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?
 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
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?

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 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?

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 NS + KCL
 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
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?
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
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
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
Kasai Portoenterostomy
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?

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
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
Duodenal Atresia
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?
A. Abdominal X-ray.
B. CT scan.
C. Upper GI series.
D. Barium enema.
E. Esophageal pH studies.
 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
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?
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?
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)
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 (Bs and Ds) abdominal wall
erythema +/- mass
Labs: metabolic acidosis thrombocytopenia
 Necrotizing Enterocolitis (NEC)

X-rays:
distended loops c/w ileus,
pneumatosis intestinalis
 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
Overall mortality complication of
20-40% intestinal strictures
and short bowel
syndrome
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?

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
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?
A. Meckels 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?
A. Meckels diverticulum.
B. Acute appendicitis.
C. Intussusception.
D. Intestinal polyp.
E. Gastritis.
 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 H 2O
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?
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?
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.
 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
 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
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?

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
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
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
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
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?

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?

A. Hepatoblastoma
B. Wilms tumor
C. Neuroblastoma
D. Ovarian teratoma
E. Rhabdomyosarcoma
 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
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
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
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
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
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%
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
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
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
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