Pediatric Surgery Review

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Pediatric Surgery Review

• Jason Frischer
• March 18, 2004

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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?
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Esophageal Atresia and Tracheoesophageal Fistula

• Incomplete partitioning of primitive foregut
• 5 types of atresias
• Esophageal atresia with distal TEF most common

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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

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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

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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

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Pyloric Stenosis

• 1 in 600 births, male female ratio 41, 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

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Pyloromyotomy
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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.

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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.

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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

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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

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Biliary Atresia
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Kasai Portoenterostomy
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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.

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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.

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Duodenal Atresia

• Failure to recanalize lumen of duodenum after
  solid phase of embryologic development
• Associated with Downs 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

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Duodenal Atresia
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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.

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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.

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Malrotation

• Lack of retroperitoneal fixation of bowel and
  presence of Ladds 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

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Malrotation
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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.

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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.

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Necrotizing Entercolitis (NEC)

• Idiopathic mucosal intestinal injury, may
  progress to transmural necrosis
• 1/2 patients
• Signs feeding intolerance , vomiting abdomina
  l distention progressive sepsis autonomic
  instability (Bs and Ds) abdominal wall
  erythema /- mass
• Labs metabolic acidosis thrombocytopenia
  

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Necrotizing Enterocolitis (NEC)

• X-rays
• distended loops c/w ileus, pneumatosis
  intestinalis

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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

Long-term complication of intestinal strictures
and short bowel syndrome
Overall mortality 20-40
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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.

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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.

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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

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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. Meckels diverticulum.
• B. Acute appendicitis.
• C. Intussusception.
• D. Intestinal polyp.
• E. Gastritis.

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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. Meckels diverticulum.
• B. Acute appendicitis.
• C. Intussusception.
• D. Intestinal polyp.
• E. Gastritis.

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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

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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.

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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.

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Hirschsprungs 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

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Hirschsprungs 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

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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.

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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.

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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

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Omphalocele
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Omphalocele

• Non-operative management with escharotic agent
• OR for reduction and closure of abdominal wall
• keep intra-abdominal pressure
• 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

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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

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Gastroschisis
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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

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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

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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

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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

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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

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Neuroblastoma
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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

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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

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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

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Wilms Tumor
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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

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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

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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

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Pulmonary Sequestration

• Most frequently diagnosed in the first 6 mos
• Males 3-41
• 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

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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

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CDH