Paediatric Surgical Emergencies Presented by Dr Ayman A A Albatanony Associate Professor of Surgery.

Paediatric Surgical Emergencies

Presented by

Dr Ayman A A Albatanony

Associate Professor of Surgery

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Neonatal Surgical Emergencies 3

Paediatric Surgical Emergencies 1

The subject is divided into 2 main topics:

1. Neonatal intestinal obstruction 2. Neonatal major abdominal wall defects3. Neonatal obstructive jaundice

Neonatal Surgical Emergencies

Neonatal intestinal obstruction

• Necrotizing enterocolitis• Atresia, stenosis• Small Bowel Atresia• Malrotation• Hirschsprung’s• Annular pancreas• Antral web• Meconium ileus• Imperforate anus• Complicated inguinal hernia• Congenital Hypertrophic pyloric stenosis

In physical Exam, remember to:

• Remove diaper

Physical Exam

• Must perform rectal exam, not just look!

Initial Management

• NG or OG (NPO!!)

• Hydrate and replace losses

• Antibiotics if suspect perforation or necrosis

• Consult surgeon and/or transfer to appropriate facility

Bowel Obstruction

• Diagnosis is often age specific

• Bilious vomiting in the infant and child is a surgical emergency until proven otherwise

• Child may look surprisingly good until it’s too late

Atresia

• Usually presents the first few days of life

• Child may feed well for a day or two with distal atresia

• Duodenal atresia often diagnosed on antenatal U/S

• Atresias can occur anywhere in GI tract from pharynx to anus

Atresias

• Esophageal: aspirate feeds immediately, OG tube won’t pass (non-bilious, but still bad)

• Duodenal: bilious vomiting immediately, “double bubble” on KUB with absence of distal gas, Down’s Syndrome

• Jejunal: usually present 1st 24 hours, large dilated proximal loop or loops

Atresias

• Ileal: may take 24-48 hours before bilious emesis

• Colonic: rare, may present with bilious emesis after 2-3 days

• Anal: should be diagnosed at birth, often a perineal fistula is labeled normal

Atresias may be multiple

Jejunal Atresia

Apple Peel Deformity (IIIb)

Imperforate Anus: Anal atresia

Hirschsprung’s Disease

• Congenital colonic aganglionosis– Physiologic obstruction

• May present first few days to weeks of life

• Starts at anus and extends proximally a variable distance

Hirschsprung’s

• Delayed passage of meconium at birth:– Meconium plug syndrome, small left colon syndrome,

Down’s syndrome

• Often present with distension• Profoundly distended abdomen with dilated

bowel• Fever and WBC’s with colitis

Hirschsprung’s

• Rectal exam may seem normal until withdraw finger

• “Explosive” release of liquid stool almost diagnostic

• Barium enema while dilated

• Irrigate and dilate until decompressed

• Rectal biopsy

Hirschsprung’s Disease

Necrotising enterocolitis:

Aetiology:– Remains unknown – Ischemia and/or reperfusion injury may play a role – Translocation of intestinal flora across compromised mucosa

may play a role

• Incidence and age at onset – More common in premature infants

• But can also be seen in term babies • Affected term neonates are usually systemically ill with other

conditions such as birth asphyxia, respiratory distress or congenital heart disease

– Babies who are breastfed have a lower incidence of NEC than formula-fed babies

Imaging findings

– Plain film of the abdomen remains method in which disease is diagnosed most often

– Findings include • Dilated loops of bowel • Thickened bowel walls

– Fixed and dilated loop that persists is especially worrisome

• Absence of bowel gas • Pneumatosis intestinalis

– Pathognomonic of NEC in newborn » Linear radiolucency parallels bowel lumen within

bowel wall » Represents air that has entered from the lumen

Toxic Megacolon

• Severe enterocolitis

• NG decompression, IV fluids, IV antibiotics

• Mortality 20-30%

Toxic Megacolon

Malrotation

Normal

Malrotation

• Most often presents during the first few days of life

• Infant with acute onset of bilious emesis• Malrotation is a surgical urgency due to

the possibility of volvulus• VOLVULUS IS A SURGICAL

EMERGENCY

Volvulus

• Malrotation most common condition resulting in midgut volvulus

• Can have volvulus with normal rotation– omphalomesenteric remnant– internal hernia– Duplication– Adhesive small bowel obstruction

Midgut Volvulus

Volvulus

• 75% First month of life• Malrotation is the risk for volvulus

– Small and large bowel are not fixed– Narrow mesentery more likely to turn around itself

• Malrotation can cause or present with:– Volvulus is dangerous– Acute obstruction– Chronic intermittent obstruction

Volvulus is lethal

• Malrotation midgut volvulus midgut intestinal death surgery (resected) short-gut syndrome death

• C/F– Bilious vomiting– +/- pain

• if +pain (irritable) likely volvulus +ischemia• - pain (calm) malrotation+obstruction

Midgut volvulus

• Infant + Bilious vomiting is EMERGENCY• Investigate (if infant is not sick)

– Upper GI series (look for malrotation)• No duodenal C-loop• Duodeno-jejunal junction (ligament of Treitz) to the right of

Vertebral col.• Duodenal obstruction • Whirlpool or corkscrew sign (volvulus)

– U/S• Can’t R/O volvulus• Can Dx volvulus

Midgut volvulus• Pt should go directly for surgery if:

– If can’t do investigation immediately – Pt is sick + bilious vomiting

• Time = Bowel

• Surgery:– Untwist (counter clock wise) assess viability– If extensive ischemia close 2nd look 24-48

hrs– Viable SB close and observe– Ladd’s procedure

Meckel’s

Duplication

Intussusception

• Inversion of the bowel upon itself secondary to a lead point

• Juvenile intussusception most often idiopathic– Also secondary to Meckel’s

• Presents 6 months to 2 years of age– As early as 1 month

Intussusception

• Acute painful episodes followed by periods of lethargy

• When incarcerated progress to continuous lethargy

• May have “currant-jelly” stool– But often stool is heme positive

• Rule out with a left lateral decubitus film

Left-lateral Decubitus Film

Intussusception

Intussusception

Bad Intussusception

Intussusception

• 7% chance of recurrence after hydroststic reduction– May recur in 48 hours

• Operative exploration warranted on second recurrence to R/O pathologic lead point

• Recurrence after surgery rare but possible• Post-op intussusception can occur after

any surgery

Congenital Hypertrophic Congenital Hypertrophic Pyloric StenosisPyloric Stenosis

Pyloric stenosis

• Presentation: vomiting occurs after all feeds copious, no bile in vomitus may be blood, initially child wants to feed again, later becomes weak, listless, metabolic alkalosis, failure to thrive

• Signs-may palpate pyloric tumour 9 in epigastrium just above umbilicus or between liver edge and right rectus, may see peristalsis

• presents in first few months of life-between 3-6 wks of age rare if <10days or older than 11 wks

• Affects 1:450 children

• Males ( 85%)more common than females

• Tx: NG tube, Rehydration, electrolyte correction, Ramstedt procedure

Incarcerated Hernia

Incarcerated Inguinal Hernia

Hernia Reduction

From Surgery of Infants and Children, Oldham, et. al., 1997

Incarcerated Hernia

• Most can be reduced in clinic or ED

• Bowel usually OK if able to reduce

• Surgical consultation if reduction difficult

• Repair with 1-2 days of incarceration

• Beware the “inguinal node’ in females– incarcerated ovary

Incarcerated Hernia

• If unable to reduce: urgent operative exploration (NPO)

• If able to reduce without sedation: urgent surgical referral with repair soon

• If extremely difficult (sedation, surgical referral): repair next day

• Watch child for obstructive symptoms

Perforated Appendix

• Children still die from complications of perforated appendicitis

• Resuscitation is critical• Diagnosis difficult...Why??

Perforated Appendix

• Suspect in children 3-5 years old with history suggestive of appendicitis

• “Bowel obstruction” in a 3-5 year old without obvious etiology is perforated appendix until proven otherwise

Perforated Appendix

Resuscitation

• NG tube, NPO

• 20 cc/kg boluses until UOP > 1 cc/kg/hr and VS stable

• 1.5-2 times maintenance fluids

• Broad Spectrum Antibiotics

Perforated Appendix

Summary

• Atresias• Hirschsprung’s• Malrotation• Volvulus• Intussusception• Incarcerated Hernia• Perforated Appendix

Neonatal major abdominal wall defects

Omphalocele

Gastroschisis

OMPHALOCOELE

• Anterior abdominal wall defect at the base of the umbilical cord with herniation of the umbilical contents

Incidence

• Small omphalocoele 1:5000

• Large omphalocoele 1:10000

• Male to female ratio 1:1

Pathophysiology

• Failure of the midgut to return to abdomen by the 10th week of gestation

Clinical Findings

• Covered clinical defect of the umbilical ring

• Defect may vary from 2-10 cm

• Sac is composed of amnion, Wharton’s jelly and peritoneum

• 50% have accompanying liver, spleen, testes/ovary

• Cord attachment is on the sac

• Presentation :• AFP level at 12 wks is elevated • Detected at routine morphology USS

GASTROSCHISIS

• herniation of bowel contents through a defect in the anterior abdominal wall,not related to the umbilicus, not in the midline and organs not confined to peritoneal sac

Incidence

• 1:20,000-30,000

• Sex ratio 1:1

• 10-15% have associated anomalies

• 40% are premature

Pathophysiology

• Abnormal involution of right umbilical vein

• Rupture of a small omphalocoele

• Failure of migration and fusion of the lateral folds of the embryonic disc on the 3rd-4th week of gestation

Clinical Findings

• Defect to the right of an intact umbilical cord allowing extrusion of abdominal content

• No covering sac

• Bowels often thickened, matted and edematous

• 10-15% with intestinal atresia

Management (both)

• ABC

• Heat Management– Sterile wrap or sterile bowel bag– Radiant warmer

• Fluid Management– IV bolus 20 ml/kg LR/NS– D10¼NS 2-3 maintenance rate

• Nutrition– NPO and TPN

• Gastric Distention– OG/NG tube

• Infection Control

• Associated Defects

• Conservative treatment– Reduction by squeezing the sac– Painting sac with escharotic agent

• 0.25% Silver nitrate• 0.25% Merbromin (Mercurochrome)

• Surgical Management– Skin Flaps– Primary Closure– Staged Closure

• Staged repair using silo pouch

Skin Flaps

Primary Closure

Staged Closure

Omphalocoele Gastroschisis

Incidence 1:6,000-10,000 1:20,000-30,000

Delivery Vaginal or CS CS

Covering Sac Present Absent

Size of Defect Small or large Small

Cord Location Onto the sac On abdominal wall

Bowel Normal Edematous, matted

Omphalocoele Gastroschisis

Other Organs Liver often out Rare

Prematurity 10-20% 50-60%

IUGR Less common Common

NEC If sac is ruptured 18%

Associated Anomalies

>50% 10-15%

Treatment Often primary Often staged

Prognosis 20%-70% 70-90%

Neonatal obstructive jaundice

Biliary atresia

Choledochal cyst

Inspissated bile syndrome

Sepsis

Biliary Atresia

Incidence 1/20,000

• Obliterative process of the extrahepatic bile ducts

• Associated with hepatic fibrosis

• Arrest of development during the solid stage of bile duct formation.

• Aetiology unclear

• Atretic ducts – solid fibrous cords that may contain occasional islands of biliary epithelium

• Three patterns: minimal, partial complete

• Over time the failure to excrete bile results in progressive periportal fibrosis and obstruction of the intrahepatic portal veins

Presentation & Diagnosis• Grey or acholic stools – secondary to

obstructed bile flow• Failure to thrive• Liver failure and portal hypertension• Bilirubin > 3 mg/dl• Alk phos 500-1000• GGT > 300

• Technetiun-99m iminodiacetate (HIDA) after pretreatment with phenobarbital ( promotes tracer uptake)

• If radionucleotide appears in the intestine then the biliary tree in presumed to be patent

• Ultrasound can exclude choledochal cyst

Treatment

• Excise scarred bile ducts and gall bladder and Portoenterostomy

• Biliary atresia (60%)

• Neonatal Hepatitis (35%)

hepatic inflammation that can be secondary to several different causes:

• CMV, syphilis, herpes, toxoplasma

• Metabolic defects : Alpha 1-antitrypsin deficiency, galactosemia

• Choledochal cyst (5%)

Continued• Spontaneous perforation of extrahepatic bile ducts Ascites, mild jaundice, failure to thriveUsu occurs at the junction between the cystic and common

bile ducts• Inspissated bile syndrome – bile plug syndrome Extrrahepatic obstruction of the bile ducts by biliary sludgeAssociated with massive hemolysis, hemorrhage, TPN,cystic fibrosis and other intestinal diseases such as

Hirchsprungs

Diaphragmatic hernia

• Congenital diaphragmatic hernias occur in about 1 out of 2,500 live births with a 2 to 1 male to female ratio.

• Herniation of abdominal viscera occurs through a defect

in the diaphragm caused by failure of the pleural peritoneal canal to close completely during embryonic development.

• Varying degrees of herniation can occur.

• These patients will often have hypoplastic lungs due to crowding of the thoracic space.

• They may show signs of severe respiratory distress such as dyspnea and cyanosis if herniation of abdominal contents is to such an extent as to cause hypoplastic lungs.

• Signs and symptoms of acute intestinal obstruction can also occur.

• The diagnosis is usually made by radiographic examination.

• Emergency treatment: respiratory support, Nasogastric intubation with suction. Extracorporal membrane oxygenation (ECMO) may improve prognosis although mortality rate remains about 50%.

TracheoEsophageal FistulaTracheoEsophageal Fistula

TracheoEsophageal Fistula5 Types (Gross and Vogt)

7.7% 0.8% 86% 0.7% 4.2%

Tracheoesophageal Fistula

Incidence: 1:4000 live births

M > F (25:3)

10-40% are preterm

Antenatal history: polyhydramnios (60%)

Etiology: failure in mesenchymal separation of upper foregut

Tracheoesophageal Fistula

Clinical Presentation

choking on 1st feed

coughing

cyanosis

excessive salivation

aspiration pneumonia

Tracheoesophageal Fistula

Diagnosis

• inability to pass a suction catheter

into the stomach

• CXR: coiled orogastric tube in the

cervical pouch; air in the stomach

and intestine

Tracheoesophageal FistulaTracheoesophageal Fistula

Emergency management

NPO

head-up position

sump tube on low continuous suction

± gastrostomy under local anesthesia

Antibiotics

12-L ECG and Echocardiogram :

mandatory???

IV access ± arterial line

Pediatric surgical emergency 1

Differential diagnosis of an acutely painful scrotal swelling in a 6 years old boy

• Testicular torsion• Acute epididymitis (with or without orchitis)• Orchitis (e.g., mumps)• Trauma eg testicular haematoma• Testicular tumor (hemorrhage within tumor)• Incarcerated inguinal hernia

Thank you