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COMMON GASTROINTESTINALPROBLEMS AND EMERGENCIES IN

NEONATES AND CHILDREN

Jeffrey M. Halter, MD, Thomas Baesl, MD,Linda Nicolette, MD, and Michael Ratner, MD

The large number of infants and children who are seen in physiciansoffices, urgent care centers, and hospital emergency rooms for signs andsymptoms related to an abdominal etiology attests to the fact that this is anexceedingly common problem. Among this large group of patients is asignificant number that have serious surgical diagnoses. This includes anumber of disorders, many of which are uncommon. In addition, there aremany common and uncommon neonatal surgical problems that are seen inthe newborn nursery and are referred to a neonatal intensive care unit and

pediatric surgical specialist. This article concentrates on a few of the mostcommon problems that we encounter in our practice and discusses a fewof the potentially devastating congenital anomalies that may appear later ininfancy and childhood. Our intent is to give a more in-depth discussion ofeach of these topics and to have the points we make more easilyincorporated into daily practice. Much of the material comes from the ourexperience but is also based on recent literature. Where controversyexists, we endeavor to include the best evidence-based practices.

APPENDICITIS

Background

Since Fitz described appendicitis in 1886 [1] and McBurney deliveredhis classic paper in 1889 [2] describing McBurneys Point, the diagnosis of

GASTROENTEROLOGY 15225720/04 $15.00 + .00

From the Department of Surgery, SUNY Upstate Medical University, Syracuse, New York(JMH); and the Department of Surgery, Division of Pediatric Surgery, SUNY UpstateMedical University, Syracuse, New York (TB, LN, MR)

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appendicitis has remained controversial. In our experience, approximately25% of the time the diagnosis is obvious and straightforward. In roughly25% of patients, the diagnosis requires a careful history and physical

examination and some basic laboratory studies. In the last approximately50% of patients, the diagnosis can be difficult to make accurately. This lastgroup includes the very young (ie, those under 5 years old); patients whohave been symptomatic for more than 2 or 3 days; adolescent girls aged 13to 18; and a group of patients in whom the history, physical examination,and laboratory studies do not fit the usual picture of appendicitis. Imagingstudies are necessary in this last group of 50% of cases, and exploratorylaparotomy or diagnostic laparoscopy may also be required to make anaccurate diagnosis.

History and Physical Examination

Symptoms of appendicitis usually begin with abdominal pain, often inthe periumbilical area. Classically, after 6 to 18 hours from onset, the painmigrates to McBurneys point, defined as the point two thirds of thedistance from the umbilicus along a straight line toward the anteriorsuperior iliac spine of the pelvis. In most cases, the pain steadily worsensuntil the appendix perforates. At that time, the localized pain maydecrease, but the more generalized pain of peritonitis takes over. Anorexia

is the next most common symptom, occurring in about 95% of patients.Nausea is seen in approximately 75% of cases, and vomiting is seen in 60%to 70% of patients. Diarrhea, or more accurately tenesmus, may occur but isusually seen late in the clinical course of appendicitis when the sigmoidcolon becomes irritated by a severely inflamed or perforated appendix.

About 75% of patients have a low-grade fever around 38C (100.4F), but ahigher fever or the absence of fever should not eliminate the diagnosis ofappendicitis [3].

The physical examination to rule in or rule out appendicitis beginswhen the patient walks into the providers office or emergency department.Many patients exhibiting this type of abdominal symptomatology walk in asomewhat bent over (flexed abdomen) or guarded fashion, giving the

provider valuable information regarding the potential for peritonealirritation. We generally begin the abdominal portion of the examinationby asking the patient to cough. If this maneuver causes pain in the rightlower quadrant, this is considered to be a positive sign for peritonealirritation. The rapid release of the hand on the abdomen after deep

palpation as a test for rebound tenderness is almost uniformly worthless inchildren because virtually all children respond by jumping with thismaneuver. Palpating the abdomen softly allows the provider to comparethe right- and left-sided rectus abdominus muscles and lets the evaluatorknow whether or not there is rectus rigidity. The location of the maximaltenderness at McBurneys point is helpful. Rovsings sign, or referredtenderness from the left lower quadrant to the right lower quadrant during

palpation, is a significant sign of appendicitis. The psoas sign, elicited by

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extending the hip posteriorly with the patient lying prone, and theobturator sign, elicited by abducting the right hip with the patient lyingsupine, have not been helpful. Auscultation of bowel sounds is generally

not considered to be helpful in examining a child for potential appendicitisbecause bowel sounds tend to be hypoactive or absent once peritonitisoccurs after appendiceal perforation. The rectal examination in a case ofsuspected appendicitis does not provide much useful information unlessthere is a pelvic abscess present. Because other diagnoses besidesappendicitis exist in children who may present with these signs andsymptoms (eg, severe constipation), a rectal examination should be

performed, preferably only once by the senior-most provider evaluating thepatient.

Laboratory Investigation

There is no definitive laboratory test for accurately diagnosingappendicitis. When evaluating these patients, we order only a completeblood count (CBC) and a urinalysis. An elevated white blood cell (WBC)count, particularly with a bandemia, helps confirm the diagnosis but byitself is not considered to be diagnostic. In these cases we expect to see aWBC count in the range of 12,000 to 16,000, but a very high WBC count

does not exclude the diagnosis. In addition, a normal WBC count may notexclude the diagnosis of appendicitis. Consistently, 4% of our patients withappendicitis have a normal WBC count and differential and are afebrileupon presentation. Upward of 20% to 25% of patients have a normal WBCcount regardless of febrile status [3]. The urinalysis is perfumed to rule outthe potential diagnosis of urinary tract disease. The finding of a few WBCsor red blood cells (RBCs) in the urine, however, does not rule out a case ofappendicitis. Occasionally, the appendix abuts the right ureter or the domeof the bladder; WBCs or RBCs in the urine are then more likely but areusually found only in small numbers. C-reactive protein has become

popular in helping to rule in or rule out appendicitis, particularly in Europe,but over the years studies have not demonstrated this test to be morepredictive than the WBC count [4].

Imaging Studies

In patients where the history, physical examination, and laboratoryfindings do not lead to the diagnosis of appendicitis, imaging studies areindicated. A plain film radiograph of the abdomen or an abdominal seriesmay reveal a fecalith, and in a patient with significant right lower quadranttenderness on physical examination, this is generally considered adequatedata to make the correct diagnosis. However, a fecalith is present in onlyapproximately 10% of the cases, so an ultrasound or a CT scan isnecessary. This has been an area of controversy for the last 5 to 10 years.

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There are proponents on both sides of the ultrasound versus CTevaluation discussion, and both groups claim to have a near 100%accuracy in making the diagnosis [5,6]. Ultrasound evaluation tends to be

an operator-dependent modality. Our experience has been that it is ourfirst choice in the thin patient, particularly within the first 12 to 18 hoursof the presenting illness. A skilled technician or radiologist can accuratelydiagnose appendicitis with an ultrasound evaluation in roughly 50% ofcases [5]. In the evaluation of a more obese child, a CT scan is the

preferred choice [5]. This is performed after the administration of oral orrectal iodinated contrast so that the gastrointestinal (GI) tract (ileum,cecum, and potentially the appendix) in the right lower quadrant is filledwith the radio-opaque contrast agent. One prospective study of 100

patients demonstrated up to a 98% accuracy rate using this method of

evaluation [6].The use of intravenous (IV) contrast material in making the diagnosis

of appendicitis remains controversial. Recent studies have shown that IVcontrast helps to better delineate an inflamed appendix and the

periappendiceal inflammatory response [7]. A CT scan performed tooearly in the course of the illness leads to a false-negative result. This is asignificant problem because we are seeing more patients in our emergencydepartment in whom a CT scan has been performed before the patient isevaluated by a resident or attending surgeon.

Our recommendations for imaging studies in the diagnosis of

appendicitis are to perform them in a systematic fashion. A resident orattending surgeon should evaluate the patient first; then, if it is felt that onthe basis of the history, physical examination, and laboratory studies the

patient has appendicitis, the patient should go directly to the operatingroom without an imaging study [6]. In the equivocal patient who is notobese and who is within the first 24 hours of the illness, an ultrasoundstudy should be performed to rule in or rule out appendicitis. If theultrasound is positive, with a noncompressible, nonperistaltic tubularstructure 6 mm or greater in diameter, the patient goes to the operatingroom for an exploratory laparotomy to evaluate the appendix. If theultrasound is negative, without visualization of the appendix, or equivocal,we proceed with a CT scan. Whether or not this is performed with oral orrectal contrast depends upon the standard protocol for the particularinstitution in which the patient is being evaluated. We almost always usethe oral route, which is less invasive, but the patient must be able totolerate the contrast without vomiting. Although the positive predictive

value (PPV) for the oral route has been reported to be 94%, the rectal routeusing a limited right lower quadrant CT scan has a PPV as high as 98%[8,9].If the result is positive, showing a dilated appendix and signs ofinflammation in the area (Fig. 1), the patient is taken to the operatingroom for surgical evaluation. If the CT results are negative, the patient maybe admitted for observation or discharged at the discretion of the examinerand parents with instructions for follow-up if symptoms worsen. A

prospective study using these criteria has yielded good results with anaccuracy rate of correct diagnosis of 94% [6].

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Perforated Appendicitis

The perforation rate in cases of appendicitis has varied from 10% to85% depending upon age, gender, and whether or not the institution wherethe patient is evaluated is located in the inner city. In most major hospitalcenters, the incidence of perforation in the general pediatric populationranges from 20% to 40%. These patients often present as being dehydratedand toxic-appearing with obvious physical signs of peritonitis. When these

patients present, they should be immediately fluid resuscitated and treatedwith antibiotics before being taken to the operating room.

There is a small subgroup of patients with appendiceal perforations inwhom the diagnosis is missed or who do not present for medical evaluationuntil late in the course of the illness. In this type of patient, who may havebeen ill for a period of 7 to 10 days, radiographic studies often reveal awalled-off abscess or phlegmon in the right lower quadrant. These patientscan often be treated medically and have their abscesses drained

percutaneously by an interventional radiologist. When the patientstabilizes and recovers, they are generally sent home. After a period of 6weeks, we opt to perform an interval appendectomy.

Surgery

At the conclusion of a careful history and physical examination,laboratory studies, and imaging studies, there is a small group of patients in

FIGURE 1.CT scan of the abdomen/pelvis in a patient with acute appendicitis. Note the

circular object in the right paracolic gutter (white arrowhead), with thethickened wall of the cecum and associated mesenteric fat stranding. Theradio-opaque density within the lumen of the appendix is consistent with afecalith.



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whom the diagnosis is unclear. A diagnostic laparoscopy is a reasonableprocedure in this group of patients, and during the procedure the appendixshould be resected, regardless of its appearance. Our experience has been

that frequently the appendix is clearly inflamed on microscopic examina-tion when grossly, via laparoscopy, it appears normal.

Whether a routine appendectomy should be performed in the tradi-tional open manner or via laparoscopy is a point of debate because thereare good studies that show contradictory results [10,11]. Our experiencehas been that in obese patients, who comprise a larger percentage of our

population every year, the laparoscopic approach seems to have anadvantage, yielding decreased operating time and a shorter hospital stay.In the thin, young child, there does not seem to be an advantage usingeither technique.

PYLORIC STENOSIS

Background

Vomiting is a common problem in infancy and childhood. It istherefore essential to differentiate among the various etiologies of

vomiting, including that caused by a nonanatomic etiology, that causedby an anatomic abnormality, or cases that require surgical attention. Most

vomiting caused by an anatomic or mechanical obstruction is associatedwith green or bilious emesis. The most common etiology of anatomicnonbilious vomiting in infancy is infantile hypertrophic pyloric stenosis.

Infantile hypertrophic pyloric stenosis is the progressive elongationand thickening of the circular layer of the antral-pyloric muscle resulting in

progressive gastric outlet obstruction. Pyloric stenosis occurs in 3 of 1000live births [12]. The reported male-to-female ratio ranges from 2:1 to 5:1[13]. Although the etiology of pyloric stenosis is unclear, proposedetiologies include gastric hyperacidity leading to muscle spasm andhypertrophy; abnormal pyloric innervation; and abnormal local concen-trations of vasoactive intestinal peptide, nitric oxide, and substance P.None of these etiologies has been proven or can adequately explain thefavorable response to surgery [14].


Pyloric stenosis usually presents between the third and sixth week oflife and is rarely present at birth. Infants usually present with progressivenonbilious vomiting. This may increase to what is typically described asbeing forceful or projectile vomiting by the parent. Occasionally, the

vomiting can be of an acute onset. It is not uncommon for infants who present with this symptom to be initially diagnosed as having gastro-esophageal reflux. Treatment of these infants with thickened formulas or

prokinetic agents frequently exacerbates their symptoms. Infants may

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have had multiple formula changes in the belief that the vomiting is due toa milk protein allergy. Although vomiting is a typically a part of the milk

protein allergy symptom complex, diarrhea and blood in the stools are

more frequently associated with this entity. Long-standing vomiting may beassociated with coffee-ground emesis due to a secondary gastritis. If the

vomiting is of significant duration, there is associated weight loss, a keysign that may help differentiate pyloric stenosis from gastroesophagealreflux or milk protein allergy.

Physical examination may reveal a normal, healthy-appearing infantor an infant who is lethargic with signs of dehydration. If the vomiting islong standing, the infant may have the appearance of a child with failure tothrive. Generally, from 5% to 10% of infants with pyloric stenosis have anindirect hyperbilirubinemia that resolves after surgical correction. The

diagnosis of pyloric stenosis can be made on physical examination. Twosigns are considered to be pathognomonic: the gastric wave and a palpablepyloric olive, defined as an olive-sized horizontal mass representing thehypertrophic pyloric muscle in the epigastrium. Usually, the presence of agastric wave and a palpable olive cannot be demonstrated during the sameexamination in a child because they have conflicting requirements for theirdemonstration. The gastric wave requires that the stomach be full.

Visualization of the wave requires patience and a restful infant. Frequently,it is easier to see the wave by viewing the childs abdomen tangentially. Thewave appears as a bulging mass that traverses the infants epigastrium

from the left flank to just right of the midline. Palpation of the pyloric oliverequires the infants stomach to be empty and the abdominal wall to berelaxed. By standing on the right side of the infant, the evaluating providercan grasp the legs with the left hand and flex the legs at the hips, thusrelaxing the abdominal wall. Sometimes the administration of an oralglucose water solution may help to settle the infant to facilitate thismaneuver. With the providers right hand starting underneath the liveredge, one can palpate the abdomen deeply and sweep inferiorly. The

pyloric olive can then be appreciated as the providers fingers trap thehypertrophic muscle along the back. The olive can be palpated and

visualized as the hypertrophic pylorus pops as the fingers roll over theolive.

Imaging Studies

The radiologic confirmation of pyloric stenosis is based upon the upperGI series and, more recently, on the pyloric ultrasound evaluation.Typically, the upper GI study shows delayed or no gastric emptying, anelongated pyloric channel with a string sign (a barium-lined elongated andnarrowed pyloric channel) (Fig. 2), and an indentation of the antrum by thehypertrophic pyloric muscle. More recently, the pyloric ultrasound has beenused in the evaluation of pyloric stenosis. Ultrasound has an advantage overthe upper GI series because there is no radiation exposure to the infant andno barium is present in the stomach before the induction of anesthesia.



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Sonography has been shown to be highly sensitive (90% to 96%) and specific(100%) for the diagnosis of pyloric stenosis [15,16]. Using the pyloricultrasound study, two views are paramount: a transverse view that lookslike a bulls-eye to measure the pyloric muscle thickness and a longitudinal

view to measure the muscle wall thickness and the length of the pyloricchannel. The criteria for the diagnosis of hypertrophic pyloric stenosis varyin different reports. The generally accepted measurement criteria are a

muscle thickness of 4 mm and a pyloric channel length of 17 mm (range 34mm and 1617 mm, respectively) [17,18]. The disadvantages of theultrasound diagnosis of pyloric stenosis include a dependence upon theskill of the ultrasound technician, and, in small infants, the measurementcriteria may not be met because of the infants size. Sonography and contrastradiography should not be viewed as competing diagnostic modalities butrather as complementary techniques. Physical examination and radiologicconfirmation may not be diagnostic early in the evolution of pyloric stenosisand may need to be repeated 3 to 7 days after the initial presentation.

Fluids and Electrolytes

Once the diagnosis of pyloric stenosis is suspected, an electrolytepanel should be obtained, and an IV infusion should be started. The infant

FIGURE 2.Barium upper GI series in an infant with pyloric stenosis, demonstrating the

classic string sign with a wisp of contrast passing through the hypertrophicpylorus(white arrowhead).

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should be assumed to be dehydrated in these cases, and an infusion of a 5%glucose solution in a half-normal saline solution with the addition of 10mEq of potassium chloride per liter begun at a rate of 150 mL/kg/d [19]. If

the infant seems to be severely dehydrated, a bolus of 10 to 20 mL/kg of NSmay be required. The typical IV solution recommended for infants of thisage, a 5% glucose solution in a quarter-normal saline solution, should beavoided because this usually worsens any underlying metabolic derange-ment despite attempts at rehydration. Because prolonged vomiting ofgastric contents leads to the loss of hydrogen and chloride ions, the typicalmetabolic abnormality seen is a hypochloremic metabolic alkalosis,evidenced by high serum bicarbonate and low chloride levels. Themetabolic abnormality should be corrected quickly to ensure a serumchloride level above 95 mEq/dL before surgery. In severe cases, this

abnormality may take over 24 hours to correct.

Surgery

Ramstedt [20] first described the basic principles of surgicalcorrection of pyloric stenosis in 1912. The procedure involves thelongitudinal splitting of the hypertrophic muscle down to the gastricmucosa without reapproximation of the muscle (Fig. 3). The basic

operation has not changed since its introduction. Over the years, severalsurgical approaches to the pylorus have evolved. The classical approach isa transverse right upper quadrant incision. Tan and Bianchi [21] introduceda circumumbilical incision for pyloromyotomy in 1986. This technique hasthe advantage of a better cosmetic appearance, leaving an almostundetectable scar for the infant. The mean operating time is slightlylonger for the periumbilical incision than the right upper quadrant incision,reflecting the increased difficulty of delivering the pylorus through thisincision. Some series report a higher rate of mucosal perforation andwound infection using the periumbilical approach [22], whereas othersreport complication rates similar to the classical approach [23]. Recently,

proponents of minimal access surgery have advocated for a laparoscopicapproach for pyloromyotomy [24]. This approach has the advantage of asuperior cosmetic appearance and a lower wound infection rate.

There is a debate between the open and laparoscopic pyloromyotomyapproaches that centers on a higher mucosal perforation rate, particularlyduring the surgeons initial experience with this procedure. Recently, Hall[25] reported a meta-analysis of 345 infants who underwent open

pyloromyotomy versus 230 infants who underwent the laparoscopicprocedure. There were five mucosal perforations in the open pyloromyot-omy group versus eight mucosal perforations in the laparoscopic group[25]. As more pediatric surgeons are trained in advanced laparoscopicsurgery, there will more than likely be a gradual transition from the open

procedure to the laparoscopic pyloromyotomy. In Halls meta-analysis, theaverage operating time for both approaches was 31 minutes [25].



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Pyloromyotomy universally relieves the gastric outlet obstruction.Parents should be warned that their infant may initially vomit after surgeryand that this is not unexpected. Feedings are usually begun 4 to 6 hoursafter surgery, then subsequently every 3 to 4 hours. Most infants are readyfor discharge from the hospital 24 hours after surgery. After discharge, theinfant usually has an increased appetite until they are back on their normalgrowth curve, which usually takes 3 to 4 weeks. There have been no knownlong-term consequences of pyloromyotomy reported in the literature. Themost common operative complications of this procedure include woundinfection, a mucosal perforation rate of 0.08% to 4%[26], and an inadequate

pyloromyotomy. Unrecognized mucosal perforation leads to peritonitis

FIGURE 3.Intraoperative photographs of a pyloromyotomy. The hypertrophied pylorus is

delivered up into the abdominal wound (A), and the muscular wall is dividedlongitudinally and spread to expose the intact mucosa (B).

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and sepsis, and inadequate pyloromyotomy leads to persistent post-operative emesis.

INTUSSUSCEPTION

Background

Intussusception, the telescoping of a proximal limb of intestine (theintussusceptum) into the adjacent distal intestine (intussuscipiens) (Fig. 4),is the most common cause of intestinal obstruction between 3 months and6 years of age, with an incidence of 1 to 4 cases per 1000 live births [27,28].The pediatric clinical presentation of intussusception is distinctly different

from that seen in adults. Intussusception in children involves the terminalileum in 90% of cases, including ileocolic, ileocecal, and ileoileocolicforms, whereas small bowel intussusception is more common in adults(60% of cases) [2931]. Furthermore, pathologic lesions serve as the lead

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The classic presentation of intussusception begins with a previously

well-appearing child who suffers a sudden onset of episodic crampy orcolicky pain accompanied by flexed limbs and straining. Episodes of paingenerally occur 10 to 20 minutes apart and often last for several minutes.The infant may seem asymptomatic between episodes. These painfulepisodes gradually become more frequent and are often accompanied byfever, lethargy, bilious emesis, dehydration, and currant jelly stools. Theclassic triad of colicky abdominal pain, vomiting, and currant stools is

present in 21% of cases [34]. Physical examination of the child may reveal asoft, nontender abdomen early in the course of the illness, although a

palpable, vertically oriented sausage-shaped mass in the right upper

quadrant may be present in up to 50% of cases [35].

Imaging Studies

The value of confirmatory radiographic studies in the diagnosis andtreatment of intussusception has been thoroughly reviewed. Plainabdominal films are often the initial study, and suggestive findings includethe meniscus sign (leading edge of the intussusceptum outlined byadjacent colonic gas), the target sign (mass in the right upper quadrant),

and the absence of cecal gas and stool in the right lower quadrant. Plainradiographs have been shown to be diagnostic in up to 45% of children withintussusception [36]. Barium enema has historically been the traditionalstudy of choice, with diagnostic sensitivity and specificity approaching100%. Evidence of a filling defect at the apex of the column of barium, withtracking around the invaginating intestine to produce a coiled-springappearance, is diagnostic for intussusception. The therapeutic value of abarium enema is also well documented, with reduction of the intussus-ception seen in approximately 70% of the cases [37]. If an obstructive

pattern exists on plain radiographs, reduction of the intussusception viabarium enema is unlikely. Technologic advances combined with trendstoward radiation reduction in the pediatric population have moveddiagnostic ultrasound to the forefront at many centers, with accuracyrates comparable to that of barium contrast enemas [38,39]. Ultrasound-directed pneumatic reduction with an air enema has been shown to havegreater success rates as compared with barium enema or saline hydro-static reduction (90% compared with 70% and 67%, respectively) andgenerally fewer complications [37].

Surgery

In circumstances where hydrostatic or pneumatic reduction areunsuccessful or contraindicated (eg, hemodynamically unstable patients,signs of peritonitis on physical examination), surgical intervention is

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required. Initial intraoperative manual reduction is attempted and isusually successful. The portion of nonviable or irreducible intestine isresected, and an end-to-end primary anastomosis is performed. An

incidental appendectomy is usually performed during this procedure.Laparoscopy is gaining favor, particularly in cases of uncertain or failedreduction by conservative methods [40], and has demonstrated successfulreduction in up to 65% of cases refractory to enema therapy [41].

INTESTINAL MALROTATION

Background

Intestinal malrotation describes an interruption of the typicalintestinal rotation and fixation during fetal development with arrest ofthe process at various stages producing clinically distinct acute or chronic

presentations. Although case reports date back to before 1900, Ladd [42] published a seminal article in 1936 on the treatment of malrotation inwhich he described the surgical procedure that carries his name andremains the standard of care for the operative correction of intestinalmalrotation.

The incidence of intestinal malrotation in the United States isapproximately 1 in 500 live births [43]; 40% of these infants are diagnosed

during the first week of life, and 75% are diagnosed by 1 year of age. Themale-to-female ratio is 2:1 up to 1 year of age; prevalence is equalthereafter. Mortality is highest with a younger presentation, with mortalityrates ranging from 2% to 24% when identified in infancy.

The gestational defect of intestinal malrotation involves the normalembryologic extracoelomic herniation and rotation of the intestinal tractaround the superior mesenteric artery (SMA). This rotation occurssimultaneously with a profound lengthening of the intestinal tract. Duringthe remainder of gestation, the cecum migrates from the right of the SMAdown to the familiar right lower quadrant of the abdomen. Thiscombination of rotation, intestinal lengthening, and cecal migration tothe right lower quadrant results in a broad mesenteric root, running fromthe left upper quadrant to the right lower quadrant of the abdomen, andcontaining all of the vasculature of the intestinal tract from the ligament ofTreitz to the midtransverse colon. Defects at various stages in thissequence can produce different clinical presentations, which have beenbroadly classified into four distinct entities: acute or chronic midgut

volvulus and acute or chronic duodenal obstruction.

Volvulus

Acute midgut volvulus usually presents in the first year of life. Theprimary symptom is sudden onset of bilious emesis and abdominal pain.Because this syndrome entails the vascular compromise of the SMA, the



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entire small bowel is at risk for infarction without prompt intervention.Furthermore, the relatively late appearance of additional diagnostic signs,such as abdominal distension, rectal bleeding, hematemesis, and abdomi-

nal tenderness, makes a high index of suspicion critical for successfulmanagement of these infants.

The presentation of chronic midgut volvulus, resulting from anintermittent or partial twisting producing venous obstruction, is oftenmore subtle. The infant may tolerate feedings and gain weight initially. Ahistory of occasional abdominal pain and malabsorption may be the onlyhints toward diagnosis of this condition. Furthermore, the physicalexamination may be benign if the infant or child presents during anunobstructed interval. Examination during a symptomatic episode isessentially identical to that of an acute midgut volvulus, with abdominal

distension, tenderness, and guarding. Other features of this syndrome caninclude recurrent constipation alternating with diarrhea, intolerance tosolid foods, and gastroesophageal reflux [44].

Duodenal Obstruction

Acute duodenal obstruction results from the compression or kinkingof the duodenum by isolated bands of peritoneum under tension, or Ladds

bands. The typical presentation is an infant with forceful bilious vomiting.On examination, gastric waves may be present, much like that seen in thecase of pyloric stenosis, and peritonitis and shock are present only in rarecircumstances with coincident downstream volvulus.

Intermittent bilious vomiting with pain is the most common presenting complaint with chronic duodenal obstruction from a partiallyocclusive Ladds band. Physical examination is often unimpressive, anddiagnosis is reliant upon a high index of suspicion and radiographicstudies. Depending upon the frequency of emesis, diagnosis can rangefrom during infancy to school-aged children.

Imaging Studies

Radiographic examination is the hallmark of diagnosis in intestinalmalrotation. Abdominal plain films and CT scanning have shown limitedeffectiveness in identifying malrotation. However, the upper GI series andthe contrast enema are valuable, and recent studies involving ultrasoundshow promise [45,46]. The upper GI series is the gold standard in stable

patients and rules out malrotation if the duodenal C-loop crosses to the leftof the midline at a level greater or equal to the pylorus ( Fig. 5). A contrastcolumn that ends abruptly or tapers in a corkscrew fashion is suspiciousfor midgut volvulus. In the case of contrast enema, a normally placedcecum in the right lower quadrant substantially reduces, but does not ruleout, a possible intestinal malrotation.

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Surgery

Medical care is directed toward the stabilization of the patient in preparation for surgical correction. Nasogastric decompression, IVresuscitation, and correction of electrolyte abnormalities should be amongthe first interventions. Evaluation by a pediatric surgeon in unstable

patients should not be delayed for confirmatory contrast studies. The stepsof surgical correction, known collectively as the Ladd procedure, entail thederotation of any volvulus, division of mesenteric bands, placement of thesmall bowel in the right hemiabdomen and large bowel to the left,

appendectomy, and resection of any nonviable loops of small bowel.

MECKELS DIVERTICULUM

Background

Meckels diverticulum, named for the German comparative anatomistwho first described these small bowel diverticula in 1809, results from anincomplete obliteration of the fetal omphalomesenteric or vitelline duct.During the eighth week of gestation, the vitelline duct, a communicationwith the yolk sac, involutes as the placenta replaces the yolk sac as thesource of fetal nutrition. The failure of this vitelline duct obliteration canresult in several anomalies, including omphalomesenteric fistulae, entero-cysts, fibrous bands connecting the small intestine to the umbilicus, or aMeckels diverticulum. Meckels diverticulum, which accounts for 90% of

FIGURE 5.Barium upper GI series in an infant with intestinal malrotation in which the

duodenum fails to cross the midline from the infants right to left and all of theopacified small intestine resides in the right hemiabdomen.



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vitelline duct anomalies [47], is a true diverticulum composed of all layersof the normal intestinal wall. Although their position along the intestinaltract is variable, most Meckels diverticula are found within 100 cm of the

ileocecal valve. The average length is 3 cm [48], and up to 60% containheterotopic mucosa, the most common being gastric mucosa (60%),

pancreatic tissue (16%), jejunal mucosa (2%), or Brunners glands (2%) [49].Meckels diverticulum is considered to be the most common congen-

tial GI tract anomaly, present in up to 2% of the population [50], with amale-to-female ratio of 3:2. Meckels diverticulum has been associated withseveral other anomalies, including congenital cardiac malformations,anorectal atresia, exophthalmos, cleft palate, annular pancreas, and cen-tral nervous system malformations [51]. Complications of Meckels diver-ticulum, estimated at 4% lifetime [52], are most commonly the result of

ectopic tissue or fibrous bands [53] and include intestinal obstruction(36%), diverticulitis/inflammation (32%), intussusception (13%), hemor-rhage (11%), and perforation (7%) [49]. In 0.5% to 3% of symptomatic casesof Meckels diverticula, tumors are present, the most common of which arecarcinoid tumors in 33% of cases [49].

History and Physical

Because Meckels diverticula can imitate a variety of familiar disease

states, including appendicitis, peptic ulcer disease, biliary colic, gastro-enteritis, colonic diverticulitis, and milk protein allergy [53], its diagnosis is problematic and should be considered in all pediatric patients withunexplained abdominal pain, nausea and vomiting, or intestinal bleeding.In children, painless bleeding is the most common presentation for Meckelsdiverticulum (mean age of 5 years) and is the result of ileal mucosalulceration adjacent to acid-producing gastric mucosa. Obstruction is themost common presenting complaint in adults and occurs in up to 40% of

pediatric patients [54]. Most patients are asymptomatic, with the anomalydiscovered on contrast study or laparotomy for another indication.

Physical examination findings are variable and are dependent uponthe nature of the presenting complication. Infants and children presentingwith rectal bleeding often have a benign abdominal examination, andtachycardia is more consistent with a brisk intestinal bleed (bright redblood) rather than slower bleeding (mahogany stool). Obstructed patientsare generally distended and have hyperactive bowel sounds and abdominaltenderness without peritoneal signs and rarely have a palpable mass.Similar to appendicitis, patients with Meckels diverticulitis have focaltenderness with guarding, hypoactive bowel sounds, and peritonitis if

perforation has occurred.

Imaging Studies

Barring the patient that presents with an acute abdomen mandatingemergent surgical exploration, the diagnostic workup is largely based

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upon imaging targeted at a strong clinical suspicion of Meckelsdiverticulum. Routine lab studies, including a CBC, electrolytes, and liverfunction studies, are generally not helpful in making the diagnosis.

Although plain abdominal radiographs, a CT scan, and an ultrasound maybe useful in assessing the complications from Meckels diverticulum (eg,ileus, free fluid in the abdomen, inflammation), they are not diagnostic forMeckels diverticulum [55]. The most useful method of diagnosis is with atechnetium-99m pertechnetate scan, which is dependent upon uptake ofthe isotope in heterotopic gastric tissue and has a reported accuracy of 90%in the pediatric population [56], although documented accuracy is 46% inthe adult population [57]. This accuracy can be enhanced to 95% in childrenwith the combination of pentagastrin (to enhance technetium uptake byectopic gastric mucosa), cimetidine (to decrease intraluminal technetium

release), and glucagon (to decrease peristalsis) [58,59].

Surgery

Treatment for Meckels diverticulum has traditionally been dependentupon whether the entity is symptomatic or discovered incidentally.Symptomatic Meckels diverticula warrant a surgical resection of thediverticulum itself or the segment of small bowel containing it (Fig. 6).When bleeding is the presenting symptom, segmental small bowel

resection is indicated because the bleeding source is often caused bymucosa adjacent to the diverticulum. Whereas surgical treatment forsymptomatic Meckels diverticula is generally straightforward, themanagement of those discovered incidentally has been the topic of muchinvestigation and debate. Given earlier published lifetime complicationrates near 4% for unresected asymptomatic Meckels diverticula [52,60]

versus a reported morbidity of 7% to 12% for elective resection [49],expectant management without resection had been the standard of care.More recent studies have proposed a lifetime complication rate requiringsurgical intervention to be around 6%, whereas the overall morbidity ofremoval of incidental Meckels diverticula is 2% [61], suggesting thatsurgical resection has become the norm for incidental, asymptomaticcases.

HIRSCHSPRUNG DISEASE

Background

The classic description of Hirschsprung disease was credited toHarald Hirschsprung in 1888. This disease entity is characterized byaganglionosis in the myenteric (Auerbach) and submucosal (Meissner)

plexi of the distal colon causing a sustained contraction of that segment. Inapproximately 80% of cases, the aganglionic segment involves the rectumand the sigmoid colon only, whereas in 20% of cases, the aganglionic



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segment involves the more proximal bowel and may involve the entirecolon or the entire colon and variable length of the small bowel. Enlarged

nerve trunks are present in the submucosa between the two muscle layersand contain increased levels of acetylcholinesterase. The smooth musclein the aganglionic segment has a normal tone and a normal excitation-contraction coupling mechanism, and it responds to cholinergic andadrenergic agonists in a way that indicates normal functioning receptors.Based upon this physiology, the disease seems to be neurogenic in nature[62].

The enteric nervous system is formed by cells that migrate to thebowel from the neural crest. Hirschsprung disease is thought to be aneurocristopathy, related to the premature arrest of the craniocaudalmigration of neural crest cells during the fifth to the twelfth week ofgestation. In human fetal models of Hirschsprung disease, neural crestcells had not been observed in the distal gut before their appearance in the

proximal gut [63]. This has significant implications for the surgicaltreatment of the disease in that there are no skip lesions. Once thetransition zone has been identified, the surgeon can be confident that heknows the exact extent of the disease.

Hirschsprung disease occurs in approximately 1 in 5000 live births.There is evidence that genetic factors play a role in this disease entity.Increased risk in siblings is reported to be from 2% to 9%, with a dominant

pattern of inheritance seen in many families. There is increasedassociation of Hirschsprung disease in other chromosomal abnormalities,including trisomy 21 (2% to 10% of cases). There is evidence for autosomaldominant, autosomal recessive and polygenic forms [6466]. There is alsoan association of Hirschsprung disease and the multiple endocrine

FIGURE 6.Intraoperative photograph of a Meckels diverticulum originating from a

segment of small intestine.

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neoplasia syndrome MEN 2a, which predisposes these patients to thyroidcancer [67].


Hirschsprung disease presents most commonly in the newborn withabdominal distension and sometimes bilious vomiting. There is often afailure to pass meconium within the first 24 hours of life. Some infants

present after the newborn period with severe chronic constipation,whereas some present with full-blown sepsis from enterocolitis causingtoxic megacolon, characterized by fever, bilious vomiting, explosivediarrhea, abdominal distension, and shock [68].

Diagnosis

The diagnosis of Hirschsprung disease is made initially by bariumcontrast enema. By using this modality, one can visualize the classictransition zone where the distal bowel is smaller in diameter than the

proximal dilated bowel. This transition zone may not be distinct in thenewborn or in the infant or child with very short-segment Hirschsprungdisease. The diagnosis is confirmed by suction rectal biopsy, usually done

at the bedside, which confirms the absence of ganglion cells and the presence of nerve trunk hypertrophy. Some centers also stain thesespecimens for excess acetylcholinesterase to aid in the diagnosis [69]. Inthe older child who presents with severe constipation since infancy,malnutrition, and an empty rectal vault on physical examination, a full-thickness biopsy under anesthesia is indicated.

Surgery

Treatment of the patient with Hirschsprung disease starts withcolonic irrigations with warm saline to assist in the evacuation of gas andstool. Broad-spectrum antibiotics should be administered in the infant orchild who has any suspicion for enterocolitis (eg, fever, tachycardia, atender abdomen, leukocytosis, or leukopenia). There have been manychanges in the surgical approach to Hirschsprung disease in the lastseveral years. In the past, a two- or three-stage correction was planned,with a leveling colostomy in the newborn period and formal pull-through

procedure when the child was between 1 and 2 years of age. Today, thereare four basic pull-through procedures (Swenson, Duhamel, Rehbein, andSoave) that are used by pediatric surgeons. An in-depth discussion of thesubtleties of how one chooses which version of a given procedure isbeyond the scope of this article. In recent times, single-stage pull-throughs without interval colostomies are being done routinely for short-segment disease [70].



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Laparoscopy is used by many surgeons to minimize the size ofsurgical wounds and to facilitate the pelvic dissection. Complete transanal

pull-throughs without any abdominal portion of the procedure are also

being performed. These can be done safely in children who have short-segment disease with a transition zone in the mobile part of the sigmoidcolon. Finally, the formal pull-through surgery is being offered in thenewborn as small as 2 to 3 kg [71]. These innovations have led tothe corrective surgery being done at an earlier age, which is easier for the

parents to manage with respect to enterostomies and post-operative rectaldilations. There is some evidence that the long-term results in these

patients are improved when the pull-through is done early in the newbornperiod, but long-term data are not available [72]. Avoiding a colostomy hasdecreased the overall morbidity of the treatment of Hirschsprung disease

because a substantial number of complications are related to this stage oftherapy. The minimally invasive approach has also decreased morbidityfrom surgical wounds and postoperative adhesions. Whenever the infanthas other concomitant medical issues or has longer segment disease, afirst-stage colostomy with a pull-through at a later time is the most prudentapproach.

Early complications of surgical therapy include an anastomotic leakleading to pelvic sepsis. This complication occurs in less than 1% of cases,can be life-threatening if not treated aggressively, and can have graveconsequences on long-term continence, requiring a temporary diverting

enterostomy. Other early complications include an anastomotic stricture,enterocolitis, fecal incontinence, and bladder dysfunction (usually noted inthe older child). Late complications of surgical therapy include con-stipation and enterocolitis.

Outcomes for Hirschsprung disease are variable, depending upon howthe practitioner defines fecal continence and soiling. According to Cass[73], In practice, fecal continence can be defined as the passage of stool inthe toilet such that no bowel motion occurs into the diaper orunderclothes, whereas soiling requires a more stringent definition, suchas the presence of any fecal staining on underclothes occurring more thanonce a month. Continence is the result of a complex physiologic functionthat is dependent on motor function, sensory input, extrinsic neuralmodulation, spinal pathways, and cerebral control [58]. A lack of normalfecal control is a significant problem in long-term follow-up, with anincidence ranging from 10% to 50% [73,74].

SUMMARY

The approach to the child with a surgical GI problem should include athorough history and physical examination, followed by appropriatelaboratory and imaging studies. An understanding of anatomy and in somecases embryology can be helpful in establishing an accurate diagnosis.Early surgical consultation should be the rule because some of these

problems rapidly become life threatening.

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Although CT scan and ultrasound have exceptional sensitivity and spe-cificity for acute appendicitis, they should be performed only after thediagnosis is in question after a thorough history and physical examinationby an experienced pediatric surgeon (Evidence Level C).

Pyloric stenosis should be suspected in an infant with nonbilious emesis andweight loss. The preferred radiographic study is diagnostic ultrasound,which avoids radiation exposure and the risk of barium aspiration incurredwith an upper GI (Evidence Level A).

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Address reprint requests to

Michael Ratner, MDSuny Upstate Medical University

Department of SurgeryDivision of Pediatric Surgery

750 East Adams StreetSyracuse, NY 13210

e-mail: [email protected]

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