infliximab and require premedication with prednisone and diphenhydramine before infusion to prevent reactions. Long intervals between infusions may predispose patients to serum sickness (an immune response causing fever, muscle pain, arthritis, and hives) (Baron, 2002). Severe complications of this drug include lupus-like syndrome or lymphoma. Other biologic medications include methotrexate, interleukin-10, and thalidomide.

Nutritional Support.Nutritional support is a primary component of the treatment of IBD. Growth failure is a common serious complication, especially in CD. Growth failure is characterized by weight loss, alteration in body composition, retarded height, and delayed sexual maturation. Malnutrition causes the growth failure, and its etiology is multifactorial. Malnutrition occurs as a result of inadequate dietary intake, excessive GI losses, malabsorption, drug-nutrient interaction, and increased nutritional requirements. Inadequate dietary intake occurs with anorexia and episodes of increased disease activity. Excessive loss of nutrients (protein, blood, electrolytes, and minerals) occurs secondary to intestinal inflammation and diarrhea. Carbohydrate, lactose, fat, vitamin, and mineral malabsorption as well as vitamin B12 and folic acid deficiencies occur with disease episodes and with drug administration and when the terminal ileum is resected. Finally,

nutritional requirements are increased with inflammation, fever, fistulas, and during periods of rapid growth (e.g., adolescence).

The goals of nutritional support include (1) correction of nutrient deficits and replacement of ongoing losses, (2) provision of adequate energy and protein for healing, and (3) provision of adequate nutrients to promote normal growth. Nutritional support includes both enteral and parenteral nutrition. A well-balanced, high-protein, high-calorie diet is recommended for children whose symptoms do not prohibit an adequate oral intake. There is little evidence that avoiding specific foods influences the severity of the disease. Supplementation with multivitamins, iron, and folic acid is recommended.

Special enteral formulas, given either by mouth or continuous nasogastric infusion (often at night) may be required. Elemental formulas are completely absorbed in the small intestine with almost no residue. Several studies have demonstrated that a diet consisting only of elemental formula not only improved nutritional status but also induced disease remission, either without steroids or with a diminished dosage of steroids required. An elemental diet is a safe and potentially effective primary therapy for patients with CD.

TPN has also improved nutritional status in patients with IBD. Short-term remissions have been achieved after TPN, although complete bowel rest has not reduced inflammation or added to the benefits of improved nutrition by TPN (Leichtner, Jackson, and Grand, 1996). Nutritional support is less likely to induce a remission in UC than in CD. Improvement of nutritional status is important, however, in preventing deterioration of the patient's health status and in preparing the patient for surgery.

Surgical Treatment.Surgery is indicated for UC when medical and nutritional therapies fail to prevent complications. Surgical options include a subtotal colectomy and ileostomy that leaves a rectal stump as a blind pouch. A reservoir pouch is created in the configuration of a J or S to help improve continence postoperatively. An ileoanal pull-through preserves the normal pathway for defecation. In many cases UC can be cured with a total colectomy.

Surgery may be required in children with CD when complications cannot be controlled by medical and nutritional therapy. Segmental intestinal resections are preformed for small bowel obstructions, strictures, or fistulas. Partial colonic resection is not curative, and the disease often recurs.

Prognosis.IBD is a chronic disease. Relatively long periods of quiescent disease may follow exacerbations. The outcome of the disease is influenced by the regions and severity of involvement, as well as by appropriate therapeutic management. Malnutrition, growth failure, and bleeding are serious complications. The overall prognosis for UC is good.

The development of carcinoma of the colon is a long-term complication of IBD. In UC, removal of the diseased bowel prevents development of carcinoma. In CD, however, surgical removal of the affected bowel does not prevent bowel cancer, and routine screening of stool specimens is necessary for early detection.

Nursing ConsiderationsThe nursing considerations in the management of IBD extend beyond the immediate period of hospitalization. These interventions involve (1) continued guidance of families in terms of dietary management, (2) coping with factors that increase stress and emotional lability, (3) adjusting to a disease of remissions and exacerbations, and (4) when indicated, preparing the child and parents for the possibility of diversionary bowel surgery.

Because nutritional support is an essential part of therapy, encouraging the anorectic child to consume sufficient quantities of food is often a challenge. Successful interventions include: involving the child in meal planning; encouraging small, frequent meals or snacks rather than three large meals a day; serving meals around medication schedules when diarrhea, mouth pain, and intestinal spasm are controlled; and preparing high-protein, high-calorie foods such as eggnog, milkshakes, cream soups, puddings, or custard (if lactose is tolerated) (see Feeding the Sick Child, Chapter 22). Foods that are known to aggravate the condition are avoided. Using bran or a high-fiber diet for IBD is questionable. Bran, even in small

amounts, has been shown to worsen the patient's condition. Occasionally the occurrence of aphthous stomatitis further complicates adherence to dietary management. Mouth care before eating and the selection of bland foods help relieve the discomfort of mouth sores.

When nasogastric feedings or TPN are indicated, nurses play an important role in explaining the purpose and the expected outcomes of this therapy. The nurse should acknowledge the anxieties of family members, and give them adequate time to demonstrate the skills necessary to continue the therapy at home if needed (see Critical Thinking Exercise).

CRITICAL THINKING EXERCISE: Inflammatory Bowel DiseaseSusan, a 13-year-old girl, was admitted to the hospital because of bloody diarrhea, abdominal pain, and weight loss. After a thorough evaluation, including laboratory tests, radiographic studies, and gastrointestinal endoscopy procedures, the diagnosis of Crohn disease (CD) was made. Medical treatment, including corticosteroid drugs and nutritional support, was implemented during this hospitalization.

Susan has improved considerably, and is to be discharged home this week. Enteral formula administered by continuous nighttime nasogastric (NG) tube infusion will be continued at home, and both Susan and her family are eager to learn how to perform these feedings. You are the nurse who is responsible for Susan's discharge planning. Which interventions relating to these feedings should you include in Susan's preparations for discharge?

QUESTIONS1. Evidence—Are there sufficient data to formulate any specific interventions for discharge?


a. The goals of nutritional support for children with CD

b. Teaching required by an adolescent or family member who is administering NG tube feedings at home

c. Psychosocial issues related to CD

3. What are the priorities for discharge planning at this time?

4. Does the evidence support your conclusion?

5. Are there alternative perspectives to your conclusion? What are they?

ANSWERS1. Yes, there is sufficient evidence to arrive at some conclusions about what to include in Susan's discharge planning.

2. a. The goals of nutritional support for a patient with CD include (1) correction of nutrient deficits and replacement of ongoing losses, (2) provision of adequate energy and protein for healing, and (3) provision of adequate nutrients to support normal growth.

b. See Chapter 22, Gavage Feeding, pp 778.

c. Adolescents who are diagnosed with CD must adjust to the fact that they have a chronic illness that is characterized by remissions and exacerbations. CD may affect their activities of daily living, their social interactions with peers, and their ability to attend school. An important goal of therapy for adolescents with CD is to allow them to have as normal a lifestyle as possible.

3. The most immediate priority for discharge is to teach Susan and her family how to insert the NG tube, how to administer the feedings, how to obtain the supplies needed for the tube feedings at home, and how to observe for any untoward effects of the NG feedings. As Susan's discharge nurse, you should have Susan and another family member insert the NG tube, demonstrate how to check the placement of the NG tube, and how to start and stop the feedings while Susan is in the hospital. As Susan's nurse, you will also need to arrange for the appropriate vendors to deliver the feeding tube

supplies and feeding pump to Susan's home prior to discharge so the supplies will be in place when Susan is discharged. While doing all this teaching, you should also be alert to any questions, worries, or anxieties that Susan or her family members may express.

4. Yes, Susan is to receive nighttime NG tube infusions at home, and her family has expressed a desire to perform this procedure at home. Therefore, this discharge teaching is needed and required.

5. Now that the acute disease exacerbation is under control, Susan will be able to resume school attendance and activities with her peers. However, some of her activities of daily living have been changed (e.g., the nighttime NG feedings), and she will need to adjust to remissions and exacerbations that characterize CD. To help Susan and her family cope with these changes, you should refer them to the services of the Crohn's and Colitis Foundation of America, Inc. Perhaps Susan can become involved in one of the adolescent peer-support groups that are sponsored by this group.

The importance of continued drug therapy despite remission of symptoms must be stressed to the child and family members. Failure to adhere to the pharmacologic regimen can result in exacerbation of the disease (see Compliance, Chapter 22).

Family Support.The nurse should attend to the emotional components of the disease and assess any sources of stress. Frequently, the nurse can help children to adjust to problems of growth retardation, delayed sexual maturation, dietary restrictions, feelings of being "different" or "sickly," inability to compete with peers, and necessary absence from school during exacerbations of the illness (see Impact of Chronic Illness, Chapter 18).

If a permanent colectomy/ileostomy is required, the nurse can teach the child and family how to care for the ileostomy. The nurse can also emphasize the positive aspects of the surgery, particularly accelerated growth and sexual development, permanent recovery, and the eliminated risk of colonic cancer in UC; and the normality of life despite bowel diversion. Introducing the child and parents to

other ostomy patients, especially those who are the same age, can be effective in fostering eventual acceptance. Whenever possible, the continent ostomies should be offered as options to the child, although they are not performed in all centers in the United States.

Because of the chronic and often lifelong nature of the disease, families benefit from the educational services provided by organizations such as the Crohn's and Colitis Foundation of America, Inc. (CCFA).* If diversionary bowel surgery is indicated, the United Ostomy Association† and the Wound Ostomy and Continence Nurses Society‡ are available to assist with ileostomy care and provide important psychologic support through their self-help groups. Adolescents often benefit by participating in peer-support groups, which are sponsored by the CCFA.

* 386 Park Avenue South, 17th Floor, New York, NY 10016; (800) 932-2423; website: http://www.ccfa.org.† 19772 MacArthur Boulevard, Suite 200, Irvine, CA 92612-2405; (714) 660-8624; website: http://www.uoa.org.‡ 1550 South Coast Highway, Suite 201, Laguna Beach, CA 92651; (888) 224-9626; website: http://www.wocn.org. In Canada: Crohn's and Colitis Foundation of Canada, website: http://www.ccfc.ca; and United Ostomy Association, Canada, PO Box 825-50, Charles Street East, Toronto, Ontario M4Y 2N7; (416) 595-5452; fax: (416) 595-9924.

PEPTIC ULCER DISEASEPeptic ulcers may be classified as acute or chronic, and peptic ulcer disease (PUD) is a chronic condition that affects the stomach or duodenum. Ulcers are described as gastric or duodenal and as primary or secondary. A gastric ulcer involves the mucosa of the stomach; a duodenal ulcer involves the pylorus or duodenum. Most primary ulcers occur in the absence of a predisposing factor, and tend to be chronic occurring more frequently in the duodenum. Stress ulcers result from the stress of a severe underlying disease or injury (e.g., severe burns, sepsis, intracranial disease, severe trauma, multisystem organ failure), and are more frequently acute and gastric.

About 1.7% of children in general pediatric practices have PUD, and the disease represents about 3.4% per 10,000 of pediatric hospital admissions. Primary ulcers are more common in children older than 6 years, and stress ulcers are more common in infants younger than 6

months. Except for very young children, the incidence is two to three times greater in boys than in girls (Motil, 1999).

EtiologyThe exact cause is unknown, although infectious, genetic, and environmental factors are important. There is an increased familial incidence and the disease is increased in persons with blood group O.

There is a significant relationship between the bacterium, Helicobacter pylori (previously called Campylobacter pylori), and ulcers. H. pylori is known to colonize the gastric mucosa and has been identified in 90% to 100% of adult patients with PUD. It may cause ulcers by weakening the gastric mucosal barrier and allowing acid to damage the mucosa. It is believed that H. pylori is acquired via the fecal-oral route, and this hypothesis is supported by finding viable H. pylori in feces. The exact mechanism by which it causes gastric inflammation is unclear; however, the large amount of urease present in H. pylori may be a factor. Urease hydrolyses urea to ammonia and bicarbonate in the gastric mucosa and ammonia can be toxic to gastric cells (Chelimsky and Czinn, 2001).

In addition to ulcerogenic drugs, both alcohol and smoking contribute to ulcer formation. There is no conclusive evidence to implicate particular foods, such as caffeine-containing beverages or spicy foods, but polyunsaturated fats and fiber may play a role in ulcer formation.

Psychologic factors may play a role in the development of PUD, and stressful life events, dependency, passiveness, and hostility have all been implicated as contributing factors.

PathophysiologyMost likely, the pathology is due to an imbalance between the destructive (cytotoxic) factors and defensive (cytoprotective) factors in the GI tract. The toxic mechanisms include acid, pepsin, medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), bile acids, and infection with H. pylori. The defensive factors include the mucous layer, local bicarbonate secretion, epithelial cell renewal, and mucosal blood flow (Motil, 1999). Prostaglandins play a role in mucosal defense because they stimulate both mucus and alkali secretion. The primary mechanism that

prevents the development of peptic ulcer is the secretion of mucus by the epithelial and mucus glands throughout the stomach. The thick mucus layer acts to diffuse acid from the lumen to the gastric mucosal surface, thus protecting the gastric epithelium. The stomach and the duodenum produce bicarbonate, and production of bicarbonate decreases acidity on the epithelial cells thereby minimizing the effects of the low pH (Chelimsky and Czinn, 2001). When abnormalities in the protective barrier exist, the mucosa is vulnerable to damage by acid and pepsin. Exogenous factors, such as aspirin and NSAIDs cause gastric ulcers by inhibition of prostaglandin synthesis. Zollinger-Ellison syndrome may occur in children who have multiple, large, or recurrent ulcers. This syndrome is characterized by hypersecretion of gastric acid, intractable ulcer disease, and intestinal malabsorption caused by a gastrin-secreting tumor of the pancreas (Motil, 1999).

Diagnostic EvaluationDiagnosis is based on the history of symptoms, physical examination, and diagnostic testing. The focus is on symptoms such as epigastric abdominal pain, nocturnal pain, oral regurgitation, heartburn, weight loss, hematemesis, and melena (Box 24-9). History should include questions relating to the use of potentially causative medications such as NSAIDS, corticosteroids, alcohol, and tobacco. Laboratory studies may include a CBC to detect anemia, stool analysis for occult blood, liver function tests, sedimentation rate, or CRP to evaluate inflammatory bowel disease, amylase and lipase to evaluate pancreatitis, and gastric acid measurements to identify hypersecretion. A lactose breath test may be performed to detect lactose intolerance.

Radiographic studies such as an upper GI series may be performed to evaluate obstruction or malrotation. An upper endoscopy is the most reliable procedure to diagnose PUD. A biopsy is taken to determine the presence of H. pylori. H. pylori can also be diagnosed by a blood test that identifies the presence of the antigen to this organism. The C urea breath test measures bacterial colonization in the gastric mucosa. This test is used to screen for H. pylori in adults and is now being evaluated for children.

BOX 24-9 Characteristics of Peptic UlcerNEONATES

Usually gastric and secondary

Commonly has a history of prematurity, respiratory distress, sepsis, hypoglycemia, or an intraventricular hemorrhage

Perforation may be first sign that massive bleeding may occur

INFANTS TO 3-YEAR-OLD CHILDREN

Most likely to have a secondary ulcer located equally in the stomach or duodenum

Primary ulcers less common and usually located in stomach

Likely to present in relation to illness, surgery or trauma

Hematemesis, melena or perforation

2- TO 6-YEAR-OLD CHILDREN

Primary or secondary ulcers

Located equally in stomach and duodenum

Perforation more likely in secondary ulcers

Periumbilical pain, poor eating, vomiting, irritability, nighttime waking, hematemesis, melena

CHILDREN 6 YEARS AND OLDER

Usually primary and most often duodenal

More typical of adult type

Chance of recurrence greater

Often associated with H. pylori

Epigastric pain or vague abdominal pain

Nighttime waking, hematemesis, melena, and anemia may occur

Therapeutic Management

The major goals of therapy for children with PUD are to relieve discomfort, promote healing, prevent complications, and prevent recurrence. Management is primarily medical and consists of administration of medications to treat the infection and to reduce or neutralize gastric acid secretion.

Antacids are beneficial medications to neutralize gastric acid. However, in terms of healing the ulcer or eradication of H. pylori, antacids are not as effective as medications that inhibit acid secretion.

Histamine (H2) receptor antagonists (antisecretory drugs) act to suppress gastric acid production. Cimetidine (Tagamet), ranitidine (Zantac), and famotidine (Pepcid) are examples of these medications. These medications have few side effects.

Proton pump inhibitors (PPI), such as omeprazole and lansoprazole, act to inhibit the hydrogen ion pump in the parietal cells, thus blocking the production of acid. Controlled studies of these drugs have been done in adults, and these drugs are now commonly used to treat ulcers in children. They appear to be well tolerated and to have infrequent side effects (e.g., headache, diarrhea, nausea and vomiting).

Mucosal protective agents, such as sucralfate and bismuth-containing preparations, may be prescribed for PUD. Sucralfate is an aluminum-containing agent that forms a protective barrier over ulcerated mucosa to protect against acid and pepsin. Sucralfate is available in both pill and liquid forms. Because sucralfate blocks the absorption of other medications, it should be given separately from other medications.

Bismuth compounds are sometimes prescribed for the relief of ulcers but they are used less frequently than PPIs. Although these compounds inhibit the growth of microorganisms, the mechanism of their activity is poorly understood. In combination with antibiotics, bismuth is effective against H. pylori. Although concern has been expressed about the use of bismuth salts in children because of potential side effects, none of these side effects have been reported when these compounds have been used in the treatment of H. pylori infection.

Triple drug therapy is the recommended treatment regimen for H. pylori. Combination therapy has demonstrated 90% effectiveness in

eradication of H. pylori when compared with antibiotic monotherapy (Motil, 1999). Examples of drug combinations used in triple therapy are: (1) bismuth, clarithromycin, and metronidazole; (2) lansoprazole, amoxicillin, and clarithromycin; and (3) metronidazole, clarithromycin, and omeprazole.

In addition to medications, the child with PUD should be given a nutritious diet and advised to avoid caffeine. Adolescents are warned about gastric irritation associated with alcohol use and smoking.

Children with an acute ulcer who have developed complications, such as massive hemorrhage, require emergency care. The administration of IV fluids, blood, or plasma depends on the amount of blood loss. Replacement with whole blood or packed cells may be necessary for significant loss.

Surgical intervention may be required for complications such as hemorrhage, perforation, or gastric outlet obstruction. Ligation of the source of bleeding or closure of a perforation is performed. A vagotomy and pyloroplasty may be indicated in children with recurring ulcers despite aggressive medical treatment.

Prognosis.The long-term prognosis for PUD is variable. Many ulcers are successfully treated with medical therapy; however, primary duodenal peptic ulcers often recur. Complications such as GI bleeding can occur and extend into adult life. The effect of maintenance drug therapy on long-term morbidity remains to be established with further studies.

Nursing ConsiderationsThe primary nursing goal is to promote healing of the ulcer through compliance with the medication regimen. If an analgesic/antipyretic is needed, acetaminophen, not aspirin or NSAIDs, is used. Critically ill neonates, infants, and children in intensive care units should receive antacids and H2 blockers to prevent stress ulcers. Critically ill children receiving IV H2 blockers should have their gastric pH values checked at frequent intervals and buffered with antacid if necessary.

The role of stress in ulcer formation should be considered for nonhospitalized children with chronic illnesses. In children, many

ulcers occur secondarily to other conditions, and the nurse should be aware of family and environmental conditions that may aggravate or precipitate ulcers. Children may benefit from psychologic counseling and from learning how to cope constructively with stress.

HEPATIC DISORDERS

ACUTE HEPATITIS

EtiologyHepatitis is an acute or chronic inflammation of the liver that can result from several different causes (e.g. a virus, chemical or drug reaction, or other diseases). Nonviral causes of hepatitis include autoimmune hepatitis, Wilson's disease, alpha 1 antitrypsin deficiency, and steatohepatitis. The following six viruses cause most cases (90%) of viral hepatitis:

• Hepatitis A virus (HAV)

• Hepatitis B virus (HBV)

• Hepatitis C virus (HCV)

• Hepatitis D virus (HDV)

• Hepatitis E virus (HEV)

• Hepatitis G virus (HGV)

(Table 24-4)

TABLE 24-4 Comparison of Types A, B, and C Hepatitis

Hepatitis A.HAV is the most common form of acute viral hepatitis in most parts of the world. It is a member of the picornavirus family. The virus produces a contagious disease transmitted primarily in contaminated stool spread via the fecal-oral route from person to person. HAV has been associated with miniepidemics in areas of poor hygiene and high population density. There is no chronic or carrier state. HAV infection affects individuals of all ages, but the

highest incidence occurs among preschool- or school-age children younger than 15 years. Children may serve as the source of HAV infection in adults, such as in childcare center exposures. Usually HAV disease in children is mild. It is frequently anicteric and often subclinical. Infected children who show no symptoms may still spread the virus to others. HAV can be severe in children with immunodeficiency disorders. The incubation period is approximately 3 weeks. Although some cases may be prolonged, the prognosis is excellent. A highly effective vaccine for HAV was introduced in 1997 (Balistreri and others, 2002; Regev and Schiff, 2000).

Hepatitis B.HBV infection can occur as an acute or chronic infection and may range from being asymptomatic and limited to causing fatal fulminant (rapid and severe) hepatitis. HBV varies greatly throughout the world. High prevalence areas have been identified in Africa and Asia; the United States is considered a low prevalence area. Transmission is usually via the parenteral route through the exchange of blood or any bodily secretion or fluid. Infections from blood transfusion have been reduced as a result of blood product-screening procedures. Transplantation of organs, intimate physical contact, transmission from mother to infant, and the splashing of contaminated fluids into the mouth or eyes are other sources of infection. Adults whose occupations are associated with exposure to blood or blood products (such as health care workers) are at increased risk for infection and should receive HBV vaccination.

Most HBV infection in children is acquired perinatally. Newborns are at risk for hepatitis if the mother is infected with HBV or was a carrier of HBV during pregnancy. Possible routes of maternal-fetal-infant transmission include (1) leakage of virus across the placenta late in pregnancy or during labor and (2) ingestion of amniotic fluid or maternal blood.

HBV infection occurs in children and adolescents in the following high-risk groups: (1) individuals with hemophilia and others who have received multiple transfusions, (2) children and adolescents involved in IV drug abuse, (3) institutionalized children and adolescents, (4) preschool-age children in endemic areas, and (5) individuals engaged in heterosexual or homosexual activity with infected partners. The incubation period of HBV infection varies from 45 to 160 days.

Hepatitis C.About 0.2% to 0.4% of children younger than 12 years of age are infected with HCV. It is estimated that 4 million people in the United States are anti-HCV-positive. Approximately 7% of HCV-infected mothers transmit the HCV virus to their newborns (Balistreri and others, 2002). The second most common route of infection is by percutaneous exposure, which occurs through transfusion of blood or blood products, transplantation of organs or tissues, or through sharing used needles. Transfusion-associated HCV infection is low, but a common cause of infection is injection drug use. The American Academy of Pediatrics (1998) suggests screening the following groups: (1) all infants born to HCV-infected women, (2) individuals who received blood products before 1992, (3) individuals involved in injection drug use, and (4) individuals who receive hemodialysis. The length of time that maternal antibody is present in infants born to HCV-infected women must be considered, and screening should be done after the infant is 12 months old. However, a routine screening program, such as that for HBV, is not recommended.

The clinical course of HCV infection is variable. Incubation averages 6 to 7 weeks, with a range of 2 weeks to 6 months. HCV causes acute hepatitis that progresses to chronic disease in more than 70% of affected individuals and can cause end-stage liver disease in 10% of these patients. However, both acute and chronic HCV infection often produce only mild nonspecific symptoms or no symptoms at all (Bonkovsky and Mehta, 2001)

Current recommendations are to evaluate HCV-infected children at regular intervals to monitor for chronic hepatitis. Most children will be asymptomatic with evidence of chronic hepatitis on liver biopsy. Liver enzyme levels may fluctuate between periods of normal and elevated values (Balistreri, 1999).

Hepatitis D.HDV is an important cause of acute and chronic liver disease. HDV is a defective RNA virus that requires the function of HBV. HDV infection occurs primarily in hemophiliac patients and IV drug abusers. The incubation period is 2 to 8 weeks. Both acute and chronic forms are more severe than HBV infection and can lead to cirrhosis. Testing for HDV infection is recommended in children with

chronic HBV infection or severe liver disease or in children with acute exacerbation of a previously stable liver disease.

Hepatitis E.HEV infection is enterally transmitted. Transmission may occur through the fecal-oral route or from contaminated water. The incubation period is 2 to 9 weeks. This illness is uncommon in children, does not cause chronic liver disease, is not a chronic condition, and has no carrier state. The mortality rate resulting from submassive hepatic necrosis is low except in pregnant women in their third trimester, in whom mortality reaches 20%.

Hepatitis G.HGV is a blood borne virus that may also be transmitted by organ transplantation. High-risk groups include transfusion recipients, IV drug users, and individuals infected with HCV. Individuals with the virus are often asymptomatic, and most infections are chronic. The incubation period is unknown.

Diagnostic EvaluationDiagnosis is based on the history (especially regarding possible exposure to a hepatitis virus), physical examination, and serologic markers (antibodies or antigens) indicating the presence of active infection with hepatitis A, B, and C or previous infection. Because the liver has a large function reserve, abnormal laboratory tests may be the only indication of hepatitis. However, liver function tests (LFTs) are not specific for the diagnosis of viral hepatitis. Although serum aspartate and alanine aminotransferase (AST and ALT) levels are markedly elevated in viral hepatitis, other diseases or conditions may cause their elevation. When hepatitis is severe, albumin levels are depressed and prothrombin times are increased. Serum bilirubin levels peak 5 to 10 days after clinical jaundice appears.

Diagnosis of viral hepatitis is based on the presence of specific viral markers. Diagnosis of acute HAV infection is based on the presence of anti-HAV immunoglobulin (Ig)M antibody in the serum. HBV diagnosis depends on the presence of hepatitis B surface antigen (HBsAG) or anti-HBV core (anti-HBc) IgM antibody. Chronic HBV infection is associated with the persistence of HBsAg and HBV DNA

markers. The diagnosis of HCV is based on the detection of anti-HCV antibodies and confirmation by polymerase chain reaction for hepatitis C RNA.

Other diagnostic studies include a urinalysis to evaluate the bilirubinemia and to rule out other causes of hepatitis. An abdominal ultrasound provides measurement of liver size, detection of cystic lesions and stones, and imaging of the gallbladder. Cholescintigraphy radionuclide imaging detects abnormalities in liver uptake, concentration, and excretory function. Finally, a liver biopsy aids in assessing the severity of the disease.

PathophysiologyPathologic changes occur primarily in the parenchymal cells of the liver and result in varying degrees of swelling, infiltration of liver cells by mononuclear cells, subsequent degeneration, necrosis, and fibrosis.

Hepatitis can be self-limited, and complete regeneration of liver cells without scarring may occur. However, some forms of hepatitis do not result in complete return of liver function. These include fulminant hepatitis, which is characterized by a severe, acute course and massive destruction of the liver, which results in liver failure and death in 1 to 2 weeks. Subacute or chronic active hepatitis is characterized by progressive liver destruction, uncertain regeneration, scarring, and potential cirrhosis.

The initial anicteric (absence of jaundice) phase usually lasts 5 to 7 days and is often mistaken for influenza. Symptoms include nausea, vomiting, extreme anorexia, malaise, easy fatigability, arthralgia, skin rashes, slight to moderate fever, and epigastric or upper right quadrant abdominal pain. Dark urine is a symptom of the icteric (jaundice) phase. Pruritus may accompany jaundice and can be a bothersome, but many children with acute viral hepatitis do not develop jaundice.

Therapeutic ManagementTreatment options for viral hepatitis are limited. The goals of management include early detection, recognition of chronic liver disease, support and monitoring, and prevention of spread of the disease.

HAV infection is an acute disease that resolves with support and management of symptoms. HBV and HCV treatment is directed at managing the viral load to prevent further destruction of the liver. Currently, interferons are used to treat HBV and HCV. Interferons are naturally occurring proteins that exert antiviral, antiproliferative, and immunomodulatory effects. A recent interferon formulation, pegylated interferon, can be administered once a week and has been found to sustain plasma levels and enhance viral suppression (Karnam and Reddy, 2003). Lamivudine and adefovir are two other interferon analogues that suppress the replication of HBV (Yuen and Lai, 2001). A combination of alpha interferon and ribavirin has resulted in a sustained response in some adult patients with HBV and HCV (Regev and Schiff, 2000).

Another important aspect of the therapeutic management of hepatitis involves hospitalization. Hospitalization is necessary if coagulopathy or fulminant hepatitis are present.

Prevention.Proper handwashing and standard isolation precautions can prevent the spread of hepatitis. Prophylactic use of standard immune globulin (IG) is effective in preventing HAV infection in situations of preexposure (e.g., anticipated travel to areas where HAV is prevalent) or in situations of postexposure during the early part of the incubation period. Hepatitis B immune globulin (HBIG) is effective in preventing HBV infection after exposure. IG must be administered less than 2 weeks after exposure.

Vaccines have been developed to prevent HAV and HBV infection. HBV vaccination is recommended for all newborns and for high-risk groups. HAV is also recommended for high-risk groups (see Immunizations, Chapter 10). Active immunizations are not available against HCV. It is possible to prevent HDV infection by preventing HBV infection.

Prognosis.The prognosis for children with hepatitis is variable and depends on the type of virus. HAV usually causes a mild and brief illness with no carrier state. HBV causes a wide spectrum of acute and chronic illness. Chronic HBV infection leads to cirrhosis in approximately one fourth to one third of the cases. Hepatocellular carcinoma is a

potentially fatal complication of HBV infection. HCV infection frequently becomes chronic, and cirrhosis may develop in some patients. Chronic HCV infection is the leading indication for liver transplantation in adults in the United States (Regev and Schiff, 2000). Fulminant hepatic failure occurs in a small number of cases of viral hepatitis, regardless of the etiology, and is associated with a high mortality rate.

Nursing ConsiderationsNursing objectives depend on the severity of the hepatitis, the medical management, and factors influencing the control and transmission of the disease. Children with benign viral hepatitis are frequently cared for at home, and the clinic or office nurse must explain the medical therapy and control measures. If further assistance is needed for parents to comply with therapy, a public health nursing referral may be necessary.

A well-balanced diet and a realistic schedule of rest and activity adjusted to the child's condition are encouraged. HAV is not infectious within a week after the onset of jaundice, and children may feel well enough to resume school. Parents are cautioned about administering any medication to the child, because normal doses of many drugs may become dangerous because of the liver's inability to detoxify and excrete them. Handwashing is the single most critical measure in reducing risk of transmission. The nurse should explain to parents and children the ways in which HAV (oral-fecal route) and HBV (parenteral route) are spread.

Children who are hospitalized are not usually isolated in a separate room unless they are fecally incontinent or their toys and other items become contaminated with feces. They are discouraged from sharing their toys. (For further discussion, see Infection Control, Chapter 22.)

Nurses who care for young people with HBV infection who have a known or suspected history of illicit drug use should help these teens to realize the dangers of drug abuse. Nurses should stress the parenteral mode of transmission of hepatitis, and encourage them to seek counseling through a drug program. HBV and HCV are chronic diseases that require frequent monitoring and management. Many communities have multidisciplinary clinics dedicated to the management of these diseases.

CIRRHOSISCirrhosis occurs at the end stage of many chronic liver diseases, including biliary atresia and chronic hepatitis. Cirrhosis can also result from infectious, autoimmune, or toxic factors and form chronic diseases such as hemophilia and cystic fibrosis. A cirrhotic liver is irreversibly damaged.

Clinical manifestations in children are similar to those seen with all chronic liver disorders. Children exhibit jaundice, poor growth, anorexia, muscle weakness, and lethargy. Ascites, edema, GI bleeding, anemia, and abdominal pain may be present with impaired intrahepatic blood flow. Pulmonary function may be impaired because of pressure against the diaphragm from hepatosplenomegaly and ascites. Dyspnea and cyanosis may occur, especially on exertion. Intrapulmonary arteriovenous shunts may develop and cause hypoxemia. Spider angiomas and prominent blood vessels are often present on the upper torso.

Therapeutic ManagementTherapy is directed toward (1) frequent assessment of liver status with physical examination and liver function tests and (2) management of specific complications. The only successful treatment for end-stage liver disease and liver failure may be liver transplantation, which has improved the prognosis substantially for many children with cirrhosis. Currently, the 1-year survival rate for liver transplantation is 85%. Increasing numbers of recipients are reaching their second decade after transplant. The increasing life span after transplantation is due to advances in surgical techniques, and improved preoperative, intraoperative, and postoperative care (Atkison and others, 2002).

Prognosis.Liver transplantation has revolutionized the approach to liver cirrhosis. Liver failure and cirrhosis are indications for transplantation. Liver transplantation reflects the failure of other medical and surgical measures to prevent or treat cirrhosis. Careful monitoring of the child's condition and quality of life are necessary to evaluate the need for and timing of transplantation (see Family Focus box).

FAMILY FOCUS: End-Stage Liver DiseaseIn many cases the child and family must cope with an uncertain progression of the disease. The only hope for long-term survival may be liver transplantation. Transplantation can be very successful, but the waiting period may be long, and there are many more children in need of organs than there are donors. The procedure is very expensive and is performed only at designated medical centers that are often far from the family's home. The nurse should recognize the unique stresses of coping with end-stage liver disease and waiting for transplantation and assist the family in coping with these stressors. The assistance of social workers and support from other parents can be very beneficial.

Nursing ConsiderationsNursing care of the child with cirrhosis is determined by the cause of the cirrhosis, the severity of complications, and the prognosis. The prognosis for life is poor unless successful liver transplantation occurs. Nursing care of this child is similar to that for any child with a life-threatening illness (see Chapter 18). Hospitalization is usually required when complications occur.

BILIARY ATRESIABiliary atresia, or extrahepatic biliary atresia (EHBA), is a progressive inflammatory process that causes both intrahepatic and extrahepatic bile duct fibrosis, resulting in obstructed bile flow. EHBA has been detected in 1 in 10,000 to 1 in 15,000 live births. The disorder is more common in girls and premature infants. In the United States, the incidence is twice as high in African Americans as in white infants, and more common in Chinese than in either Japanese or white populations. If untreated, EHBA usually leads to cirrhosis, liver failure, and death in the first 2 years of life.

Etiology/PathophysiologyThe exact cause of biliary atresia is unknown. Because EHBA has two distinct forms, postnatal and fetal/embryonic, different pathogenic mechanisms are suggested. Postnatal EHBA represents 65% to 90% of cases and is probably the result of infection or an immune-mediated mechanism. Direct hyperbilirubinemia first appears after

the resolution of physiologic jaundice. Histology demonstrates bile duct remnants and a progressive inflammatory process. In the fetal embryonic form, which represents 10% to 35% of cases, there is a congenital absence of biliary ductal patency and an absence of bile duct remnants. Many infants have associated congenital anomalies. The pathology of EHBA varies. Varying degrees of cholestasis occur, resulting in retention of irritants and toxins. Cholestasis is the accumulation of compounds that cannot be excreted because of occlusion or obstruction of the biliary tree. Injury to the liver occurs as the result of the inflammation caused by homeostasis.

Diagnostic EvaluationEarly diagnosis is the key to the survival of the child with EHBA. Infants who undergo surgery in the first 60 days of life have an 80% chance of establishing bile flow. Between 60 to 90 days of life, the chance of reestablishing flow drops to 50% and after 90 days to 10% (Sinatra, 2001). Growth parameters and nutritional status should be assessed, because many infants and children have nutritional deficiencies and poor growth. Several clinical signs may indicate the presence of EHBA (Box 24-10). Blood tests should include a CBC, electrolytes, bilirubin, and liver enzymes. Additional laboratory analyses include alpha1-antitrypsin level, TORCH titers (see Maternal Infections, Chapter 9), hepatitis serology, alpha-fetoprotein, urine cytomegalovirus, and a sweat test, which is indicated to rule out other conditions that cause persistent cholestasis and jaundice. Abdominal ultrasonography allows inspection of the liver and biliary system. Hepatobiliary scintigraphy demonstrates biliary patency but does not provide diagnostic certainty. Endoscopic retrograde cholangiopancreatography (ERCP) is performed in very young infants. This procedure, which is done under general anesthesia, has an 80% reported diagnostic accuracy. Percutaneous liver biopsy is highly reliable when the biopsy contains specimens from a number of portal areas. Definitive diagnosis of EHBA is obtained during surgical laparotomy and an intraoperative cholangiogram.

BOX 24-10 Clinical Manifestations of Extrahepatic Biliary Atresia

Jaundice

Earliest manifestation and most striking feature of disorder

First observed in sclera

May be present at birth

Usually not apparent until age 2 to 3 weeks

Urine dark and stains diaper

Stools lighter than expected or white or tan

Hepatomegaly and abdominal distention common

Splenomegaly occurs later

Poor fat metabolism results in:

Poor weight gain

General failure to thrive

Pruritus

Irritability

Difficult to comfort infant

Therapeutic ManagementThe primary treatment of biliary atresia is hepatic portoenterostomy (Kasai procedure) in which a segment of intestine is anastomosed to the resected porta hepatis to attempt bile drainage. Bile drainage is achieved in approximately 80% to 90% of infants who undergo surgery when younger than 10 weeks of age (Halamek and Stevenson, 1997). However, progressive cirrhosis still occurs in many children, and up to 80% to 90% eventually require liver transplantation (Andres, 1996). Prophylactic antibiotics are given following the Kasai procedure to minimize the risk of ascending cholangitis.

Medical management is primarily supportive. It includes nutritional support with infant formulas that contain medium-chain triglycerides and essential fatty acids. Supplementation with fat-soluble vitamins, multivitamin, and minerals, including iron, zinc, and selenium, is usually required. Aggressive nutritional support with continuous tube feedings or TPN is indicated for moderate to severe failure to thrive.

The enteral solution should be low in sodium. Ursodeoxycholic acid is used to treat pruritus and hypercholesterolemia.

Prognosis.Untreated biliary atresia results in progressive cirrhosis and death in most children by 2 years of age. The Kasai procedure improves the prognosis but is not a cure. Biliary drainage can often be achieved if the surgery is done before the intrahepatic bile ducts are destroyed. Long-term survival has been reported in children who receive the Kasai procedure; however, even with successful bile drainage, many children ultimately develop liver failure.

Advances in surgical techniques and the use of immunosuppressive and antifungal drugs have improved the success of transplantation. The major obstacle continues to be a shortage of donor livers. Reduced-sized, split-liver transplantation, retransplantation, and increased public awareness may improve donor organ availability in the future.

Nursing ConsiderationsNursing interventions for the child with biliary atresia include support of the family before, during, and after surgical procedures as well as education regarding the treatment plan. In the postoperative period of a portoenterostomy, nursing care is similar to that after major abdominal surgery. Family members need education relating to the proper administration of medications and nutritional therapy, including special formulas, vitamin and mineral supplements, tube feedings, or parenteral nutrition. Pruritus can often be relieved by drug therapy or comfort measures such as baths and trimming of fingernails.

Children and their families also need psychosocial support. The uncertain prognosis, discomfort, and waiting for transplantation produces stress, and hospitalizations, pharmacologic therapy, and nutritional therapy impose financial burdens on the family. Families can receive help from the Children's Liver Disease Foundation,* an organization that provides educational materials, programs, and support systems.

* 36 Great Charles Street, Birmingham, B33 JY, United Kingdom; (0121) 212-3839; fax: (0121) 212-4300; website: http://www.childliverdisease.org.

STRUCTURAL DEFECTS

CLEFT LIP OR CLEFT PALATEClefts of the lip (CL) and palate (CP) are facial malformations that occur during embryonic development, and are the most common congenital deformity of the head and neck. They may appear separately or, more often, together. CL results from failure of the maxillary and median nasal processes to fuse; CP is a midline fissure of the palate that results from failure of the two sides to fuse. This discussion is concerned primarily with cleft lip and palate (CL/P).

CL may vary from a small notch to a complete cleft extending into the base of the nose (Fig. 24-3). Clefts can be unilateral or bilateral. Deformed dental structures are associated with CL. CP alone occurs in the midline and may involve the soft and hard palates. When associated with CL, the defect may involve the midline and extend into the soft palate on one or both sides.FIG. 24-3

Variations in clefts of lip and palate at birth. A, Notch in vermilion border. B, Unilateral cleft lip and palate. C, Bilateral cleft lip and palate. D, Cleft palate.

CL/P is more common than CP and varies by ethnicity. The occurrence in whites is 1:1000 births; 1:500 in Native Americans and Asians, and 1:2000 in African Americans. CP occurs alone in only 1:2500 cases and does not display variation by ethnicity (Wilkins-Haug, 2003). Approximately 60% to 80% of children born with cleft lip and palate are male. Females have a higher frequency of isolated clefts of the secondary palate. Unilateral clefts are nine times more common than bilateral clefs and occur twice as frequently on the left side. Isolated bilateral CLs are uncommon; approximately 86% present with palatal clefts. Approximately 68% of unilateral CLs have an associated palatal cleft (Kirschner and LaRossa, 2000).

EtiologyCleft deformities may be an isolated anomaly or they may occur with a recognized syndrome. CL with or without CP is distinct from isolated CP. Clefts of the secondary palate alone are more likely to be associated with syndromes than isolated CL or CL and CP.

CL and CP may be caused by exposure to teratogens such as alcohol, anticonvulsants, and isotretinoin, but there is little evidence to link isolated clefts to any single teratogenic agent with the exception of phenytoin. Use of phenytoin during pregnancy is associated with a 10-fold increase in the incidence of CL. The incidence of CL among mothers who smoke during pregnancy is twice as great as the incidence in mothers who do not smoke during pregnancy (Kirschner and LaRossa, 2000).

PathophysiologyCleft deformities represent a genetic defect in cell migration that results in a failure of the maxillary and premaxillary processes to come together between the third and twelfth week of embryonic development. Although often appearing together, CL and CP are distinct malformations embryologically, occurring at different times during the developmental process. Merging of the upper lip at the midline is completed between the seventh and eleventh weeks of gestation. Fusion of the secondary palate (hard and soft palate) takes place later, between the seventh and twelfth weeks of gestation. In

the process of migrating to a horizontal position, they are separated by the tongue for a short time. If there is delay in this movement, or if the tongue fails to descend soon enough, the remainder of development proceeds but the palate never fuses.

Diagnostic EvaluationCL with or without CP is apparent at birth. The defect elicits severe emotional reactions in parents. CP is less obvious than CL and may not be detected without a thorough assessment of the mouth. CP is identified when the examiner places a finger directly on the palate. Clefts of the hard palate form a continuous opening between the mouth and the nasal cavity. The severity of the CP has an impact on feeding; the infant is unable to generate negative pressure and create suction in the oral cavity. This impairs feeding, even though in most cases the infant's ability to swallow is normal.

Prenatal diagnosis with fetal ultrasound is not reliable until the soft tissues of the fetal face can be visualized at 13 to 14 weeks. The sensitivity of fetal ultrasound for facial clefting is almost 100% when CL/P is associated with other structural anomalies. In isolated CL/P, sensitivity may be 50% with CP; an intact lip is the most difficult to diagnose prenatally (Wilkins-Haug, 2003).

Therapeutic ManagementTreatment of the child with CL is surgical and involves no long-term interventions other than possible scar revision. The management of CP involves the cooperative efforts of a multidisciplinary health care team, including pediatrics, plastic surgery, orthodontics, otolaryngology, speech/language pathology, audiology, nursing, and social work. Management is directed toward closure of the cleft(s), prevention of complications, and facilitation of normal growth and development in the child. Until recently repair of cleft deformities in the neonate was not considered safe. Surgery is now possible in younger neonates because of advance in pediatric anesthesiology and neonatology. However, the infant must be free of any oral, respiratory, or systemic infections. In deformities of both the lip and palate, the palate is repaired first to avoid disrupting the lip after it has been repaired.

Surgical correction: CL. The two most common procedures for repair of CL are the and the Millard rotational advancement

technique. The difference between these two is the Tennison-Randall procedure crosses the philtral line and the Millard procedure advances a triangle of tissue in the upper third of the lip and does not cross the midline. Surgeons often use a combination of these two techniques to address individual differences. Improved surgical techniques have minimized scar retraction, and in the absence of infection or trauma, healing occurs with little scar formation. Optimum cosmetic results, however, are difficult to obtain in severe defects. Additional revisions may be necessary at a later age.

Surgical correction: CP. CP repair was previously postponed until a later age than the repair of the CL to take advantage of palatal changes that take place with normal growth. With advanced surgical and anesthesia techniques, many surgeons are currently performing palatal repairs in the neonatal period (Sandber, Magee, and Denk, 2002). The timing of repair remains controversial. Most surgeons prefer to close the cleft before the child develops faulty speech habits.

Prognosis.Even with good anatomic closure, most children with CL/P have some degree of speech impairment that requires speech therapy. Physical problems result from inefficient functioning of the muscles of the soft palate and nasopharynx, improper tooth alignment, and varying degrees of hearing loss. Improper drainage of the middle ear, as a result of inefficient function of the eustachian tube, contributes to recurrent otitis media with scarring of the tympanic membrane, which leads to hearing impairment in many children with CP. Upper respiratory infections require immediate and meticulous attention, and extensive orthodontics and prosthodontics may be needed to correct problems of malposition of teeth and maxillary arches.

Long-term problems are related to social adjustment of the child. The better the physical care, the better is the chance for emotional and social adjustment, although the presence of the defect and the degree of residual disability are not always directly related to a satisfactory adjustment. Physical defects are a threat to the self-image, and abnormal speech quality is an impediment to social expression.


AssessmentThe lip defect is visible at birth, and assessment involves describing the location and extent of the defect; the CP is estimated by visualization during crying. CP without CL is detected by palpating the palate with the finger during the newborn assessment. The emotional impact of the birth of a child with a cosmetic and functional disability is especially traumatic to the family. Consequently, nursing assessment is also concerned with the emotional reaction of the family.

Nursing DiagnosesBased on a thorough physical assessment, a number of nursing diagnoses are evident and described in the Nursing Care Plan on pp. 875–876.

PlanningThe goals of care are related to preoperative care, short-term postoperative care, and long-term management. Goals for the infant and family include:

Preoperative care:

1. Family will cope with the impact of an infant with a defect.

2. Infant will receive optimum nutrition.

3. Infant will be prepared for surgery.

Postoperative care:

1. Infant will experience no trauma and minimal or no pain.

2. Infant will receive optimum nutrition.

3. Infant will experience no complications.

4. Infant and family will receive adequate support.

5. Family will be prepared for care at home and long-term needs of a child with CP.

ImplementationThe immediate nursing problems for an infant with CL/P deformities are related to feeding and dealing with the parental reaction to the defect. Facial deformities are particularly disturbing to parents. CL is a visible defect that may produce a strong negative response in parents, which could influence maternal-infant attachment. However, a study of infants with CL or CP indicated that maternal-infant attachment was not negatively affected when measured at 1 year of age (Speltz and others, 1997). During the initial phase following birth, it is important for the nurse to place emphasis not only on the infant's physical needs, but also on the parents' emotional needs (Speltz and others, 1997). The manner of handling the infant should convey to the parents that the infant is a precious human being. (See Chapter 18 for interventions in assisting parents in accepting a birth defect.) Throughout the course of therapy, parents need explanations of the immediate and long-range problems associated with CP. They may be unaware that more is involved than repairing the defect. Whenever possible, they should be referred to a comprehensive CP team.

Feeding.Feeding the infant presents a challenge to nurses and parents. Growth failure in infants with CL or CP has been attributed to preoperative feeding difficulties. After surgical repair, most infants who have isolated CL or CP with no associated syndromes, gain weight or achieve adequate weight and height for age (Lee, Nunn, and Wright, 1997).

CL or CP reduces the infant's ability to suck making bottle-feeding and breast-feeding difficult. In breast-feeding the CL or CP interferes with compression of the areola. Liquid taken into the mouth escapes via the cleft through the nose. Feeding is best accomplished with the infant's head in an upright position, either held in the caregiver's hand or cradled in the arm. Normal nipples are unsuitable for these infants, who are unable to generate the suction required. A variety of special "cleft palate" nipples have been devised and used with some success. However, large, soft nipples with large holes; Nursettes; or the long, soft lamb's nipples appear to offer the best means for nipple feeding (Fig. 24-4). The newer "gravity flow" nipple* attached to a squeezable plastic bottle allows formula to be deposited directly into the mouth in the

same manner as with a bulb syringe. Success has also been achieved by modification of a standard nipple. A single small slit or crosscut is made in the end of the nipple with a sharp surgical blade or pair of sharp thin scissors. The enlarged opening, which can be adjusted to the infant's individual needs, allows the infant to swallow the formula easily bypassing the suction problem.FIG. 24-4

Some devices used to feed an infant with a cleft lip and palate. (Photo by Paul Vincent Kuntz, Texas Children's Hospital.)

The ESSR feeding technique also works well with these infants. The steps in ESSR are: 1) enlarge the nipple; 2) stimulate the suck reflex; 3) swallow fluid appropriately; and 4) rest when the infant signals with facial expression. Infants fed with the ESSR method revealed a significantly greater increase in their mean weight before surgery than infants fed with traditional methods (Richard, 1994).

Using special or modified nipples or feeding techniques help to meet the infant's sucking needs. Muscle development is important for later development of speech. During feeding, the nipple is positioned so that it is compressed by the infant's tongue and existing palate. If a single-slit nipple is used, the slit is placed

vertically so that the infant will be able to produce and stop a flow of milk by alternately opening and closing the opening. No matter which type of nipple is used, gentle, steady pressure on the base of the bottle reduces the chance of choking or coughing. The person feeding should resist the temptation to remove the nipple because of the noise the infant makes or for fear that the infant will choke. These infants swallow excessive amounts of air, and they require frequent burping.

When the infant has trouble with nipple feeding, a rubber-tipped medicine dropper, Asepto syringe, or Breck feeder (a large syringe with soft rubber tubing) provides an efficient, safe feeding device. The rubber extension should be long enough to extend back into the mouth to reduce the likelihood of regurgitation through the nose. The formula is deposited on the back of the tongue, and the flow is controlled by bulb or syringe compression that is adjusted to the infant's needs. With some infants, spoon feeding works best, especially if the formula is slightly thickened with cereal. After feeding, the infant is given water to rinse the mouth.

Breast-feeding is also an option. The nipple is positioned and stabilized well back in the oral cavity so that tongue action facilitates milk expression. The suction required to stimulate milk may be absent initially, and a breast pump may be useful before nursing to stimulate the let-down reflex.

Regardless of the feeding method used, the mother should begin feeding the infant as soon as possible, preferably after the initial nursery feeding. When maternal feeding is initiated early, the mother can help to determine the method best suited to her and the infant and can become adept in the technique before discharge from the hospital.

* Ross Laboratories, 625 Cleveland Avenue, Columbus, OH 43216.

Preoperative Care.In preparation for surgical repair, parents are frequently taught to accustom the infant to the needs of the early postoperative period, especially if surgery is delayed for several months. It is mandatory for the infant to be positioned on the back or side postoperatively. Most infants tolerate these positions well because they are accustomed to being supine for sleeping. It is also helpful

to place the infant or child in arm restraints periodically before admission and to feed the infant with a rubber-tipped Asepto syringe or other device that will be used postoperatively.

Postoperative Care: CL.The major efforts in the postoperative period are directed toward protecting the operative site. After CL repair (cheiloplasty), a metal appliance or adhesive strips are securely taped to the cheeks to relax the surgical site and prevent tension on the suture line caused by crying or other facial movement. Elbow restraints are used to prevent the infant from rubbing or disturbing the suture line and are applied immediately after surgery. It is advisable to pin the cuff of the restraints to the infant's clothing to keep the restraints in place. Older infants who roll over will require a jacket restraint in addition to restricting arm movement to prevent rolling on the abdomen and rubbing the face on the sheet, especially if the repair involves the lip. It is important to remove the elbow restraints periodically to exercise the arms, to provide relief from restrictions, to observe the skin for signs of irritation, and to provide an opportunity for cuddling and body contact. Restraints should be released one at a time. Sitting the infant in an infant seat provides a change of position and a different view of the environment. Adequate analgesia is required to relieve postoperative pain and to provide restlessness.

Clear liquids are offered when the infant has fully recovered from the anesthesia, and feeding is resumed when tolerated. The suture site is carefully cleansed of formula or serosanguineous drainage as needed with a cotton-tipped swab dipped in saline. A thin layer of antibiotic ointment may be prescribed for application to the suture line after cleansing. Meticulous care of the suture line is essential because inflammation or infection will interfere with optimum healing and the ultimate cosmetic effect of the surgical repair. Gentle aspiration of mouth and nasopharyngeal secretions may be necessary to prevent aspiration and respiratory complications. An upright or infant seat position is helpful in the immediate postoperative period (especially for infant who has difficulty in handling secretions).

Postoperative Care: CP.

The child with CP repair (palatoplasty) is allowed to lie on the abdomen immediately postoperatively. The child may resume feeding by bottle, breast, or cup shortly after surgery.

! NURSING ALERTAvoid the use of suction or other objects in the mouth, such as tongue depressors, thermometers, spoons, or straws.

Oral packing may be secured to the palate after palatoplasty; this packing is usually removed after 2 to 3 days. Sometimes the infant will have difficulty breathing following surgery, because it is often necessary to alter an established pattern of breathing and adjust to breathing through the nose. This is frustrating but seldom requires more than positioning and support. The elbows may be restrained to keep the child's hands away from the mouth. Parents are instructed to maintain elbow restraints at home until the palate is healed, usually in 4 to 6 weeks. They are instructed to remove the restraints (one at a time) at frequently to allow the child to exercise the arms.

The nurse must assess the infant or child's level of postoperative pain. Opioids may be prescribed initially, and acetaminophen may be given as needed thereafter.

The older infant or child may be discharged on a blenderized or soft diet, and parents are instructed to continue the diet until the surgeon directs them otherwise. Parents are cautioned against allowing the child to eat hard items (such as toast, hard cookies, and potato chips) that can damage the repaired palate.

Long-Term Care.Children with CL/P often require a variety of services during recovery. Family members need support and encouragement by health professionals and guidance in activities that facilitate a normal outcome for their child. Financial stress is frequently cited as a difficult issue by parents. With the combined efforts of the family and the health team, most children achieve a satisfactory outcome. Many children with CL/P have surgical correction that creates a near-normal-appearing lip and permits good function. Parents need to understand the function of therapy and the

purpose and care of all appliances, as well as the importance of establishing good mouth care and proper brushing habits.

Throughout the child's development, an important goal is the development of a healthy personality and self-esteem. Many communities have CP parents' groups that offer help and support to families. Agencies that provide services and information for children with CL/P and their families include the American Cleft Palate Association, The Cleft Palate Foundation, the Association of Birth Defect Children, the March of Dimes-Birth Defects Foundation, and the various state Children's Medical Services.

EvaluationTo determine the effectiveness of nursing intervention, continual reassessment and evaluation of care is based on the following observational guidelines:

Preoperative care:

1. Observe and interview family members about their understanding, feelings, and concerns regarding the defect, any anticipated surgery and their interactions with the infant.

2. Observe infant during feeding.

3. Complete preoperative checklist.

Postoperative care:

1. Inspect operative site, including the protective device.

2. Observe for behavioral and physiologic indicators of pain and response to analgesics.

3. Observe infant during feeding, measure intake and output, and weigh infant daily.

4. Observe operative site for evidence of infection, bleeding, sloughing, or irritation.

5. Observe and interview family regarding their understanding and concerns about the infant, including long-term needs.

The expected outcomes are described in the Nursing Care Plan on pp. 875–876.

NURSING CARE PLAN: The Child With Cleft Lip or Cleft PalatePREOPERATIVE CARE

NURSING DIAGNOSIS: Altered nutrition: less than body requirements related to physical defect

PATIENT GOAL 1: Will consume adequate nourishment


Administer diet appropriate for age (specify)

Assist mother with breast-feeding if this is mother's preference, because the newborn with either defect can breast-feed

Position and stabilize nipple well back in oral cavity so that tongue action facilitates milk expression

Stimulate let-down reflex manually or with breast pump before nursing, because suction required to stimulate milk may be absent initially

Modify feeding techniques to adjust to defect, because infant's ability to suck is reduced

Hold child in upright (sitting) position to minimize risk of aspiration

Use special feeding appliances that compensate for infant's feeding difficulty

Try to feed infant using nipple to meet infant's need for sucking and to promote muscle development for speech

Position nipple between infant's tongue and existing palate to facilitate compression of nipple

When using devices without nipples (e.g., Breck feeder, Asepto syringe), deposit formula on back of tongue to facilitate swallowing and adjust flow according to infant's swallowing to prevent aspiration

Bubble (burp) frequently because of tendency to swallow excessive amounts of air

Encourage parents to begin feeding infant as soon as possible so that they become adept in feeding technique before discharge

Monitor weight to assess adequacy of nutritional intake

EXPECTED OUTCOMES

Infant consumes an adequate amount of nutrients (specify amount)

Infant exhibits appropriate weight gain

NURSING DIAGNOSIS: Risk for altered parenting related to infant with a highly visible physical defect

PATIENT (FAMILY) GOAL 1: Will demonstrate acceptance of infant


Allow expression of feelings to encourage family's coping

Convey attitude of acceptance of infant and family because parents are sensitive to affective attitudes of others

Indicate by behavior that child is a valuable human being to encourage acceptance of infant

Describe results of surgical correction of defect

Use photographs of satisfactory results to encourage feeling of hope

Arrange meeting with other parents who have experienced a similar situation and coped successfully

EXPECTED OUTCOMES

Family discusses feelings and concerns regarding child's defect, its repair, and future prospects

Family exhibits an attitude of acceptance of infant

See also Nursing Care Plan: The Child Undergoing Surgery, Preoperative Care, Chapter 22

POSTOPERATIVE CARE

NURSING DIAGNOSIS: Risk for trauma of the surgical site related to surgical procedure, dysfunctional swallowing

PATIENT GOAL 1: Will experience no trauma to operative site


Position on back or side or in infant seat (cleft lip [CL]) to prevent trauma to operative site

Maintain lip-protective device (CL) to protect the suture line

Use nontraumatic feeding techniques to minimize risk of trauma

Restrain elbows to prevent access to operative site

Use jacket restraints on older infant to prevent rolling onto abdomen and rubbing face on sheet

Avoid placing objects in the mouth after cleft palate (CP) repair (suction catheter, tongue depressor, straw, pacifier, small spoon) to prevent trauma to operative site

Prevent vigorous and sustained crying, which can cause tension on sutures

Cleanse suture line gently after feeding and as necessary in manner ordered by surgeon (CL), because inflammation or infection will interfere with healing and the cosmetic effect of surgical repair

Teach cleansing and restraining procedures, especially when infant will be discharged before suture removal, to minimize complications after discharge

EXPECTED OUTCOME

Operative site remains undamaged

PATIENT GOAL 2: Will exhibit no evidence of aspiration


Position to allow for drainage of mucus (partial side-lying position, semi-Fowler position) and to prevent aspiration of formula

EXPECTED OUTCOME

Child manages secretions and formula without aspiration

NURSING DIAGNOSIS: Altered nutrition: less than body requirements related to difficulty eating following surgical procedure

PATIENT GOAL 1: Will consume adequate nourishment


Monitor intravenous fluids (if prescribed)

Administer diet appropriate for age and as prescribed for postoperative period (specify)

Involve family in determining best feeding methods, because family assumes feeding responsibility at home

Modify feeding techniques to adjust to defect and surgical repair

Feed in sitting position to minimize risk of aspiration

Use special appliances that compensate for feeding difficulties without causing trauma to operative site

Bubble frequently because of tendency to swallow large amounts of air

Assist with breast-feeding if method of choice

Teach feeding and suctioning techniques to family to ensure optimum home care

EXPECTED OUTCOMES

Infant consumes an adequate amount of nutrients (specify amounts)

Family demonstrates ability to carry out postoperative care

Infant exhibits appropriate weight gain

NURSING DIAGNOSIS: Pain related to surgical procedure

PATIENT GOAL 1: Will experience optimum comfort level


Assess behavior and vital signs for evidence of pain

*Administer analgesics or sedatives as ordered

Remove restraints periodically while supervised to exercise arms, provide relief from restrictions, and observe skin for signs of irritation

Provide cuddling and tactile stimulation and other nonpharmacologic interventions as needed for optimum comfort

Involve parents in infant's care to provide comfort and sense of security

EXPECTED OUTCOME

Infant appears comfortable and rests quietly

NURSING DIAGNOSIS: Altered family processes related to child with a physical defect, hospitalization

PATIENT (FAMILY) GOAL 1: Will receive adequate support

NURSING INTERVENTIONS/RATIONALES AND EXPECTED OUTCOMES

Refer family to appropriate agencies and support groups

See Nursing Care Plan: The Family of the Ill or Hospitalized Child, Chapter 21


ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULACongenital atresia of the esophagus and tracheoesophageal fistula (TEF) are rare malformations that are believed to result from failed separation of the esophagus and trachea by a septum that forms by the fourth week of gestation. These defects occur as separate entities or in combination (Fig. 24-5). They have a fatal outcome without early diagnosis and treatment.FIG. 24-5 Five most common types of esophageal atresia and tracheoesophageal fistula.

EtiologyEsophageal atresia (EA) with or without an associated TEF is the most common esophageal malformation. It occurs in 1:2,000 to 1:5,000 live births. There appears to be an equal sex incidence, but the birth weight of most affected infants is significantly lower than average, and there is an unusually high incidence of prematurity in infants with EA. A history of maternal polyhydramnios is present in approximately 50% of infants with the defects. EA/TEF is often present with the VATER or VACTERL syndromes. VATER and VACTERL are acronyms that describe the associated anomalies. These syndromes involve a combination of vertebral, anorectal, cardiovascular, tracheoesophageal, renal, and limb abnormalities. The cardiac anomalies occur most frequently with EA/TEF.

PathophysiologyThe cause of EA/TEF is unknown. In the most frequently encountered form of esophageal atresia and TEF (80% to 95% of cases), the

proximal esophageal segment terminates in a blind pouch, and the distal segment is connected to the trachea or primary bronchus by a short fistula at or near the bifurcation (Fig. 24-5, C ) . The second most common variety (5% to 8%) consists of a blind pouch at each end, widely separated and with no communication to the trachea (Fig. 24-5, A ) . Less frequently, an otherwise normal trachea and esophagus are connected by a common fistula (Fig. 24-5, E ) . Extremely rare anomalies involve a fistula from the trachea to the upper esophageal segment (Fig. 24-5, B ) or to both the upper and the lower segments (Fig. 24-5, D ) .

Diagnostic EvaluationThe disorder is suspected on the basis of clinical manifestations (Box 24-11). EA should also be suspected in cases of maternal polyhydramnios. Although the diagnosis is established on the basis of clinical signs and symptoms, the exact type of anomaly is determined by radiographic studies. A radiopaque catheter is inserted into the hypopharynx and advanced until it encounters an obstruction. Chest films are taken to ascertain esophageal patency or the presence and level of a blind pouch. Sometimes fistulas are not patent, which makes their presence more difficult to diagnose. The presence of gas in the stomach or small bowel is indicative of a coexisting TEF.

BOX 24-11 Clinical Manifestations of Tracheoesophageal Fistula

Excessive salivation and drooling

Three Cs of tracheoesophageal fistula (TEF)

Coughing

Choking

Cyanosis

Apnea

Increased respiratory distress after feeding


Therapeutic ManagementEA is a surgical emergency. The treatment includes maintenance of a patent airway, prevention of pneumonia, gastric or blind pouch decompression and surgical repair of the anomaly. When EA/TEF is suspected, the infant is immediately taken off oral intake, started on IV fluids, and placed in the position least likely to cause aspiration of either mouth or stomach secretions. Removal of secretions from the mouth and upper pouch requires frequent or continuous suction. Because aspiration pneumonia is almost inevitable and appears early, broad-spectrum antibiotic therapy is often instituted.

Primary surgical correction consists of a thoracotomy with division and ligation of the TEF and an end-to-side anastomosis of the esophagus. This may consist of one operation or be staged with two or more procedures. For infants who are premature, have multiple anomalies, or are in very poor condition, a staged procedure is preferred that involves palliative measures, including gastrostomy, ligation of the TEF, and provision of constant drainage of the esophageal pouch. A delayed esophageal anastomosis is usually attempted after several weeks to months when the upper pouch elongates. Further surgical techniques may be performed later to facilitate esophageal lengthening. If an esophageal anastomosis still cannot be accomplished, a cervical esophagostomy (to allow drainage of saliva) and gastrostomy are performed.

A primary anastomosis may be impossible because of insufficient length of the two segments of the esophagus. In these cases, an esophageal replacement procedure using a part of the colon or gastric tube interposition may be necessary to bridge the missing esophageal segment. Endotracheal intubation may be required, because many infants (10% to 20%) also have tracheomalacia, a weakness in the tracheal wall that occurs when a dilated proximal pouch compresses the trachea in early fetal life or when the trachea does not develop normally because of a loss of intratracheal pressure.

Complications of a primary repair include an anastomotic leak, strictures resulting from tension or ischemia, esophageal motility disorders causing dysphagia, and gastroesophageal reflux.

Prognosis.

The prognosis is related to the birth weight, associated congenital anomalies, and time of diagnosis. The survival rate is nearly 100% in full-term infants without severe respiratory distress or other anomalies. In premature low-birth-weight infants with associated anomalies, the incidence of complications is high. The overall mortality is 50% (Ryckman, Flake, and Balistreri, 1997).

Nursing ConsiderationsNursing responsibility for detection of this malformation begins immediately after birth. Ideally, the diagnosis should be made before the initial feeding, but often it is not. If fed, the infant swallows normally but suddenly coughs and struggles, and the fluid is aspirated or returns through the nose and mouth. For this reason, it is customary for the nurse to give the infant the first feeding of plain water or to be present when a parent feeds the child to observe the infant's response. Early breast-feeding should not be prevented unless there is a strong suspicion of EA.

! NURSING ALERTAny infant who has an excessive amount of frothy saliva in the mouth or difficulty with secretions and unexplained episodes of cyanosis should be suspected of having an EA/TEF, and referred immediately for medical evaluation.

Cyanosis is usually the result of laryngospasm caused by overflow of saliva into the larynx from the proximal esophageal pouch. It normally clears after removal of the secretions from the oropharynx by suctioning. Any suspicion of TEF is reported immediately. The infant is placed in an incubator or a radiant warmer, and oxygen is administered to help relieve respiratory distress. Intubation and assisted mechanical ventilation may be necessary if the infant is in respiratory distress. When a newborn is suspected of having a TEF, the most desirable position is supine with the head elevated on an inclined plane of at least 30 degrees. This position minimizes the reflux of gastric secretions up the distal esophagus into the trachea and bronchi.

It is imperative that the source of aspiration be removed at once. Oral fluids are withheld, and the infant's fluid needs are met parenterally or via gastrostomy. Until surgery the blind pouch is kept empty by intermittent or continuous suction through an indwelling nasal

catheter that extends to the end of the pouch. The catheter needs attention because it has a tendency to become clogged with mucus. It is usually replaced daily by the practitioner. In the event that a staged repair is performed, a gastrostomy tube is inserted and left open so that air entering the stomach through the fistula can escape, thus minimizing the danger that gastric contents will be regurgitated into the trachea. The tube empties by gravity drainage. Feedings through the gastrostomy tube and irrigations with fluid are contraindicated before surgery in the infant with a distal TEF. Nursing interventions include respiratory assessment, airway management, thermoregulation, fluid and electrolyte management, and potential nutritional support.

Postoperative Care.Postoperative care is essentially the same as the care of any high-risk newborn (see Nursing Care of the High-Risk Newborn and Family, Chapter 9). The infant is returned to the radiant heater, and the gastrostomy tube is connected to gravity drainage until the infant can tolerate feedings. At this time, the tube is elevated and secured at a point above the level of the stomach. This allows gastric secretions to pass to the duodenum and swallowed air can escape through the open tube. Tracheal suction should only be done using a premeasured catheter and with extreme caution to avoid injury to the suture line. If tolerated, gastrostomy feedings may be started and continued until the esophageal anastomosis is healed. Before oral feedings are initiated and the chest tube is removed a contrast study or esophagram is performed to verify the integrity of the esophageal anastomosis.

The initial attempt at oral feeding must be carefully observed to make sure that the infant can swallow without choking. Oral feedings are begun with sterile water, followed by frequent small feedings of formula. Until the infant can take a sufficient amount by mouth, gastrostomy feedings or parenteral nutrition may supplement oral intake. Infants are usually not discharged until they are taking oral fluids well and the gastrostomy tube is removed. However, the infant who has palliative surgery will be discharged with the gastrostomy tube in place. The nurse is responsible for making certain that the caregiver is educated and has practiced the care of the gastrostomy (see Chapter 22).

Special Problems.

Upper respiratory complications are a threat to life in both the preoperative and the postoperative period. In addition to pneumonia, there is a constant danger of respiratory distress resulting from atelectasis, pneumothorax, and laryngeal edema. Any persistent respiratory difficulty after removal of secretions is reported to the surgeon immediately. The infant is monitored for anastomotic leaks, as evidenced by purulent chest tube drainage, increased WBC count, and temperature instability.

In the infant awaiting esophageal replacement surgery, the catheter is removed and the upper esophageal segment is drained through a cervical esophagostomy. An esophagostomy is difficult to care for because the skin becomes irritated by moisture from the continuous discharge of saliva. Frequent removal of drainage and application of a layer of protective ointment may remedy the problem. A dressing or ostomy appliance may be applied to collect the drainage, and an enterostomal therapist can provide additional guidance to prevent or treat skin breakdown.

For the infant who requires esophageal replacement, nonnutritive sucking is provided by a pacifier. Sometimes small amounts of water or formula are given orally, and although the liquid drains from the esophagostomy, this process allows the infant to develop mature sucking patterns. Other appropriate oral stimulation prevents feeding aversions. Infants who remain NPO for an extended period or who have not received oral stimulation have difficulty with eating by mouth after corrective surgery and may develop oral hypersensitivity and food aversion. They require patient, firm guidance to learn how to take food into the mouth and swallow after repair. A referral to a multidisciplinary feeding behavior program is often necessary.

As with any congenital anomaly, parents need support in adjusting to the child's condition (see Chapter 18). One difficulty is the immediate transfer of the sick newborn to the intensive care unit and the length of hospitalization. Encouraging parents to visit the infant, participate in care when appropriate, and express their feelings regarding the infant's condition facilitates the attachment process. The nurse in the intensive care unit should assume responsibility for ensuring that the parents are kept fully informed of the infant's progress.

Preparing parents for discharge involves teaching them skills that they will need at home. Parents are taught to observe for behaviors

that indicate the need for suctioning as well as signs of respiratory distress, and constriction of the esophagus (e.g., poor feeding, dysphasia, drooling, regurgitation of undigested food). Discharge planning also includes obtaining the necessary equipment and home nursing services to provide home care.

HERNIASA hernia is a protrusion of a portion of an organ or organs through an abnormal opening. The danger from herniation arises when the organ protruding through the opening is constricted to the extent that circulation is impaired or when the protruding organs encroach on and impair the function of other structures. A hernia that cannot be reduced easily is called an incarcerated hernia. A strangulated hernia is one in which the blood supply to the herniated organ is impaired. The herniations of concern are those that protrude through the diaphragm, the abdominal wall, or the inguinal canal (see also Genitourinary Tract Disorders/Defects, Chapter 27). The other hernias of significance to the pediatric age groups are outlined in Table 24-5.

TABLE 24-5 Summary Outline of Hernias

OBSTRUCTIVE DISORDERSObstruction in the GI tract occurs when the passage of nutrients and secretions is impeded by a constricted or occluded lumen or when there is impaired motility (paralytic ileus). Obstructions may be congenital or acquired. Many congenital obstructions such as atresia, imperforate anus, meconium plug and meconium ileus usually appear in the neonatal period. Other obstructions of congenital etiology such as

malrotation, Hirschsprung disease, volvulus, incarcerated hernia, Meckel diverticulum appear after the first few weeks of life. Intestinal obstruction from acquired causes such as intussusception, pyloric stenosis, and tumors may occur in infancy or childhood. Intestinal obstructions from any cause are characterized by similar signs and symptoms (Box 24-12).

BOX 24-12 Clinical Manifestations of Mechanical/Paralytic Intestinal Obstruction

Colicky abdominal pain—From peristalsis attempting to overcome the obstruction

Abdominal distention—As a result of accumulation of gas and fluid above the level of the obstruction

Vomiting—Often the earliest sign of a high obstruction; a later sign of lower obstruction (may be bilious or feculent)

Constipation and obstipation—Early signs of low obstructions; later signs of higher obstructions

Dehydration—From losses of large quantities of fluid and electrolytes into the intestine

Rigid and boardlike abdomen—From increased distention

Bowel sounds—Gradually diminish and cease

Respiratory distress—Occurs as the diaphragm is pushed up into the pleural cavity

Shock—Plasma volume diminishes as fluids and electrolytes are lost from the bloodstream into the intestinal lumen

Sepsis—Caused by bacterial proliferation with invasion into the circulation

HYPERTROPHIC PYLORIC STENOSISHypertrophic pyloric stenosis (HPS) occurs when the circumferential muscle of the pyloric sphincter becomes thickened, resulting in elongation and narrowing of the pyloric channel. This produces an outlet obstruction and compensatory dilation, hypertrophy, and

hyperperistalsis of the stomach. This condition usually develops in the first few weeks of life, causing projectile vomiting, dehydration, metabolic alkalosis, and failure to thrive. The precise etiology is unknown. There is a genetic predisposition, and siblings and offspring of affected persons are at increased risk of developing HPS. Firstborn children and males are affected five times more frequently than females. HPS is more common in full-term than in premature infants, and it is seen less frequently in African American and Asian infants than in Caucasian infants.

PathophysiologyThe circular muscle of the pylorus thickens as a result of hypertrophy (increased size) and hyperplasia (increased mass). This produces severe narrowing of the pyloric canal between the stomach and the duodenum causing partial obstruction of the lumen (Fig. 24-6, A ) . Over time, inflammation and edema further reduce the size of the opening resulting in complete obstruction. The hypertrophied pylorus may be palpable as an olive-like mass in the upper abdomen. Pyloric stenosis is not a congenital disorder. Evidence suggests that local innervation is involved in the pathogenesis. In most cases this is an isolated lesion; however, it may be associated with intestinal malrotation, esophageal and duodenal atresia, and anorectal anomalies.FIG. 24-6

Hypertrophic pyloric stenosis. A, Enlarged muscular area nearly obliterates pyloric channel. B, Longitudinal surgical division of muscle down to submucosa establishes adequate passageway.

Diagnostic EvaluationThe diagnosis of HPS is often made after the history and physical examination. The olive-like mass is easily palpated when the stomach is empty, the infant is quiet, and the abdominal muscles are relaxed. Vomiting usually occurs 30 to 60 minutes after feeding, and becomes projectile as the obstruction progresses. Emesis is nonbilious usually consisting of stale milk. Often these infants become dehydrated and lethargic and appear significantly malnourished.

If the diagnosis is inconclusive from the history and physical signs (Box 24-13), ultrasonography will demonstrate an elongated, sausage-shaped mass with an elongated pyloric channel. If ultrasound fails to demonstrate a hypertrophied pylorus, then upper GI radiography should be done to rule out other causes of vomiting. Laboratory findings reflect the metabolic alterations created by severe depletion of both fluid and electrolytes from extensive and prolonged vomiting. There are decreased serum levels of both sodium and potassium, although these may be masked by the hemoconcentration from ECF depletion. Of greater diagnostic value is a decrease in serum chloride levels and increases in pH and bicarbonate (carbon dioxide content) characteristic of metabolic alkalosis. The BUN level will be elevated as evidence of dehydration.

BOX 24-13 Clinical Manifestations of Hypertrophic Pyloric Stenosis

Projectile vomiting

May be ejected 3 to 4 feet from the child when in a side-lying position, 1 foot or more when in a back-lying position

Occurs shortly after a feeding (may not occur for several hours)

May follow each feeding or appear intermittently

Nonbilious vomitus; may be blood-tinged

Infant hungry, avid nurser; eagerly accepts a second feeding after vomiting episode

No evidence of pain or discomfort except that of chronic hunger

Weight loss

Signs of dehydration

Distended upper abdomen

Readily palpable olive-shaped tumor in the epigastrium just to the right of the umbilicus

Visible gastric peristaltic waves that move from left to right across the epigastrium

Therapeutic ManagementSurgical relief of the pyloric obstruction by pyloromyotomy (sometimes called Fredet-Ramstedt procedure) is the standard treatment for this disorder. The procedure is performed through a right upper quadrant incision (laparotomy) and consists of a longitudinal incision through the circular muscle fibers of the pylorus down to, but not including, the submucosa (Fig. 24-6, B ) . The procedure has a high success rate when infants receive careful preoperative preparation to correct fluid and electrolyte imbalances.

Feedings are usually begun 4 to 6 hours postoperatively, beginning with small, frequent feedings of glucose water or electrolyte solutions. If clear fluids are retained, about 24 hours after surgery, formula is started in stepwise increments, with the amount and interval between feedings gradually increased until a full feeding schedule is reinstated (usually over a 48 hour period). The infant is discharged from the hospital by about the second or third postoperative day.

Another procedure, laparoscopy, may be performed for infants with HPS. The use of a small incision for the laparoscope results in shorter surgical time, more rapid postoperative feeding, and quicker discharge.

Prognosis.Most infants recover completely and rapidly after pyloromyotomy. Postoperative complications include persistent pyloric obstruction and wound dehiscence. Some infants also have GER.


The diagnosis of HPS is considered in the very young infant who appears alert but fails to gain weight and has a history of vomiting after meals. Assessment is based on observation of eating behaviors and evidence of other characteristic clinical manifestations.

Preoperative Care.Preoperatively the emphasis is placed on restoring hydration and electrolyte balance. Infants are usually given no oral feedings and receive IV fluids with glucose and electrolyte replacement based on laboratory serum electrolyte values. Careful monitoring of the IV infusion and diligent attention to intake, output, and urine specific gravity measurements are important. Vomiting and the number and character of stools are observed and recorded accurately.

Observations also include assessment of vital signs, particularly those that might indicate fluid or electrolyte imbalances. These infants are prone to metabolic alkalosis from loss of hydrogen ions and to potassium, sodium, and chloride depletion. The skin and mucous membranes as well as daily weight are assessed for alterations in hydration status and water gain or loss.

If stomach decompression and gastric lavage are used preoperatively, the nurse is responsible for ensuring that the tube is patent and functioning properly and for measuring and recording the type and amount of drainage. The infant is usually positioned flat or with the head slightly elevated. Infants who are receiving IV fluids and/or have a nasogastric tube for continuous drainage must be observed to prevent the needle or tube from becoming dislodged.

General hygienic care, with attention to the skin and mouth in dehydrated infants, is essential. Protection from infection is also important, because infants with impaired nutritional status are more susceptible than normal newborns. Parental involvement is encouraged and promoted.

Postoperative Care.Postoperative vomiting may occur, and most infants, even with successful surgery, exhibit some vomiting during the first 24 to 48 hours. IV fluids are administered until the infant can retain adequate amounts by mouth. Observation of physical signs, monitoring of IV

fluids, careful observation, and recording of intake and output is maintained. The infant is also observed for evidence of pain, and appropriate analgesics are given. The nasogastric tube may be maintained after surgery for a variable time.

Feedings are usually instituted soon after surgery, beginning with clear liquids containing glucose and electrolytes. They are offered slowly, in small amounts, and at frequent intervals as ordered by the practitioner. If the infant has been breast-fed, breast milk, expressed by the mother, is given by bottle when the infant is able to tolerate feedings. Breastfeeding is resumed as soon as feasible. Observation and recording of feedings and the infant's responses to feedings are a vital part of postoperative care. Positioning with the head elevated is usually continued postoperatively. Care of the operative site consists of observation for any drainage or signs of inflammation and care of the incision as directed by the surgeon.

Parents are encouraged to remain with their child and become involved in the child's care. Vomiting of a projectile nature is frightening to parents, and they often believe that they may have done something wrong or that surgery was not successful. Most parents need support and reassurance that the condition is caused by a structural problem and is in no way a reflection on their parenting skills and capacities.

INTUSSUSCEPTIONIntussusception is one of the most frequent causes of intestinal obstruction in children between the ages of 3 months and 3 years. The peak occurrence is between the ages of 5 and 9 months (Brandt, 1999). Intussusception is more common in males than in females and in children with cystic fibrosis. Although specific intestinal lesions can be found in a small percentage of these children, the cause is usually not known. More than 90% of intussusceptions do not have a pathologic lead point, such as a polyp, lymphoma, or Meckel diverticulum. The idiopathic cases are most likely a result of hypertrophy of intestinal lymphoid tissue secondary to viral infection.

PathophysiologyIntussusception occurs when a proximal segment of the bowel telescopes into a more distal segment, pulling the mesentery with it. The mesentery is compressed and angled, resulting in lymphatic and

venous obstruction. As edema from the obstruction increases, pressure within the area of intussusception increases. When the pressure equals the arterial pressure, arterial blood flow stops, resulting in ischemia and the pouring of mucus into the intestine. Venous engorgement also leads to leaking of blood and mucus into the intestinal lumen, forming the classic currant jelly stools (Brandt, 1999). The most common site is the ileocecal valve (ileocolic), where the ileum invaginates into the cecum and colon (Fig. 24-7). Other forms include ileoileal (one part of the ileum invaginates into another section of the ileum), and colocolic (one part of the colon invaginates into another area of the colon) usually in the area of the hepatic or splenic flexure or at some point along the transverse colon.FIG. 24-7 Ileocolic intussusception.

! NURSING ALERTThe classic signs (e.g., severe colicky abdominal pain in a child with vomiting, currant jelly stools) may not be present. A more chronic picture, characterized by diarrhea, anorexia, weigh loss, occasional vomiting, and periodic pain, may be present. Because intussusception is potentially life-threatening, the nurse should be aware of alternate presentations and observe these children closely and refer them for further evaluation.


Frequently the diagnosis can be made on subjective findings alone (Box 24-14). However, definitive diagnosis is based on a barium enema, which clearly demonstrates the obstruction to the flow of barium. Initially, however, an abdominal radiograph is obtained to detect intraperitoneal air from a bowel perforation, which would contraindicate a barium enema. A rectal examination reveals mucus, blood, and, occasionally, a low intussusception itself.

BOX 24-14 Clinical Manifestations of Intussusception

Sudden acute abdominal pain

Child screams and draws the knees onto the chest

Child appears normal and comfortable during intervals between episodes of pain

Vomiting

Lethargy

Passage of red, currant jelly-like stools (stool mixed with blood and mucus)

Tender, distended abdomen

Palpable sausage-shaped mass in upper right quadrant

Empty lower right quadrant (Dance sign)

Eventual fever, prostration, and other signs of peritonitis

Therapeutic ManagementIn most cases the initial treatment of choice is nonsurgical hydrostatic reduction traditionally by barium enema. In this procedure, correction of the invagination is carried out at the same time as the diagnostic testing. The force exerted by the flowing barium is usually sufficient to push the invaginated portion of the bowel into its original position, similar to pushing an inverted "finger" out of a glove. This procedure is not recommended if there are clinical signs of shock or perforation.

The use of barium as the contrast agent is becoming less routine, and a high percentage of radiologists use water-soluble contrast and air pressure to reduce intussusceptions. The increased use of water-soluble contrast reflects concern regarding the risk of barium peritonitis. The administration of air pressure to reduce intussusception is as successful as, and more rapid than, barium without the risk of peritonitis, and with decreased exposure to radiation. Some use carbon dioxide instead of air. It has the advantage of being absorbed by the gut and is associated with less discomfort. It also eliminates the risk of an air embolism. IV fluids, nasogastric decompression, and antibiotic therapy may be used before hydrostatic reduction is attempted. If these procedures are not successful, the child may require surgical intervention. Surgery involves manually reducing the invagination and, when indicated, resecting any nonviable intestine.

Prognosis.Nonoperative reduction is successful in approximately 75% of cases. Surgery is required for patients in whom the contrast enema is unsuccessful. With early diagnosis and treatment, serious complications and death are uncommon.

Nursing ConsiderationsThe nurse can help establish a diagnosis by listening to the parent's description of the child's physical and behavioral symptoms. It is not unusual for parents to express that they felt something was seriously wrong before others shared their concerns. The description of the child's severe colicky abdominal pain combined with vomiting is a significant sign of intussusception.

As soon as a possible diagnosis of intussusception is made, the nurse prepares the parents for the immediate need for hospitalization, the nonsurgical technique of hydrostatic reduction, and the possibility of surgery. It is important to explain the basic defect of intussusception. A model of the defect is easily demonstrated by pushing the end of a finger on a rubber glove back into itself or using the example of a telescoping rod. The principle of reduction by hydrostatic pressure can be simulated by filling the glove with water, which pushes the "finger" into a fully extended position.

Physical care of the child does not differ from that for any child undergoing abdominal surgery. Even though nonsurgical intervention may be successful, the usual preoperative procedures, such as withholding of fluids by mouth, routine laboratory testing (CBC and urinalysis), signed parental consent, and preanesthetic sedation, are performed. For the child with signs of electrolyte imbalance, hemorrhage, or peritonitis, additional preparation, such as replacement fluids, whole blood or plasma, and nasogastric suctioning may be needed. Before surgery the nurse monitors all stools.

! NURSING ALERTPassage of a normal brown stool usually indicates that the intussusception has reduced itself. This is immediately reported to the practitioner, who may choose to alter the diagnostic/therapeutic plan of care.

Postprocedural care includes observations of vital signs, blood pressure, intact sutures and dressing, and the return of bowel sounds. After hydrostatic reduction or autoreduction, the nurse observes for passage of barium or water-soluble contrast material and the stool patterns, because there may be recurrences of the intussusception. Children may be admitted to the hospital or monitored on an outpatient basis. A recurrence of intussusception is treated with hydrostatic reduction, but a laparotomy is considered for multiple recurrences.

Because hospitalization may be the child's first separation from the parents, it is important to preserve the parent-child relationship by encouraging rooming-in or extended visiting. It may be the parents' first experience with hospitalization, necessitating their preparation for procedures such as IV therapy, frequent vital sign and blood pressure monitoring, dressings, and special orders, such as NPO. Because of the rapidity of the onset, diagnosis, and treatment, parents may feel stunned or numb. They may ask few questions, or they may constantly make inquiries, sometimes the same ones several times. If the nurse realizes the circumstances surrounding this condition, the parents' reactions are more likely to be understood and accepted.

MALROTATION AND VOLVULUS

Malrotation of the intestine is due to the abnormal rotation of the intestine around the superior mesenteric artery during embryologic development. Malrotation may present in utero or may be asymptomatic throughout life. Infants with malrotation have intermittent vomiting, recurrent abdominal pain, distention, or lower GI bleeding. Malrotation is the most serious type of intestinal obstruction, because if the intestine undergoes complete volvulus (the intestine twisting around itself), compromise of the blood supply will result in intestinal necrosis, peritonitis, perforation, and death.

Diagnostic EvaluationIt is imperative that malrotation and volvulus be diagnosed promptly and surgical treatment instituted quickly. Any infant with bilious vomiting should be evaluated carefully for malrotation, volvulus, and obstruction. An upper GI series is the definitive procedure to diagnose this condition.

Therapeutic ManagementSurgery is indicated to remove the affected area. Because of the extensive nature of some lesions, short-gut syndrome is a postoperative complication.

Nursing ConsiderationsPreoperatively the nursing care is the same as that provided to an infant/child with intestinal obstruction. Postoperatively the nursing care is similar to that provided to the infant/child who has undergone abdominal surgery.

ANORECTAL MALFORMATIONSAnorectal malformations include a number of anomalies of the genitourinary and pelvic organs. These malformations are among the more common congenital malformations caused by abnormal development, with an incidence of 1 in 2000 to 5000 live births (Hendren, 1998). The anus and rectum originate from an embryologic structure called the cloaca. Lateral growth of the cloaca forms the urorectal septum that separates the rectum dorsally from the urinary tract ventrally. The rectum and urinary tract separate completely by

the seventh week of gestation. Anomalies that occur reflect the stage of development of these processes.

Imperforate anus includes several forms of malformation without an obvious anal opening. Many have a fistula from the distal rectum to the perineum or genitourinary system. Anorectal malformations may occur in isolation or as part of the VACTERL or VATER syndromes.

A persistent cloaca is a complex anorectal malformation in which the rectum, vagina, and urethra drain into a common channel that opens onto the perineum via the usual urethral site. Cloacal exstrophy is a rare, severe defect in which there is externalization of the bladder and bowel through the abdominal wall. Often the genitalia are indefinite, and chromosome studies are necessary to determine the child's sex. Gender assignment is almost always female. The exstrophic bladder is separated into two halves by the cecum; other features may include an omphalocele, imperforate anus, and, at times, a neural tube defect. With improved surgical techniques, survival rates for this condition are 88% to 90% in some centers (Smith and others, 1997).

Anorectal anomalies are classified according to sex and abnormal anatomic features, including genitourinary and associated pelvic anomalies. The level of rectal descent is determined by the relationship of the termination of the bowel to the puborectalis sling of the levator ani musculature. Anorectal malformations are classified according to sex and the level of the malformation (high, intermediate, and low) (Table 24-6). About 50% of children with anorectal anomalies have a urologic problem.

TABLE 24-6 Classification of Anorectal Malformations

From Stephens FD and others: Pediatr Surg Int 1:200, 1986.

Diagnostic EvaluationChecking for patency of the anus and rectum is a routine part of the newborn assessment and should include observations regarding the passage of meconium. Inspection of the perineal area reveals absence of the normal anal opening; however, the appearance of the perineum alone does not accurately predict the level of the lesion. Genitourinary and pelvic anomalies associated with anorectal malformations should be considered.

In the newborn the presence of meconium on the perineum does not always indicate anal patency (particularly in girls), because a fistula may be present and allow evacuation of meconium through the vagina. Fistulas may not be apparent at birth but may become obvious as peristalsis gradually forces the meconium through the fistula. Rectourinary fistulas should be suspected if there is meconium in the urine. Anal stenosis may not be identified until the child is older and presents with a history of difficult defecation, abdominal distention, and ribbonlike stools.

Abdominal ultrasound is performed to determine the existence of other malformations. An intravenous pyelogram (IVP) and voiding cystourethrogram are recommended for the infant with a high malformation to identify anomalies of the urinary tract. Further examination is also indicated when there is evidence of urinary tract infection or other symptoms. If a syndrome is suspected, cardiac evaluation and spinal films should be obtained.

Therapeutic ManagementSuccessful treatment for anal stenosis is generally accomplished by manual dilations. The procedure is initiated by a physician, and repeated on a regular basis by the nurses in the hospital. Parents are taught to continue the dilatations at home. Perineal fistulas are treated by anoplasty during the newborn period. The opening is moved to the center of the external sphincter and dilatations are begun. More extensive defects are usually managed with a colostomy and corrective surgical repair performed later in the first year (Pena, 2000).

The type of defect, the sacral anatomy, and the quality of muscles influence the long-term prognosis. In general, if the newborn presents with a deep midline groove, two well-formed buttocks, and an anal

dimple, the prognosis for bowel control is better than if the infant has a flat or "rocker" bottom and no midline groove because of associated neurologic problems (Flake and Ryckman, 1997). A functioning interior anal sphincter is important to achieve continence. In its absence, the child may need a bowel program to achieve socially acceptable bowel continence. Other potential complications after surgical treatment include strictures, recurrent rectourinary fistula, mucosal prolapse, and constipation.

Nursing ConsiderationsThe first nursing responsibility is identification of undetected anorectal malformations. A poorly developed anal dimple, a genitourinary fistula, or vertebral abnormalities suggest a high lesion. A newborn who does not pass a stool within 24 hours of birth requires further assessment. In addition, meconium that appears at an inappropriate orifice is reported. Preoperative care includes diagnostic evaluation, GI decompression, and IV fluids.

Nursing care after an anorectoplasty is directed toward healing the surgical site without infection or complications. Care involves keeping the anal area as clean as possible with scrupulous perineal care. A temporary dressing and drain may be placed initially to manage the continuous passage of stool. Protective ointments such as zinc oxide and occlusive dressings such as hydrocolloids decrease skin irritation from frequent loose stools. The preferred position is a side-lying prone position with the hips elevated or a supine position with the legs suspended at a 90-degree angle to the trunk to prevent pressure on perineal sutures.

There may be a nasogastric tube for abdominal decompression and IV feedings. The infant is given formula when normal peristalsis is noted. Care of the infant with a colostomy involves frequent dressing changes, meticulous skin care, and correct application of a collection device (see Chapter 22).

Family Support, Discharge Planning, and Home Care.Long-term follow-up is important for children with high malformations. After the definitive pull-through procedure, toilet training is delayed, and complete continence is seldom achieved at the usual age of 2 to 3 years of age. Prevention of constipation is

important, and breast-feeding is encouraged postoperatively. If a cow's milk-based formula is used, a laxative may be prescribed. Bowel habit training, diet modification, and administration of stool softeners or fiber are important aspects of bowel management. Optimum bowel function may not be achieved until late childhood or adolescence. Support and reassurance are important during the slow progression to normal function.

Parents are instructed in perineal and wound care or care of the colostomy. Anal dilations may be necessary for some infants. Parents are advised to observe stooling patterns and notify the physician if there are any signs of anal stricture or complications.

MALABSORPTION SYNDROMEChronic diarrhea and malabsorption of nutrients characterize malabsorption syndromes. An important complication of malabsorption syndromes in children is failure to thrive. Most cases are classified according to the location of the supposed anatomic or biochemical defect. The term celiac disease is often used to describe a symptom complex with four characteristics: (1) steatorrhea (fatty, foul, frothy, bulky stools), (2) general malnutrition, (3) abdominal distention, and (4) secondary vitamin deficiencies.

Digestive defects are conditions in which the enzymes necessary for digestion are diminished or absent, such as (1) cystic fibrosis, in which pancreatic enzymes are absent; (2) biliary or liver disease, in which bile flow is affected; or (3) lactase deficiency, in which there is congenital or secondary lactose intolerance.

Absorptive defects are conditions in which the intestinal mucosal transport system is impaired. This may occur because of a primary defect (e.g., celiac disease) or secondary to inflammatory disease of the bowel that results in impaired absorption because bowel motility is accelerated (e.g., ulcerative colitis). Obstructive disorders (e.g., Hirschsprung disease) also cause secondary malabsorption from enterocolitis.

Anatomic defects, such as extensive resection of the bowel or "short-bowel syndrome," affect digestion by decreasing the transit time of substances and affect absorption by severely compromising the absorptive surface.

CELIAC DISEASECeliac disease (CD), also known as gluten-induced enteropathy, gluten-sensitive enteropathy (GSE), and celiac sprue, is a disease of the proximal small intestine characterized by abnormal mucosa and permanent intolerance to gluten. CD is second only to cystic fibrosis as a cause of malabsorption in children. The age that this condition first appears and its prevalence have changed over the past 30 to 40 years. Celiac sprue used to be considered a disease of childhood, but adult presentation is becoming more common. It is seen more frequently in Europe than in America and is rarely reported in Asians or blacks. Although previous figures suggested that CD affected 1 in 6000 individuals, recent studies suggest the prevalence is more likely to be 1 in 250 individuals (American Gastroenterological Association Medical Position Statement, 2001). The exact cause of CD is unknown, but there appears to be an inherited predisposition with an influence by environmental factors.

PathophysiologyThe disease is characterized by intolerance to the protein gluten, found in wheat, barley, rye, and oats. Although the pathologic process is still obscure, susceptible individuals are unable to digest the gliadin component of gluten, resulting in an accumulation of a toxic substance that is damaging to the mucosal cells.

Diagnostic EvaluationSymptoms of CD are usually noted several months after the introduction of gluten-containing grains into the diet, typically between the ages of 1 and 5 years (Box 24-15). The clinical manifestations are usually insidious and chronic. The first evidence may be failure to thrive and diarrhea.

BOX 24-15 Clinical Manifestations of Celiac DiseaseIMPAIRED FAT ABSORPTION

Steatorrhea (excessively large, pale, oily, frothy stools)

Exceedingly foul-smelling stools

IMPAIRED ABSORPTION OF NUTRIENTS

Malnutrition

Muscle wasting (especially prominent in legs and buttocks)

Anemia

Anorexia


BEHAVIORAL CHANGES

Irritability

Fretfulness

Uncooperativeness

Apathy

CELIAC CRISIS*

Acute, severe episodes of profuse watery diarrhea and vomiting

May be precipitated by:

Infections (especially gastrointestinal)

Prolonged fluid and electrolyte depletion

Emotional disturbance

The diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of mucosal inflammation, crypt hyperplasia, and villous atrophy (Dieterich, Esslinger, and Schuppan, 2003). Within a day or two of instituting the diet, most children with CD demonstrate a favorable response, including weight gain and improved appetite. Within a few weeks there is resolution of the diarrhea and steatorrhea.

Serologic testing to detect antibodies to connective tissue (endomysium and reticulin) and to gliadin are available. The presence of antigliadin, antireticulin, and antiendomysial IgG and IgA antibodies (and the disappearance of these antibodies when gluten is

removed from the diet), aids in diagnosis (Walker-Smith, 1996). The autoantibodies of IgA antireticulin and IgA antiendomysial are more specific markers for active celiac disease than circulating IgA antigliadin antibodies, which may be present in other diseases and conditions. A more specific test is the enzyme tissue transglutaminase (tTG) which has been found to be the autoantigen recognized by antiendomysial antibody (Walker-Smith and others, 2000). Although these markers are useful in the diagnosis and screening of CD, they vary in sensitivity and specificity and are usually followed by a biopsy to confirm the diagnosis (Murray, 1999).

* In very young children.

Therapeutic ManagementTreatment of chronic CD is primarily dietary. Although the diet is called "gluten free," it is actually low in gluten, because it is impossible to remove every source of this protein. Studies indicate that most patients can tolerate restricted amounts of gluten. Because gluten is found primarily in the grains of wheat and rye, but also in smaller quantities in barley and oats, these four foods are eliminated. Corn and rice become substitute grain foods.

Children with untreated celiac disease may have associated lactose intolerance related to intestinal mucosal lesions, which usually improve with gluten withdrawal and intestinal healing. Specific nutritional deficiencies are treated with appropriate supplements, including vitamins, iron, and calories.

Prognosis.CD is regarded as a chronic disease. Its extent varies among children. The most severe symptoms usually occur in early childhood and again in adult life. Strict dietary avoidance of gluten prevents symptoms and may minimize the risk of developing lymphoma, the most serious complication of the disease.

Nursing ConsiderationsThe main nursing consideration is helping the child adhere to dietary management. Considerable time is involved in explaining to the child and the parents the disease process, the specific role of gluten in aggravating the condition, and those foods that must be restricted. It

is especially difficult to maintain a diet indefinitely when the child has no symptoms and temporary transgressions result in no difficulties. However, evidence indicates that most individuals who relax their diet experience a relapse of their disease and possibly exhibit growth retardation, anemia, or osteomalacia. There is also the risk of developing malignant lymphoma of the small intestine or other GI malignancies.

Although the chief source of gluten is cereal and baked goods, grains are frequently added to processed foods as thickeners or fillers. Gluten is also added to many foods as "hydrolyzed vegetable protein." The nurse must advise parents to read carefully all ingredients on labels to avoid hidden sources of gluten. Although the chief source of gluten is cereal and baked goods, grains are frequently added to processed foods as thickeners or fillers. Gluten is also added to many foods as "hydrolyzed vegetable protein." The nurse must advise parents to read carefully all ingredients on labels to avoid hidden sources of gluten.In addition to restricting gluten, other dietary alterations may be necessary initially. For example, in some children who have more severe mucosal damage, disaccharide digestion is impaired, especially lactose. Therefore, these children often need a temporary lactose-free diet, which necessitates eliminating all milk products.

Generally, management includes a diet high in calories and proteins, with simple carbohydrates, such as fruits and vegetables, but low in fats. Initially the bowel may be inflamed, as a result of the pathologic process, so high-fiber foods, such as nuts, raisins, raw vegetables, and raw fruits with skin, are avoided until inflammation has subsided.

Several organizations and resources are available to help families cope with this condition. The Celiac Sprue Association/United States of America* is an organization that provides support, guidance, and educational materials to families concerning a gluten-free diet, food sources, recipes, and travel information. There are also several published cookbooks that contain gluten-free recipes†.

* PO Box 31700, Omaha, NE 68131-0700; (402) 558-0600; e-mail: [email protected]. In Canada: Canadian Celiac Association, Inc., 190 Britannia Road East, Unit 11, Mississauga, Ontario, L4Z 1W6; (905) 507-6208; website: http://www.celiac.ca.† A booklet, Pointers for parents: coping with celiac sprue, provides information on shopping, cooking, and living with an affected child, and is available from the Clinical Dietetics Department, Children's

Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614; (773) 880-4793.

SHORT BOWEL SYNDROMEShort bowel syndrome (SBS) is a malabsorptive disorder that occurs when there is decreased mucosal surface area, usually as a result of extensive resection of the small intestine. The most common causes of SBS in children include congenital anomalies (jejunal and ileal atresia, gastroschisis), ischemia (necrotizing enterocolitis), and trauma or vascular injury (volvulus [twisting of bowel on itself]). Other causes include volvulus that results in massive resection, long-segment Hirschsprung disease, and omphalocele. The prognosis for infants and children with SBS has dramatically improved in the past 25 years as a result of advances in parenteral nutrition and enteral feeding. Both the amount and the location of bowel lost are important in determining the severity of the condition. The preservation of the terminal ileum and ileocecal valve influences fluid and nutrient absorption and may avoid problems of bacterial overgrowth by preventing the entrance of bacteria from the colon into the small intestine.

The small intestine has significant capacity for adaptation after resection. During the adaptation process, the villus height increases (villus hyperplasia), and the cell number and absorptive surface area are also increased. As villus length and the number of enterocytes available for absorption per centimeter of bowel increase, nutrient absorption increases. Intraluminal enteral feedings stimulate the adaptation process and maintain the structural and functional integrity of the small intestine.

Therapeutic ManagementThe goals of treatment are (1) to preserve as much length of bowel as possible during surgery; (2) to maintain the child's nutritional status, growth, and development while intestinal adaptation occurs; (3) to stimulate intestinal adaptation with enteral feeding; and (4) to minimize complications related to the disease process and therapy.

Nutritional support is the long-term focus of care. The initial phase of therapy includes TPN as the primary source of nutrition. Complications associated with SBS and long-term TPN include central venous catheter infection or occlusion, catheter migration,

thrombosis or emboli, bacterial overgrowth, metabolic complications, cholestasis, and liver dysfunction (see Chapter 22).

The second phase is the introduction of enteral feeding, which usually begins as soon as possible after surgery. Elemental formulas containing glucose, sucrose and glucose polymers, hydrolyzed proteins, and medium-chain triglycerides facilitate absorption. Usually these formulas are given by continuous infusion through a nasogastric or gastrostomy tube. As the enteral feedings are advanced, the TPN solution is decreased in terms of calories, amount of fluid, and total hours of infusion per day.

The final phase of nutritional support occurs when growth and development are sustained exclusively by enteral feedings. When TPN is discontinued, there is a risk of nutritional deficiency secondary to malabsorption of fat-soluble vitamins (A, D, E, K), and trace minerals (iron, selenium, zinc). Serum vitamin and mineral levels should be obtained, and enteral supplementation of vitamins and minerals may be required. Pharmacologic agents have been used to reduce secretory losses. H2 blockers, proton pump inhibitors, and octreotide inhibit gastric or pancreatic secretion. Cholestyramine is often prescribed to improve diarrhea that is associated with bile salt malabsorption. Growth factors have also been used to hasten adaptation and to enhance mucosal growth, but these uses are still experimental.

Numerous complications are associated with SBS and long-term TPN (see Chapter 22). Infectious, metabolic, and technical complications can occur. Catheter sepsis can occur after improper care of the catheter. The GI tract can also be a source of microbial seeding of the catheter. Bowel atrophy may foster increased intestinal permeability of bacteria. A lack of adequate sites for central lines may become a significant problem for the child in need of long-term TPN. Hepatic dysfunction, hepatomegaly with abnormal liver function tests, and cholestasis may also occur.

Bacterial overgrowth is likely to occur when the ileocecal valve is absent or when stasis exists as a result of a partial obstruction or a dilated segment of bowel with poor motility. Alternating cycles of broad-spectrum antibiotics are used to reduce bacterial overgrowth. This treatment may also decrease the risk of bacterial translocation and subsequent central venous catheter infections. Other complications of bacterial overgrowth and malabsorption include metabolic acidosis and gastric hypersecretion.

Many surgical interventions, including intestinal valves, tapering enteroplasty or stricturoplasty, intestinal lengthening, and interposed segments have been used to slow intestinal transit, reduce bacterial overgrowth, or increase mucosal surface area. Intestinal transplantation has been performed successfully in children. However, the experience is limited and the long-term results are unknown. Only children with a permanent dependence on TPN or severe complications of long-term parenteral nutrition are candidates for transplantation.

Prognosis.The prognosis for infants with SBS has improved with advances in TPN and with the understanding of the importance of intraluminal nutrition. Improved surgical techniques for the management of therapy-related problems and the development of more specific immunosuppressive medications for transplantation have all contributed to improved management. The prognosis depends in part on the length of the residual small intestine. An intact ileocecal valve also improves the prognosis. Infants and children with SBS usually die of TPN-related problems, such as fulminant sepsis or severe TPN cholestasis.

Nursing ConsiderationsThe most important components of nursing care are administration and monitoring of nutritional therapy. During TPN therapy, care must be taken to minimize the risk of complications related to the central venous access device (i.e., catheter infections, occlusions, dislodgment, or accidental removal). Care of the enteral feeding tubes and monitoring of enteral feeding tolerance are also important nursing responsibilities.

When long-term parenteral nutrition is required, preparing the family for home care is a major nursing responsibility that should be initiated early to prevent a lengthy hospitalization with subsequent problems such as family dysfunction and developmental delays. Many infants and children can be successfully cared for at home with enteral and parenteral nutrition when the family is prepared and provided with adequate support services. Follow-up by a multidisciplinary nutritional support service is essential. The nurse plays an active and important role in the success of a home nutrition program. Home infusion companies provide portable equipment,

which enables the child and family to maintain a more normal lifestyle.

When hospitalization is prolonged, the child's developmental and emotional needs must be met. This often requires special planning to promote normal family adjustment and adaptation of the hospital routines. Care of the hospitalized child is discussed in Chapter 21.

KEY POINTS• Infants are subject to fluid depletion because of their greater surface area relative to body mass, high rate of metabolism, and immature kidney function.

• Dehydration can be classified as isotonic, hypotonic, and hypertonic.

• Vomiting and diarrhea account for significant fluid depletion, especially in infants and small children.

• The amount, frequency, and characteristics of stool and vomitus are important nursing observations.

• Diarrhea can be caused by an inflammatory process of infectious origin, a toxic reaction to ingestion of poisonous substances, dietary indiscretions, or infections outside the alimentary tract. The primary treatment of diarrhea is the use of oral rehydrating solution.

• Hirschsprung disease requires surgical removal of aganglionic segments of bowel.

• Postoperative care of the child with abdominal surgery involves assessing the abdomen, providing hydration and nutrition, intravenous fluids, proper positioning, wound care, and psychologic support.

• Nursing care of gastrointestinal (GI) reflux is aimed at identifying children with suggestive symptoms, helping parents with home care feeding and positioning, and caring for the child undergoing surgical intervention.

• Although the cause of appendicitis is poorly understood, it is typically a result of obstruction of the lumen, usually by a fecalith. Common signs and symptoms are right lower quadrant abdominal pain, tenderness, and fever.

• Meckel diverticulum is a congenital malformation of the GI tract characterized by bloody stools.

• Inflammatory bowel disease refers to ulcerative colitis and Crohn disease. Chronic diarrhea is the most common feature. It is treated by dietary management and medication, although surgery is needed in some cases.

• Peptic ulcers are poorly understood, but contributing factors include interference with the normal protective mechanisms of the mucosal lining and the presence of Helicobacter pylori.

• Viral hepatitis is caused by six types of virus: hepatitis A virus, hepatitis B virus, hepatitis C virus, hepatitis D virus, hepatitis E virus, and hepatitis G virus.

• Hepatitis A virus is spread by the fecal-oral route, whereas hepatitis B and C viruses are transmitted primarily by the parenteral route. The most effective measure in prevention and control of hepatitis in any setting is handwashing.

• Structural disorders of the GI tract include cleft lip, cleft palate, esophageal atresia with tracheoesophageal fistula, anorectal malformations, and biliary atresia.

• Biliary atresia is a serious disorder, often causing progressive liver failure, which is an indication for liver transplantation.

• Cleft lip and palate, the most common facial malformation, may involve nutritional, dental, and speech problems.

• Hernias related to the GI tract can be minor (umbilical) or life-threatening (diaphragmatic, gastroschisis, omphalocele).

• General signs of obstruction include colicky abdominal pain, nausea and vomiting, abdominal distention, and decreased stool output.

• Hypertrophic pyloric stenosis is recognized by characteristic projectile vomiting, malnutrition, dehydration, and a palpable mass in the epigastrium and is relieved by pyloromyotomy.

• Intussusception is one of the most common causes of intestinal obstruction during infancy and is characterized by abdominal pain and

blood in stools. Treatment is either nonsurgical hydrostatic reduction or surgical reduction.

• Malabsorption syndromes are disorders associated with some degree of impaired digestion or absorption. They include digestive defects, absorptive defects, and anatomic defects.

• Celiac disease is characterized by an intolerance to gluten. It is thought to be either an inborn error of metabolism or an immunologic response.

• Short-bowel syndrome is characterized by a loss of intestine resulting in a diminished ability to absorb a regular diet normally. Specialized enteral and parenteral nutrition is a major element of care for these children.

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