Pediatric Surgery Review :Pediatric Surgery Review Jason Frischer March 18, 2004


1. An 8 hr old infant drools and returns his first feed. A tube in passed into the esophagus and a film obtained. What is the diagnosis? :1. An 8 hr old infant drools and returns his first feed. A tube in passed into the esophagus and a film obtained. What is the diagnosis?


Esophageal Atresia and Tracheoesophageal Fistula :Esophageal Atresia and Tracheoesophageal Fistula Incomplete partitioning of primitive foregut 5 types of atresias Esophageal atresia with distal TEF most common 8% 1% 85% 2% 4%


Esophageal Atresia and Tracheoesophageal Fistula :Esophageal Atresia and Tracheoesophageal Fistula Can be part of VACTERL anomalies vertebral, anal, cardiac, TEF, renal, limb Atresias detected by inability to pass NGT/OGT TEF w/o atresia presents with recurrent aspiration Low-risk infants should get primary repair long gap (>3 vertebral bodies) repair is delayed high-risk babies get gastrostomy Post-op complications include esophageal leak, dysmotility, GE reflux, strictures


2. A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition? :2. A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition? A. Immediate laparotomy is warranted. B. UGI series is the diagnostic procedure of choice. C. Delay in diagnosis leads to metabolic acidosis. D. Most commonly seen in females. E. Fluid replacement consists of ½ NS + KCL


2. A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition? :2. A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition? A. Immediate laparotomy is warranted. B. UGI series is the diagnostic procedure of choice. C. Delay in diagnosis leads to metabolic acidosis. D. Most commonly seen in females. E. Fluid replacement consists of ½ NS + KCL


Pyloric Stenosis :Pyloric Stenosis 1 in 600 births, male: female ratio 4:1, 3-12 weeks Gastric outlet obstruction due to hypertrophy of pyloric muscle Progressive, projectile non-bilious vomiting Hypochloremic, hypokalemic metabolic alkalosis renal compensation for hypovolvemia Sono is diagnostic procedure of choice thickness > 5 mm, channel length > 15 mm Repair via Fredet-Ramstedt pyloromyotomy


Pyloromyotomy :Pyloromyotomy


3. A 6-wk-old infant presents with jaundice. A sonogram appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management? :3. A 6-wk-old infant presents with jaundice. A sonogram appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management? A. List for liver transplant. B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.


3. A 6-wk-old infant presents with jaundice. An abdominal USG appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management? :3. A 6-wk-old infant presents with jaundice. An abdominal USG appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management? A. List for liver transplant. B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.


Biliary Atresia :Biliary Atresia Fibrous obliteration of extrahepatic bile ducts 1 in 10-15 thousand births Jaundice, conjugated hyperbilirubinemia, firm hepatomegaly due to biliary cirrhosis Lab work up should include LFTs, Alpha-1 antitrypsin, TORCH infections, sweat test, hepatitis Sono shows no extrahepatic ducts, tiny gallbladder HIDA scan reveals no emptying into the duodenum Liver biopsy reveals cholestasis and bile duct proliferation


Kasai Portoenterostomy :Kasai Portoenterostomy Roux-en-Y limb of jejenum sutured to porta where atretic bile ducts exit hepatic parenchyma Results depend on age (10 weeks), anatomy and histology of atretic bile ducts, ? degree of cirrhosis overall: 1/3 fail immediately Long term survival in 25% of those that have drainage Results of liver transplantation not affected by Kasai procedure


Biliary Atresia :Biliary Atresia


Kasai Portoenterostomy :Kasai Portoenterostomy


4. Which of the following is TRUE regarding duodenal atresia? :4. Which of the following is TRUE regarding duodenal atresia? A. It is associated with trisomy 21 in 10% cases. B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.


4. Which of the following is TRUE regarding duodenal atresia? :4. Which of the following is TRUE regarding duodenal atresia? A. It is associated with trisomy 21 in 10% cases. B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.


Duodenal Atresia :Duodenal Atresia Failure to recanalize lumen of duodenum after solid phase of embryologic development Associated with Down’s syndrome in 30% Vomiting can be bilious or non-bilious Abdominal X-ray shows “double-bubble” Best repaired by bypass -> duodenoduodenostomy or duodenojejunostomy no indication to divide annular pancreas


Duodenal Atresia :Duodenal Atresia


5. A 3-wk-old baby, previously well, presents with sudden onset of bilious vomiting. What study is most appropriate? :5. A 3-wk-old baby, previously well, presents with sudden onset of bilious vomiting. What study is most appropriate? A. Abdominal X-ray. B. CT scan. C. Upper GI series. D. Barium enema. E. Esophageal pH studies.


5. A 3-wk-old baby, previously well, presents with sudden onset of bilious vomiting. What study is most appropriate? :5. A 3-wk-old baby, previously well, presents with sudden onset of bilious vomiting. What study is most appropriate? A. Abdominal X-ray. B. CT scan. C. Upper GI series. D. Barium enema. E. Esophageal pH studies.


Malrotation :Malrotation Lack of retroperitoneal fixation of bowel and presence of Ladd’s bands partial or complete duodenal obstruction bowel may twist around SMA axis = volvulus up to 75% present w/in 1st month of life Volvulus may present as pain, rectal bleeding, cardiovascular collapse w/ metabolic acidosis untwist in direction of normal rotation (CC for surgeon) UGI shows duodenojejunal junction to the R of midline, more cephalad


Malrotation :Malrotation


6. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition? :6. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition? A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.


6. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition? :6. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition? A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.


Necrotizing Entercolitis (NEC) :Necrotizing Entercolitis (NEC) Idiopathic mucosal intestinal injury, may progress to transmural necrosis 1/2 patients < 1500 g, 80% < 2500 g at birth Signs: feeding intolerance , vomiting abdominal distention progressive sepsis autonomic instability (B’s and D’s) abdominal wall erythema +/- mass Labs: metabolic acidosis thrombocytopenia


Necrotizing Enterocolitis (NEC) :Necrotizing Enterocolitis (NEC) X-rays: distended loops c/w ileus, pneumatosis intestinalis


Necrotizing Enterocolitis (NEC) :Necrotizing Enterocolitis (NEC) Indications for OR are free air (absolute), fixed abdominal mass, abdominal wall erythema, failure to improve (controversial) OR for resection of dead bowel, formation of stomas “second-look laparotomy” 24-48 hrs if needed Overall mortality 20-40% Long-term complication of intestinal strictures and short bowel syndrome


7. Which of the following is FALSE regarding meconium ileus? :7. Which of the following is FALSE regarding meconium ileus? A. Underlying diagnosis is usually cystic fibrosis. B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (“soap bubble” sign). E. May be relieved by water-soluble contrast enema.


7. Which of the following is FALSE regarding meconium ileus? :7. Which of the following is FALSE regarding meconium ileus? A. Underlying diagnosis is usually cystic fibrosis. B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (“soap bubble” sign). E. May be relieved by water-soluble contrast enema.


Meconium Ileus :Meconium Ileus Newborn bowel obstruction secondary to inspissated meconuim in distal ileum Enema reveals microcolon -> may be therapeutic Non-operative management successful in 2/3 OR required for perforation or failed enema may flush bowel with N-acetylcysteine in saline Bishop-Koop as option if stoma required -> end-to-side w/ proximal end of distal bowel brought out as stoma


8. A listless 9-month-old boy presents with acute onset of severe intermittent abdominal pain. Rectal exam is guaiac positive. What is the most likely diagnosis? :8. A listless 9-month-old boy presents with acute onset of severe intermittent abdominal pain. Rectal exam is guaiac positive. What is the most likely diagnosis? A. Meckel’s diverticulum. B. Acute appendicitis. C. Intussusception. D. Intestinal polyp. E. Gastritis.


8. A 9-month-old boy presents with acute onset of crampy abdominal pain. Rectal exam is guiac positive. What is the most likely diagnosis? :8. A 9-month-old boy presents with acute onset of crampy abdominal pain. Rectal exam is guiac positive. What is the most likely diagnosis? A. Meckel’s diverticulum. B. Acute appendicitis. C. Intussusception. D. Intestinal polyp. E. Gastritis.


Intussusception :Intussusception Commonly affects children 3 months to 2 yrs severe crampy abdominal pain (every 10-20 minutes) vomiting, “currant jelly” stools tender, sausage-like mass in RUQ Telescoping of terminal ileum into large intestine Contrast enema for diagnosis will reduce 80% air pressure to 120 mmHg, barium to 100 cm H2O 10% recurrence, often within hours OR reduction if not reduced radiographically 5% of patients need resection


9. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis? :9. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis? A. It is more common in males. B. Suction rectal biopsy is rarely adequate for diagnosis. C. Enterocolitis is a significant cause of mortality. D. Disease is most often confined to the distal colon. E. Barium enema may be normal.


9. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis? :9. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis? A. It is more common in males. B. Suction rectal biopsy is rarely adequate for diagnosis. C. Enterocolitis is a significant cause of mortality. D. Disease is most often confined to the distal colon. E. Barium enema may be normal.


Hirschsprung’s Disease :Hirschsprung’s Disease Absence of ganglia in submucosal and myenteric plexuses variable proximal extension of aganglionosis lack of peristalsis and failure of sphincter relaxation rectosigmoid only in 75%, entire colon in 8% Presents as failure to pass meconium w/in 24 hrs or constipation in older child Diagnosis best made by rectal biopsy suction adequate if submucosa present


Hirschsprung’s Disease :Hirschsprung’s Disease OR requires biopsies to confirm ganglion cells in normal bowel “Pull-through” operations Swenson: complete excision, anastamosis to proximal anal canal at columns of Morgagni Soave: endorectal mucosal excision, pull through rectal muscular sleeve Duhamel: retains portion of aganglionic bowel anteriorly using GIA stapler


10. Which of the following statements is TRUE with respect to neonatal abdominal wall defects? :10. Which of the following statements is TRUE with respect to neonatal abdominal wall defects? A. The bowel in omphalocele is covered by a sac. B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.


10. Which of the following statements is TRUE with respect to neonatal abdominal wall defects? :10. Which of the following statements is TRUE with respect to neonatal abdominal wall defects? A. The bowel in omphalocele is covered by a sac. B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.


Omphalocele :Omphalocele Occur 1 in 5000 live births, more common in boys over 50% have associated cardiac, GI, GU, musculoskeletal, or CNS anomalies Herniation of abdominal contents through defective umbilical ring overlying sac of outer amnion and peritoneum umbilical cord in continuity with sac liver involved in larger defects High mortality (30-60%) due to other anomalies


Omphalocele :Omphalocele


Omphalocele :Omphalocele Non-operative management with escharotic agent OR for reduction and closure of abdominal wall keep intra-abdominal pressure < 20 mmHg large defects require skin flap or prosthetic Silastic silo most common, reduce daily for 3-10 days Post-op complications include sepsis, GE reflux, inguinal hernias, abdominal wall hernia


Gastroschisis :Gastroschisis Anterior abdominal wall defect (“belly cleft”) usually to right of umbilical cord no sac or membrane covering contents exposed bowel thick, edematous, exudative peel associated intestinal atresias in 10% Initial management aggressive fluid replacement (2-3X normal) protection of exposed bowel w/occlusive dressing


Gastroschisis :Gastroschisis


Gastroschisis :Gastroschisis Primary reduction and closure in 80-90% cases Silastic silo if high intra-abdominal pressure may require resection if exposed bowel non-viable Post-op complications: abdominal compartment syndrome sepsis, necrotizing enterocolitis abdominal wall cellulitis prolonged ileus short gut syndrome w/ TPN dependence


11. A 3-year-old girl is referred to you with fever, failure to thrive, periorbital ecchymoses, and a large abdominal mass. What is the most likely diagnosis? :11. A 3-year-old girl is referred to you with fever, failure to thrive, periorbital ecchymoses, and a large abdominal mass. What is the most likely diagnosis? A. Hepatoblastoma B. Wilms tumor C. Neuroblastoma D. Ovarian teratoma E. Rhabdomyosarcoma


11. A 3-year-old girl is referred to you with fever, failure to thrive, periorbital ecchymoses, and a large abdominal mass. What is the most likely diagnosis? :11. A 3-year-old girl is referred to you with fever, failure to thrive, periorbital ecchymoses, and a large abdominal mass. What is the most likely diagnosis? A. Hepatoblastoma B. Wilms tumor C. Neuroblastoma D. Ovarian teratoma E. Rhabdomyosarcoma


Neuroblastoma :Neuroblastoma Most common extracranial solid tumor in children median age of onset is 2 years over 90% present by 8 years Arises from the neural crest 60% in abdomen (mostly from the adrenal gland) thoracic tumors next most common (posterior mediastinum) Genetic abnormalities common (80%) short arm chromosome 1, N-myc amplification, MDR gene, DNA ploidy


Neuroblastoma :Neuroblastoma Most commonly presents with abdominal mass constitutional symptoms: fever, weight loss, anemia, FTT, bone pain Metastases at presentation in 3/4 of patients bone, BM, and lymph nodes most common liver and skin less frequently, rare lung and brain X-rays may reveal stippled calcifications Pre-treatment staging essential CT scan, MIBG scan, BM biopsy, urine catacholamines


Slide 50:Neuroblastoma


Neuroblastoma :Neuroblastoma Prognosis depends on age, stage, histology (Shimada classification), and genetic factors poor prognosis with N-myc amplification, allelic loss of 1p, MDR over-expression, normal ploidy Staging by INSS depends on localization and excision Survival is improving stage I 90% 4-yr survival stage IV 15-40% 4-yr survival after BM transplant


Wilms’ Tumor :Wilms’ Tumor Most common renal tumor of childhood incidence 5-10/100,000 70% present before 5 years, median age is 3 years rare non-sporadic presentation with aniridia, hemihypertrophy, urinary tract malformations 5% bilateral Most often seen as asymptomatic abdominal mass pain with tumor necrosis and hemorrhage gross hematuria rare, microscopic hematuria 40% hypertension in 25% from high circulating renin


Wilms’ Tumor :Wilms’ Tumor Pathology large, bulky, well-encapsulated lesions propensity for venous extension in renal vein, IVC, RA histology is tri-phasic: blastemal, stroma, and epithelial elements FH vs. UH (anaplastic) histology affects prognosis Pre-treatment imaging CXR, AXR (linear calcifications) USG of kidney and venous drainage CT scan of abdomen +/- chest


Slide 54:Wilms’ Tumor


Wilms’ Tumor :Wilms’ Tumor Management radical, transperitoneal nephrectomy with post-op adjuvant chemotherapy for unilateral disease pre-op chemotherapy for bilateral disease, intravascular tumor extension, and unresectable disease Outcome (NWTS trials) FH: 95% stage I to 80% stage IV UH prognosis much poorer in stage II-IV overall UH survival 62%


Slide 56:12. Which statement is false regarding extrapulmonary sequestration? A. The parenchyma is not connected to the tracheobronchial tree B. Arterial blood supply is systemic C. Venous blood supply is pulmonary D. Most frequently in males E. Commonly associated with other anomalies


Slide 57:12. Which statement is false regarding extrapulmonary sequestration? A. The parenchyma is not connected to the tracheobronchial tree B. Arterial blood supply is systemic C. Venous blood supply is pulmonary D. Most frequently in males E. Commonly associated with other anomalies


Pulmonary Sequestration :Pulmonary Sequestration Most frequently diagnosed in the first 6 mos Males 3-4:1 Usually located b/w LLL and diaphragm Systemic arterial supply – 95% Aorta 80%, PA 5% Systemic venous drainage – >80% Associated annomalies – 65% Pulmonary hypoplasia 25%, CDH 16%


Congenital Lobar Emphysema :Congenital Lobar Emphysema Air trapped in the lobe Leads to adjacent lobe atelectasis Shifts mediastinum to opposite side More common in the upper lobes CXR for diagnosis Resection provides definitive treatment


CDH :CDH