MedCosmos Surgery

Surgery Lecture Notes, Books, MCQ and Good Articles

Saturday, September 6, 2008

Pediatric Surgery MCQ

1. Polyhydramnios is frequently observed in all of the following
conditions except:
A. Esophageal atresia.
B. Duodenal atresia.
C. Pyloric atresia.
D. Hirschsprung's disease.
E. Congenital diaphragmatic hernia.
Answer: D

DISCUSSION: Polyhydramnios is defined as excessive amounts of fluid

(>2000 ml.) in the amniotic sac during pregnancy. The amniotic pool is
a dynamic pool with a relatively rapid turnover. In the fourth
intrauterine month the fetus begins to swallow amniotic fluid (25% to
40% of the volume) and absorbs the fluid from the upper
gastrointestinal tract. The fluid is urinated back out into the amniotic
pool by the fetal kidneys and a functioning bladder. Although there
are maternal causes of polyhydramnios (cardiac failure, renal failure,
other causes of fluid retention) and some idiopathic cases, many
instances are related to the presence of fetal anomalies. These
include central nervous system problems such as anencephaly, which
prevents normal swallowing, and any high alimentary tract
obstruction that blocks the passage of the amniotic fluid and prevents
its absorption (including esophageal atresia, pyloric atresia, and
duodenal atresia). In addition, infants with congenital diaphragmatic
hernia have obstructions due to herniation of the stomach and bowel
into the thoracic cavity. This is a poor prognostic finding in these
infants. Hirschsprung's disease is a form of low intestinal obstruction,
and therefore an adequate length of proximal patent intestine is
available for absorption of the swallowed amniotic fluid and
polyhydramnios is usually not present.

2. Which of the following statements about Hirschsprung's disease

is/are true?
A. There are no ganglion cells seen in Auerbach's plexus.
B. There is an increased incidence of Down syndrome.
C. It is more common in girls.
D. It may be associated with enterocolitis.
E. It may involve the small intestine.
Answer: ABDE

DISCUSSION: The affected segment of bowel in patients with

Hirschsprung's disease has hypertrophic nerves in Auerbach's
intermyenteric plexus, but no ganglion cells are present. Ganglion
cells are also absent in Meissner's submucosal plexus. Some 3% to 5%
of babies with Hirschsprung's disease also have Down syndrome.
Hirschsprung's disease should be suspected in infants with Down
syndrome that manifest evidence of abdominal distension and
constipation. Hirschsprung's disease is much more common in boys
(4:1). The enterocolitis of Hirschsprung's disease is a condition
associated with delay in diagnosis, low bowel obstruction, severe
abdominal distension, explosive diarrhea, and colonic mucosal
ulceration. The course may be fulminant. This complication is
associated with increased morbidity and mortality. Bacterial
translocation and endotoxemia may complicate the condition.
Treatment includes nasogastric suction, intravenous fluids, antibiotics,
and rectal tube decompression of the obstructed rectosigmoid
segment. In approximately 10% of cases aganglionosis extends into
varying lengths of small bowel. In rare instances, the entire small
bowel and colon may be aganglionic.

3. Which of the following statements is/are true of infants with

gastroschisis?
A. It is associated with malrotation.
B. There is a high incidence of associated anomalies.
C. There is prolonged adynamic ileus following repair.
D. It is complicated by intestinal atresia in 10% to 12% of cases.
E. It is associated with chromosomal syndromes.
Answer: ACD

DISCUSSION: Because of intrauterine herniation of bowel to an extra-

abdominal location, normal intestinal rotation and fixation do not
occur. Most infants with gastroschisis have nonrotation. In contrast to
infants with omphalocele, in which a high incidence of associated
anomalies coexist, babies with gastroschisis have little else wrong.
Following repair of the abdominal wall defect, infants with
gastroschisis have a long delay in return of intestinal function. They
usually require total parenteral nutrition to supply adequate caloric
intake until gut function returns (3 to 4 weeks). Intestinal atresia is
observed in 10% to 12% of neonates with gastroschisis. This is caused
by bowel ischemia due to intrauterine volvulus or compression of the
herniated viscera in a small, tight defect in the abdominal wall.
Although infants with omphalocele frequently have chromosomal
syndromes such as Beckwith syndrome or trisomy 13 to 15 or 16 to
18, babies with gastroschisis do not.

4. In neonates with congenital diaphragmatic hernia, which of the

following statements is true?
A. The defect is more common on the right side.
B. Survival is significantly improved by administration of pulmonary
vasodilators.
C. An oxygen index of 20 is an indication for extracorporeal
membrane oxygenation (ECMO).
D. Oligohydramnios is a frequent occurrence.
E. Mortality is the result of pulmonary hypoplasia.
Answer: E

DISCUSSION: In infants with congenital diaphragmatic hernia the

defect is more common on the left side (85%). Polyhydramnios is
sometimes noticed and is a poor prognostic indicator of survival.
Oligohydramnios is noted in fetuses with urinary tract obstruction and
may be associated with pulmonary hypoplasia with an intact
diaphragm. Although pulmonary vasodilators were used extensively
in babies with congenital diaphragmatic hernia, they have not
significantly improved survival. An oxygen index of greater than 40 is
the usual indication for ECMO. Pulmonary hypoplasia is the main
cause of mortality in babies with congenital diaphragmatic hernia.

5. Which of the following statements are true regarding the

premature neonate?
A. A 15% to 20% right-to-left shunt occurs across the foramen ovale
and patent ductus arteriosus.
B. Surfactant levels are normal after 30 weeks' gestation.
C. Fluid requirements are higher than in a full-term baby.
D. Rectal temperature is the best indicator of core body temperature.
E. They are more at risk for infection than the full-term infant.
Answer: ACE

DISCUSSION: The newborn infant has a relatively elevated pulmonary

artery pressure and shunts a significant amount of unoxygenated
blood through the foramen ovale and patent ductus arteriosus. The
normal PaO 2 below the ductus, as measured through an umbilical
artery catheter, would be between 60 and 80 mm. Hg. Surfactant
levels do not approach normal until after the 34th week of gestation,
when enzyme levels in the surfactant pathway mature. Amniocentesis
is performed to measure the lethicin-to-sphingomyelin ratio (L:S ratio)
and determine whether maturation has occurred. Fluid requirements
in the premature infant are between 140 and 150 ml. per kg. per day
in comparison with those of the normal neonate in whom 80 ml. per
kg. per day would be adequate. Increased insensible losses and the
need for overhead warmers play a role in this increase. Axillary or skin
probe temperature monitoring is more accurate than the rectal
temperature in the neonate. The rectal temperature is not a good
indicator of core body temperature until approximately 18 months of
age. Premature infants lack immunoglobulin A (IgA) and have low
levels of IgM, the C3b component of complement, and decreased
opsonins. In addition, the leukocytes have reduced phagocytic ability,
creating an increased risk of infection. Escherichia coli and
beta-hemolytic streptococcus are the two most common infectious
agents affecting the neonate.

6. In neonates with necrotizing enterocolitis, which of the following

findings is an indication of significant bowel ischemia?
A. Increased gastric residuals.
B. Septic shock.
C. Cardiac failure due to a patent ductus arteriosus.
D. Elevated platelet count.
E. Erythema of the abdominal wall.
Answer: E

DISCUSSION: Necrotizing enterocolitis (NEC) is a condition that occurs

in 2% of babies admitted to neonatal intensive care facilities.
Increased gastric residuals can occur for a number of reasons and are
seen as an early indicator of NEC, but they may not reflect the
presence of ischemic bowel. Septic shock may be due to a wide
variety of causes besides NEC. Cardiac failure due to patent ductus
arteriosus may predispose to NEC but is not necessarily an indicator
of ischemic bowel. Most babies with NEC have a progressive decrease
in their platelet count in association with bowel ischemia. Erythema of
the abdominal wall is an indication for surgical exploration and is
consistent with NEC with perforation and inflammation of the
peritoneum and abdominal wall.

7. The treatment of choice for neonates with uncomplicated

meconium ileus is:
A. Observation.
B. Emergency laparotomy, bowel resection, and Bishop-Koop
enterostomy.
C. Intravenous hydration and a gastrograffin enema.
D. Emergency laparotomy, bowel resection, and anastomosis.
E. Sweat chloride test and pancreatic enzyme therapy.
Answer: C

DISCUSSION: Meconium ileus is a form of intestinal obstruction that

occurs in 10% to 15% of neonates with cystic fibrosis. The obstruction
is related to intraluminal concretions of abnormal meconium. The
treatment of choice is adequate hydration and evacuation with a
hypertonic gastrograffin enema. The hyperosmolar contrast material
causes an outpouring of fluids into the bowel lumen, which flushes
out the obstructing meconium and negates the need for laparotomy.
Observation alone is not a useful method of treatment. When
gastrograffin evacuation fails, laparotomy, placement of a pursestring
suture in the bowel wall, and intraluminal irrigation with saline and
gastrograffin (administered through a catheter inserted through a
small enterotomy within the pursestring) will often clear the
obstructing meconium. This obviates the need for resection or
enterostomy in most cases. Postoperatively, a sweat chloride test
should be obtained to confirm the diagnosis of cystic fibrosis.
Pancreatic enzyme should be given when diet is initiated.

8. The pentalogy of Cantrell includes all of the following except:

A. Epigastric omphalocele.
B. Sternal cleft.
C. Intracardiac defect.
D. Pericardial cyst.
E. Ectopia cordis.
Answer: D

DISCUSSION: The pentalogy of Cantrell includes an epigastric-located

omphalocele, ectopia cordis, anterior pleuropericardial defect in the
diaphragm, sternal cleft, intracardiac defect (most commonly a
ventricular septal defect), and in approximately one third of the cases
a diverticulum of the left ventricle. Pericardial cysts are not part of the
pentalogy.

9. In infants with duodenal atresia all the following statements are

true except:
A. There is an increased incidence of Down syndrome.
B. Duodenal atresia can be detected by prenatal ultrasound
examination.
C. It may occur in infants with situs inversus, malrotation, annular
pancreas, and anterior portal vein.
D. It is best treated by gastroenterostomy.
E. There is a high incidence of associated cardiac defects.
Answer: D
DISCUSSION: The diagnosis of duodenal atresia can be made prior to
the infant's birth with a prenatal ultrasound examination. Infants with
duodenal atresia are often premature and have a high incidence of
associated anomalies, especially congenital heart disease. Duodenal
atresia may also coexist in patients with annular pancreas, situs
inversus, malrotation, and anterior portal vein. Approximately one
third of the cases occur in babies with Down syndrome. The operative
treatment of choice is a duodenoduodenostomy.
Duodenojejunostomy is an alternative procedure. Gastrojejunostomy
is not recommended.

10. The initial treatment of choice for a 2.5-kg. infant with a 20.0-cm.
long proximal jejunal atresia and 8.0 cm. of distal ileum is:
A. Laparotomy, nasogastric suction, proximal dilatation to lengthen
the atretic jejunum, total parenteral nutrition, and delayed
anastomosis.
B. Laparotomy and proximal end-jejunostomy.
C. Laparotomy and immediate small bowel transplantation.
D. Laparotomy and double-barrel enterostomy (jejunum and ileum),
with refeeding of jejunal contents into distal ileum and delayed
anastomosis.
E. Laparotomy, tapering jejunoplasty, and end-to-oblique jejunoileal
anastomosis.
Answer: E

DISCUSSION: The patient has short bowel syndrome with most of the
bowel length involving the dilated proximal jejunal atresia. The
treatment of choice is to perform a tapering jejunoplasty to preserve
bowel length and construct an anastomosis. Early feedings are
initiated when bowel function returns in order to stimulate bowel
adaptation. Jejunal dilatation will not significantly lengthen the atretic
jejunum and will not alter its abnormal motility. End-jejunostomy
decompresses the obstruction but produces a high ostomy with
excessive loss of succus entericus. A double-barrel enterostomy might
allow refeeding of jejunal content into the distal ileum and colon, but
the proximal atretic loop may have poor function. Small bowel
transplantation is not a feasible alternative in the neonate at the
present time.

11. A 2.8-kg. neonate with excessive salivation develops respiratory

distress. Attempts to pass an orogastric catheter fail because the
catheter coils in the back of the throat. A chest film is obtained and
shows right upper lobe atelectasis and a gasless abdomen. The most
likely diagnosis is:
A. Proximal esophageal atresia without a fistula.
B. Proximal esophageal atresia with a distal tracheoesophageal (TE)
fistula.
C. “H-type” TE fistula.
D. Esophageal atresia with both proximal and distal TE fistula.
E. Congenital esophageal stricture.
Answer: A

DISCUSSION: Proximal esophageal atresia will result in excess

salivation and aspiration of saliva. It is often associated with right
upper lobe collapse. Infants with a distal TE fistula will have air in the
stomach and intestine, as will babies with both proximal and distal
fistulas, “H-type” fistula, and an esophageal stricture. Infants with
proximal atresia but without a TE fistula will not have air beneath the
diaphragm. Attempts at passing an orogastric catheter will be met by
an obstruction and coiling of the catheter in the infant's mouth. The
catheter will pass into the stomach in infants with “H-type” TE fistula
and in most with esophageal stricture.

12. Neonates with NEC may demonstrate all of the following findings
on abdominal films except:
A. Pneumatosis intestinalis.
B. Portal vein air.
C. Pneumoperitoneum.
D. Colovesical fistula.
E. Fixed and thickened bowel loops.
Answer: D

DISCUSSION: Infants with NEC do not develop a colovesical fistula as

an initial x-ray finding. Pneumatosis, portal vein air,
pneumoperitoneum, and fixed intestinal loops with thickened bowel
wall are all observed with some regularity in babies with NEC.

13. The most common type of congenital diaphragmatic hernia is

caused by:
A. A defect in the central tendon.
B. Eventration of the diaphragm in the fetus.
C. A defect through the space of Larrey.
D. An abnormally wide esophageal hiatus.
E. A defect through the pleuroperitoneal fold.
Answer: E

DISCUSSION: Eventration of the diaphragm is related to phrenic nerve

paralysis. It is more commonly observed after a breech delivery and
may be associated with torticollis and Erb's palsy. The space of Larrey
is located anteriorly just off the midline. A Morgagni hernia passes
through this potential space. An abnormally wide esophageal hiatus
would most likely create a sliding hiatal hernia. The most common
type of congenital diaphragmatic hernia in the neonate is the
posterolateral Bochdalek hernia, which passes through a defect in the
developing pleuroperitoneal fold.

14. The calorie-nitrogen ratio for an infant should be maintained at:

A. 75:1.
B. 100:1.
C. 50:1.
D. 150:1.
E. 25:1.
Answer: D

DISCUSSION: The calorie-nitrogen ratio should be maintained at 150:1

for most infants. Fever, major illness, sepsis, or trauma may increase
the caloric requirements significantly.

15. All of the following conditions are derived from the primitive
embryonic foregut except:
A. Bronchogenic cyst.
B. Cystic adenomatoid malformation.
C. Gastric duplication.
D. Mesenteric cyst.
E. Pulmonary sequestration.
Answer: D

DISCUSSION: Mesenteric cysts are derived from lymphatic anlage in

the abdomen and are unassociated with foregut development. The
lung buds arise from the primitive foregut and anomalies associated
with tracheopulmonary development are therefore all derived from
foregut. These include tracheoesophageal fistula, congenital lobar
emphysema, enteric cysts (which may communicate to the normal
esophagus, lung, or spinal canal; e.g., neurenteric cyst), cystic
adenomatoid malformations, solitary lung cysts, intra- and extralobar
sequestrations, and bronchogenic cysts. The stomach and first part of
the duodenum are also of foregut origin, and thus a gastric
duplication is by definition derived from foregut.

16. For a 22-kg infant, the maintenance daily fluid requirement is

approximately which of the following?

a. 1100 ml
b. 1250 ml
c. 1550 ml
d. 1700 ml
e. 1850 ml
Answer: c

Maintenance water and electrolyte requirements are illustrated in the

table indicated below. The volume calculation is demonstrated. The
composition of the intravenous fluids is generally that of D5 1/4
normal saline or D5 1/2 normal saline with 10 mEq/l of KCL.

17. Which of the following statements regarding nutritional

requirements in infants are true?

a. The total daily water requirement is estimated to be 100 ml/100

kcal ingested
b. The resting energy expenditure is approximately twice that of an
adult
c. The highest rate of nitrogen retention with parenteral nutrition
occurs in infants given approximately 40% of the calories as
carbohydrate and the remainder as fat
d. The protein requirement for a newborn infant is approximately 2.5
g/kg/day
Answer: a, b, c, d

Taking all factors into account, total daily water requirements for a
term infant are estimated to be 100 ml/100 kcal ingested, assuming
an insensible loss of 50 ml/kg/day and a growth requirement of
approximately of 15 ml/kg/day. The energy expenditure of normal
neonates is approximately twice that of normal adults (50 kcal/kg/day
versus 25 kcal/kg/day).
In most circumstances, high carbohydrate/low fat ratios in parenteral
nutrition result in high rates of energy expenditure and decreased
nitrogen retention, while low carbohydrate/high fat ratios result in
excessive fat deposition. A balanced ratio (approximately 40%
carbohydrate) provides the highest rate of nitrogen retention, and is
consistent with the proportion of carbohydrate found in breast milk
and with the estimates of minimal carbohydrate needs determined by
isotope infusion studies. A consensus statement by the World Health
Organization and the United Nations University estimates the protein
requirement at 2.5 g/kg/day for an infant and 1.25 g/kg/day for a
one-year-old child. For preterm infants, the protein need ranges from
2.5 to 3.9 g/kg/day if the weight is less than 2.5 kg.

18. A term infant 48 hours of age suddenly develops hypoxemia,

irritability, and glucose and temperature instability. Which of the
following statements are true?

a. Empiric antibiotic coverage for b-hemolytic Streptococci and

Escherichia coli should be initiated
b. An intravenous infusion of prostaglandin E1 should be initiated
immediately
c. Exogenous surfactant should be given immediately
d. The mortality rate for this child is approximately 50%
Answer: a,d

This infant has the classical findings of neonatal sepsis. This is defined
as a generalized bacterial infection accompanied by a positive blood
culture during the first month of life. Early onset sepsis occurs during
the first week of life, and is due primarily to maternal organisms, such
as b-hemolytic Streptococci, Escherichia coli or Listeria
monocytogenes. The mortality rate of early onset sepsis is
approximately 50 percent. Late onset sepsis is due primarily to
hospital acquired organisms such as Staphylococcus epidermidis,
Staphylococcus aureus or Pseudomonas species, and the mortality
rate for this entity is approximately 20 percent.
The signs and symptoms of neonatal sepsis are subtle and
nonspecific. Early signs include lethargy, irritability, temperature
instability, change in the respiratory pattern, or changes in the feeding
pattern. Hematologic findings include thrombocytopenia,
leukocytosis, or leukopenia. Hemodynamic manifestations occur late.
Presumptive therapy should be based upon the suspected organism,
but often includes Ampicillin or an anti-Staphylococcal agent plus an
amino glycoside.
A prostaglandin E1 infusion is inappropriate as this relates to patients
with ductal-dependent congenital heart disease. Exogenous
surfactant is unlikely to be helpful in a full-term infant who has
previously been well and can be expected to begin his illness with a
normal complement of pulmonary surfactant.

19. Which of the following statements about pulmonary surfactant are

true?

a. Endogenous surfactant deficiency is the key physiologic problem in

preterm infants with the infant respiratory distress syndrome
b. Surfactant function can be restored to normal using aerosolized
phosphatidylcholine administration
c. Exogenous surfactant replacement has been shown to reduce
mortality in preterm infants with the infant respiratory distress
syndrome
d. Surfactant is produced by Type I alveolar epithelial cells
Answer: a, c

The pulmonary surfactant complex lowers surface tension, stabilizing

the alveolus even at low lung volumes. It is a complex material
secreted by Type II alveolar epithelial cells. It is composed of 80 to 90
percent phospholipid (primarily phosphatidylcholine) and unique
surfactant-associated proteins (10 percent). Surfactant proteins
appear to play a critical role in the organization of the phospholipid
molecules, and modify the surface-active properties of the lipids.
Phospholipid synthesis and the expression of surfactant proteins
increase with advancing gestational age. Amniotic fluid surfactant
concentrations have been used for many years to predict pulmonary
maturity. Surfactant deficiency is the primary factor in the
pathophysiology of the neonatal respiratory distress syndrome. The
use of exogenous surfactant replacement therapy is currently under
investigation for the treatment of neonatal respiratory distress and
has been shown to reduce mortality in a variety of specific
circumstances involving preterm infants. Several commercially
available preparations are available and are undergoing clinical
investigation.

20. Which of the following statements regarding premature infants

are true?

a. Complications of prematurity account for approximately 85% of

fetal deaths
b. Prematurity is defined by the World Health Organization as birth
prior to 35 weeks gestation
c. Infants with intrauterine growth retardation have physiologic
problems which are more dependent on the birth weight than the
gestational age
d. Preterm infants are at increased risk for hypocalcemia and
hypoglycemia when compared to term infants
Answer: a, d

Prematurity is defined by the World Health Organization as a

gestational age at birth of less than 37 weeks. Complications of
prematurity account for approximately 85% of fetal deaths. These
deaths are commonly due to perinatal asphyxia, respiratory failure
and infection. The term intrauterine growth retardation describes a
pathophysiologic process that results in restriction of fetal growth.
Fetal, placental or maternal abnormalities are common. These infants
are a heterogeneous population and they tend to have neonatal
problems related more to their gestational age than to their birth
weight. These problems include asphyxia, hypoglycemia,
hypothermia, hypocalcemia, pulmonary hemorrhage, necrotizing
enterocolitis and other complications related to specific syndromes or
congenital anomalies.

21. Other than the history and physical exam, which of the following
tests is considered an essential feature of the preoperative evaluation
of a patient with a suspected thyroglossal duct cyst?

a. Cervical ultrasound
b. Thyroid scan
c. Serum T3 and T4 levels
d. Needle aspiration
e. None of the above
Answer: e

A thyroglossal duct cyst is typically a midline structure connected to

the foramen cecum at the base of the tongue, that is pulled proximal
and superior as the tongue protrudes. It may be superior or inferior
to the hyoid and is occasionally slightly off the midline. Because the
thyroglossal cyst may rarely contain the patient’s only thyroid tissue,
some have recommended a technetium-99m radioisotope thyroid
scan before excision. However, excision of the cyst is indicated
regardless, because infection of the cyst is likely, and the dysgenetic
thyroid tissue in the cyst has malignant potential. For these reasons,
patients with suspected thyroglossal duct cysts require routine
surgical excision. Preoperative ultrasound, thyroid scan, T3 and T4
levels, or needle aspiration is not necessary. For those patients who
have thyroid tissue in their cysts by pathologic examination,
postoperative thyroid function tests identify those who have no
remaining thyroid tissue and replacement therapy can be prescribed.

22. Suppurative cervical lymphadenitis in a 3-year-old child is

commonly related to which of the following organisms?

a. Staphylococcus aureus
b. Atypical mycobacterial organisms
c. Streptococcal organisms
d. Lymphoma with secondary pyogenic organisms
e. Cat scratch
Answer: a, c

Acute suppurative lymphadenitis related to bacterial pathogens is

generally straightforward to diagnose. There is often accompanying
infectious illness. The lymph nodes enlarge rapidly, are tender and
erythema of the overlying skin is present. Fever and an elevated white
blood count with a left shift are usually present. Fluctuant nodes may
be aspirated. Streptococcus and Staphylococcus aureus are the most
common organisms and the initial course of antibiotic therapy is
directed to these organisms. If the adenopathy fails to resolve in 2 to
3 weeks, then the patient should likely undergo an excisional biopsy.
Atypical Mycobacteria, cat scratch disease, and Mycobacterium
tuberculosis are more uncommon than bacterial lymphadenitis and
do not typically produce tenderness or systemic signs. Lymphoma
with secondary pyogenic infection is similarly uncommon.

23. Branchial cleft remnants most often present with which of the
following clinical problems?

a. Infection
b. Airway obstruction
c. Hemorrhage
d. Malignant degeneration
e. Pain
Answer: a

The more common second branchial cleft anomalies present typically

as a pinpoint opening on the anterior border of the
sternocleidomastoid muscle. Attention is drawn to the defect often by
the appearance of small drops of clear fluid at the orifice or by
occurrence of infection in the tract itself. Less frequently, a mass may
present anterior to the upper portion of the sternocleidomastoid
muscle representing a cyst derived from this tract. Infection is a more
common problem in the older age group. Airway obstruction and
hemorrhage are rare presentations. Pain is usually secondary to
infection and malignant degeneration is reported but exceedingly
rare. Treatment consists of surgical excision. If infection is present, a
course of antibiotics is administered first.

24. Proximity to which of the following structures places it at risk

during surgical excision of a second branchial cleft remnant?

a. Internal carotid artery

b. External carotid artery
c. Hypoglossal nerve
d. All of the above
e. None of the above
Answer: d

Operative excision of a second branchial cleft remnant begins with an

elliptical transverse incision at the sinus opening and cephalad
dissection of the tract to its furthest extent, generally reaching the
floor of the tonsillar pillar. The dissection is kept directly on the tract
to avoid injury to contiguous structures such as the internal jugular
vein, the internal or external carotid arteries (between which it
passes), and the hypoglossal nerve. The operation can almost always
be carried out through a single cosmetic incision if the tract is kept
under traction and digital pressure is exerted through the tonsillar
fossa. Dissection of the sinus tract may be facilitated by passing a fine
silver probe or piece of heavy nylon suture through the length of the
tract.

25. Standard therapy for acute epiglottitis in a child is:

a. Tracheostomy
b. Intravenous antibiotic treatment in an ICU setting
c. Endotracheal intubation in the operating room and intravenous
antibiotic therapy
d. Indirect laryngoscopy and intravenous antibiotics
e. Intravenous steroids and antibiotics
Answer: c

Acute epiglottitis is a common cause of acquired airway obstruction in

the pediatric age group. Haemophilus influenzae B is nearly always
the causative organism, and most children are toxic at presentation
with an elevated temperature and an increased pulse and respiratory
rate. Prolonged inspiratory stridor that worsens in the supine position
is characteristic. The child usually sits erect, anxious and drooling and
becomes increasingly exhausted with air hunger. No attempt should
be made to visualize the larynx outside of the operating room for fear
of sudden airway occlusion. The standard therapy is short-term
endotracheal intubation performed in the operating room with
general anesthesia. The inflammatory process resolves rapidly with
intravenous antibiotics and intubation is seldom required beyond 3
days. In the past, tracheostomy was the standard therapy, but
comparative reviews demonstrate that short-term endotracheal
intubation is associated with less morbidity and fewer complications.

26. Which of the following statements regarding congenital

diaphragmatic hernia are true?

a. The incidence of right and left-sided lesions is equal

b. Malrotation is to be expected
c. Left-to-right shunting via a patent ductus arteriosus is a serious but
expected physiologic consequence of pulmonary hypoplasia
d. Survival rates of 75% are reported in several contemporary series
e. Congenital heart disease is present in approximately 20% of these
infants
Answer: b, d, e

During organogenesis closure of the right hemidiaphragm normally

precedes that of the left. This asynchronous closure and the presence
of the liver on the right account for the finding that 85% to 90% of
congenital diaphragmatic hernias involve the left hemidiaphragm.
Malrotation is an expected finding with diaphragmatic hernia because
intestinal herniation into the thorax normally precedes the fixation of
the gut to the posterior body wall. Pulmonary hypertension is a major
feature of congenital diaphragmatic hernia and right-to-left shunting
via a patent ductus arteriosus is present in virtually all of these
children. Fifteen to 25% of infants with a diaphragmatic hernia have
an associated anomaly, the most important being cardiovascular
abnormalities. Although ventricular septal defects and aortic
coarctations are most common, virtually all cardiac and great vessel
anomalies have been reported. Cardiac ECHO screening examinations
are therefore routine. Survival rates as high as 75% to 90% in selected
high-risk congenital diaphragmatic hernia patients have been
reported in several large clinical series over the last five to ten years.
This is an apparent improvement from the historic range of 50%.

27. Of the following cystic malformations of the tracheobronchial tree,

which is most likely to be asymptomatic when discovered?

a. Intralobar pulmonary sequestration

b. Extralobar pulmonary sequestration
c. Congenital cystic adenomatoid malformation
d. Congenital lobar emphysema
Answer: b

Intralobar pulmonary sequestration and cystic adenomatoid

malformations typically present with either neonatal respiratory
distress or infection related to inadequate clearance of secretions.
Given enough time, nearly all of these lesions will become infected.
Congenital lobar emphysema is characterized by air-trapping within
an otherwise normal lung. This typically presents with respiratory
distress which ranges from mild to life-threatening. Hemodynamic
instability requiring emergency thoracotomy is occasionally present.
Extralobar sequestration is typically a mass of disorganized
pulmonary parenchymal tissue within its own investing pleura and
outside of the normal lung parenchyma. This does not communicate
with the normal tracheobronchial tree. Infection is rare and although
hemorrhage, arterial venous shunting, mediastinal compression and
occasional malignancy may occur, these lesions are typically
asymptomatic and indeed often discovered via prenatal ultrasound.
Excision is recommended for each of these lesions, typically involving
a lobectomy.

28. Infants with a double aortic arch most commonly present with
which of the following problems?

a. Dysphagia
b. High output cardiac failure related to a patent ductus arteriosus
c. Positional hyperemia and edema of the right upper extremity
d. Symptomatic tracheal compression
Answer: d

The double aortic arch represents the most common type of complete
vascular ring. It arises from the ascending aorta and bifurcates, with
one arch passing on either side of the trachea and the esophagus. The
symptoms of complete vascular rings are due to compression of the
trachea, the esophagus or both. The child with a double aortic arch is
generally the most symptomatic and most patients have symptoms in
infancy. The typical clinical picture is one of symptomatic tracheal
compression and includes inspiratory wheezing, coughing, noisy
breathing, shortness of breath, stridor and frequent bouts of
pneumonia. Feeding problems may become apparent when solid
foods are started but this is less common than tracheal compression.
The most important screening test is the barium swallow, and CT or
MRI are definitive. Angiography and endoscopy are not usually
helpful. Any patient who is symptomatic from a vascular ring should
be treated surgically.

29. Which of the following is most common after primary

esophagostomy for esophageal atresia with a distal
tracheoesophageal fistula?

a. Anastomotic leak
b. Esophageal stricture
c. Recurrent tracheoesophageal fistula
d. Gastroesophageal reflux
e. Tracheomalacia requiring aortopexy
Answer: d

The preferred approach for esophageal atresia with a distal

tracheoesophageal fistula in a patient without other problems is an
extrapleural right thoracotomy with division of the tracheoesophageal
fistula and primary esophagostomy. No gastrostomy is ordinarily
used. The results with this approach are better than those with a
staged approach. Three major complications are related to the
esophageal anastomosis: leak, stricture and recurrent fistula. The
incidence of leak varies from 10% to 20% depending on the type of
anastomosis done and the degree of tension. A distinct advantage of
an extrapleural anastomosis is the predictable resolution of these
leaks if adequately drained. Similarly, the stricture rate varies between
10% and 25%, again depending on the type of anastomosis done.
Many infants require one or two dilations, but few have significant
long-term problems. The incidence of recurrent esophageal fistula is
difficult to determine since few authors emphasize this technical
problem, but it appears to be about 10% in most reports.
Gastroesophageal reflux secondary to dysmotility of the distal
esophagus is a significant problem and occurs to some degree in
virtually all of these patients. A significant number of these infants,
25% to 30% or more, are refractory to medical therapy and require
surgical fundoplication. Tracheomalacia is a complication of the
malformation, not of the repair. This appears to result from
inadequate cartilagenous tracheal rings at the level of the fistula. The
reported incidence is up to 25% in some recent series. Most infants
with tracheomalacia improve with growth and time, but a small
percentage develop severe respiratory difficulty which requires
surgical aortopexy.

30. Which of the following is the most common primary lung tumor in
infants and children?

a. Pulmonary blastoma
b. Squamous cell carcinoma
c. Endobronchial carcinoid
d. Leiomyoma
e. Metastatic osteogenic sarcoma
Answer: c

Bronchial adenomas are the most common primary lung tumors in

childhood. Nevertheless, they are quite rare. Typically these are
low-grade adenocarcinomas that include endobronchial carcinoids,
cylindromas, mucoepidermoid tumors, and bronchomucous gland
adenomas. Carcinoid tumors are the most common bronchial
adenomas and represent over 80% of the total.
Although common in adults, bronchogenic carcinoma of the lung is
extremely rare in childhood. A review of the world’s literature in 1983
revealed only 47 such patients. Pulmonary blastoma is a rare
malignant lung tumor composed of cells that resemble fetal lung. Its
incidence is actually highest in adults, although it is reported in
children as well. Benign tumors of the lung are also rare in childhood.
The most common of these are pulmonary hamartomas or
chondromas. Others include leiomyoma, leiomyoblastoma and mucus
gland adenoma. Metastatic osteogenic sarcoma is more common
than any primary lung tumor, but is by definition a secondary
metastatic lesion and therefore not the correct answer to the
question posed here.

31. Which of the following statements regarding congenital chest wall

deformities are true?

a. Children with pectus excavatum deformities typically have

physiologically insignificant limitation of exercise tolerance
b. The rate of recurrence after operative repair of a pectus excavatum
deformity is between 5% and 10%
c. Pectus carinatum is the most common congenital chest wall defect
d. The most common indication for operative repair of congenital
chest wall deformities is cosmesis
Answer: a, d

The most common congenital chest wall deformity is pectus

excavatum, representing approximately 90% of the total.
Approximately 5% to 7% of the lesions are pectus carinatum and a
variety comprise the remainder. Most children with pectus excavatum
are asymptomatic. There have been numerous efforts to document
associated cardiac and pulmonary abnormalities. Objective data show
that although there are minor demonstrable cardiopulmonary
abnormalities demonstrable, these do not appear to be significantly
improved by surgery and they are generally insignificant
physiologically. As a result, the indications for repair of chest wall
deformities are essentially cosmetic and psychological. The
importance of these however should not be minimized in a largely
adolescent population. The repair is technically straightforward but
involves moderate morbidity. The long-term results are excellent.
Recurrence is rare (approximately 1%) in most institutions with large
experiences.

32. The definitive evaluation of a child with a suspected congenital

cystic abnormality of the tracheobronchial tree is best done using
which of the following?

a. Rigid bronchoscopy
b. Computerized tomography or magnetic resonance imaging
c. Chest x-ray
d. Angiography
e. Barium esophagogram
Answer: b
Plain film radiography is the first imaging study performed and
remains a cornerstone for the diagnosis and follow-up of this group of
lesions. The use of additional imaging provides definitive diagnosis
and allows planning for the surgical approach as well. Computed
tomography (CT) and magnetic resonance imaging (MRI) can separate
cystic from solid components in a radiopaque lung mass. These are
the most definitive diagnostic studies available. The MRI has
reconstructive capabilities that obviate the need for angiography.
Intravenous contrast with CT scan provides similar anatomic
information. Ultrasonography is less costly, more readily performed
and in select cases may be as sensitive.
Angiography is not employed regularly because these alternative
imaging strategies provide similar information at lower cost with less
morbidity. A barium esophagogram is helpful in the diagnosis of
children with dysphagia but that is a rare presentation for these
lesions. Bronchoscopy is rarely helpful for these lesions and in these
infants and small children carries the risk of general anesthesia and
positive pressure ventilation. In children with congenital lobar
emphysema and cystic adenomatoid malformation, hyperinflation
following positive pressure may induce mediastinal compression and
create a surgical emergency. For these reasons, CT or MRI are
considered the best definitive diagnostic imaging choices after the
initial chest x-ray.

33. A newborn infant develops coughing, choking and cyanosis with

his first feeding. He is noted to have excessive drooling. What are the
important associated anomalies that must be screened for prior to
surgical intervention?

a. Right-sided aortic arch

b. Hydrocephalus
c. Genitourinary obstruction
d. Congenital heart disease
Answer: c

This child has a classical history for esophageal atresia and has a very
high (90% or more) probability of a distal tracheoesophageal fistula.
The simplest way to establish the diagnosis is to attempt to pass a
catheter through the mouth or nose into the stomach. If the tube
encounters obstruction, a plain radiograph should document the
atresia.
Patients with esophageal atresia and tracheoesophageal fistula
frequently have associated anomalies. This incidence is about 30% to
50% in most reports. Anomalies vary from minor skeletal deformities
to uncorrectable cardiac defects. The most common associated
anomalies are cardiac and gastrointestinal, especially imperforate
anus (10%). Vertebral, genitourinary and limb anomalies are also
seen. Importantly, approximately 5% of patients with esophageal
atresia have a right-sided aortic arch. This is an important technical
issue as the normal approach is via a right thoracotomy and this
should be changed to a left thoracotomy in the presence of this
finding. There is no association with hydrocephalus.

34. Which of the following ventilation strategies is the best initial

approach for a neonate with a left congenital diaphragmatic hernia
and the following post ductal arterial blood gases: PaO2 50 mm Hg,
PaCO2 60 mm Hg, pH 7.35?

a. High-frequency jet ventilation

b. Permissive hypercapnia with convential pressure controlled
ventilation
c. Extracorporeal membrane oxygenation (ECMO)
d. Induced respiratory alkalosis
e. Inhaled nitric oxide with conventional volume controlled ventilation
Answer: b

Contemporary congenital diaphragmatic hernia management

emphasizes permissive hypercapnia using any necessary mode of
respiratory support. Ordinarily, pressure controlled ventilation is the
initial mode of support. The purpose is to reduce the iatrogenic lung
injury associated with high-pressure mechanical ventilation. It is this
latter problem, barotrauma-induced lung injury, which has reduced
enthusiasm for induced respiratory alkalosis. Although alkalosis can
sometimes be achieved, the price is often prohibitive mean and peak
airway pressures. High-frequency jet ventilation, ECMO and inhaled
nitric oxide are all evolving strategies which must be considered
developmental and are reserved for use after the initial strategy is
unsuccessful.

35. There is an emerging consensus that the surgical repair for

congenital diaphragmatic hernia is best done:

a. Emergently at the bedside, eliminating the risks of transporting an

unstable neonate
b. While on extracorporeal membrane oxygenation
c. When the infant is potentially extubatable
d. Within the first 48 to 72 hours of life
Answer: c

Infants with diaphragmatic hernias have considerable variation in the

degree of respiratory distress, and the degree of distress dictates
timing of repair. Traditionally, infants were rushed emergently to the
operating room for reduction of the herniated viscera and
diaphragmatic closure. Because effective preoperative decompression
of the intestine is usually possible and because it has become clear
that the underlying pulmonary hypoplasia is not reversed by
emergency operation, this sense of urgency is no longer accepted.
Most surgeons now commit themselves to a period of preoperative
stabilization which is used to confirm the diagnosis and optimize
medical care. The current recommendation is that operative repair be
undertaken in a stable patient who is nearing extubatable levels of
ventilatory support, regardless of the means of support which have
been employed. This means that repair is done at or after the end of
cardiopulmonary bypass in infants where that is necessary. The
results with a delayed approach appear at least equivalent and in
several series better than with emergent or early repair. The concept
is that the deferral of the iatrogenic operative injury to a time when
the neonatal pulmonary vascular bed is less vulnerable to vasospasm
is desirable.

36. Meckel’s diverticulum may present with which of the following

signs or symptoms?

a. Hemorrhage
b. Intussusception
c. Volvulus
d. Patent omphalomesenteric duct
e. Right lower quadrant peritoneal findings
Answer: a, b, c, d, e

The most frequent congenital anomaly of the GI tract is a Meckel’s

diverticulum. The incidence is about 2% of the general population,
many of whom remain asymptomatic throughout life. Estimations of
the frequency with which symptoms develop among people with
Meckel’s diverticula vary from 4% to 30%, but is clear that the risk
diminishes substantially with age. About half of those who become
symptomatic are under the age of 2. Hemorrhage, acute diverticulitis,
perforation, and small bowel obstruction or intussusception are all
classic presenting scenarios for a child with a Meckel diverticulum.
(Table) The approximate frequency of these presentations is shown
above. Below are illustrations of the various anatomic abnormalities
with their associated clinical presentations. (Figure) These
presentations include Meckel’s diverticulitis which is virtually
indistinguishable from acute appendicitis.
SIGNS AND SYMPTOMS OF MECKEL’S DIVERTICULUM
Clinical Presentation Approximate Frequency (%)
Hemorrhage 30–35
Small bowel obstruction 30–35
Diverticulitis 20–25
Umbilical fistula 10
Other Uncommon

Normal embryologic relations of the embryonic yolk sac, yolk stalk,

and gut.

37. A 3-week old infant has a barium upper gastrointestinal series to

evaluate vomiting. The duodenojejunal flexure is found to be to the
right of the midline as well as more caudal and anterior than a normal
ligament of Treitz. The child is seen to reflux barium spontaneously to
the level of the mid-thoracic esophagus. You would recommend which
of the following?

a. Barium enema
b. Emergency laparotomy
c. A trial of H2, blockade and cisapride therapy
d. Upper gastrointestinal endoscopy
e. Overnight pH probe analysis
Answer: b

This child has malrotation and is at risk for midgut volvulus based on
the imaging findings. Typically, malrotation produces an incomplete
obstruction of the duodenum with a corkscrew or coiled appearance
in the third or fourth portions of the duodenum. Malposition of the
duodenojejunal junction is diagnostic. In particular, this includes a
location to the right of the midline. Additionally, failure to achieve
normal posterior and cephalad fixation is typical. The small bowel
resides in the right abdomen and the colon and cecum are on the left.
Attempts to radiographically differentiate malrotation with or without
volvulus are unreliable and therefore hazardous. This child has
gastroesophageal reflux which is likely secondary to the partial
duodenal obstruction from malrotation. None of the alternatives
other than emergency laparotomy are appropriate. There is no role
for nonoperative management of malrotation in the neonate.
Assessment, resuscitation and preoperative preparation should be
conducted concurrently as the child is prepared for laparotomy. This
urgency is because a delay measured in hours may represent the
difference between a viable or infarcted midgut at laparotomy. Fifty to
75% of malrotations are discovered in the first month of life and
about 90% occur in children less than one year of age.

38. Which of the following statements regarding duodenal atresia are

true?

a. 20% to 40% of these infants have Trisomy 21

b. When associated with an annular pancreas, division of the pancreas
at the site of obstruction is curative
c. Bilious vomiting is typical because the obstruction is usually distal to
the ampulla of Vater
d. Reconstruction is best achieved with Roux-en-Y
duodenojejunostomy
Answer: a, c

Twenty to 40% of infants with congenital duodenal obstruction have

Trisomy 21. Because this syndrome is not always apparent during the
physical examination, a routine karyotype should be obtained. A
preoperative ECHO ultrasound examination is also appropriate to
evaluate the possibility of associated congenital heart disease.
Feeding intolerance and bilious vomiting in the first 24 to 48 hours of
life are characteristic. The malformations are typically distal to the
ampulla of Vater and thus the infants present with bilious vomiting
and proximal duodenal and gastric distention. Malformations
proximal to the ampulla of Vater result in nonbilious vomiting and this
possibility must not be ignored.
Generally, bypass of the obstructing lesion is the best approach
regardless of whether an atresia or annular pancreas is responsible.
Division of an annular pancreatic band is inappropriate for two
reasons: 1) The duodenum is virtually always atretic in addition. 2)
Division of this pancreatic tissue necessarily divides the accessory
pancreatic duct within it creating the real possibility of a pancreatic
fistula. Generally, the construction of a duodenostomy that minimizes
the length of defunctionalized duodenum is preferred. The procedure
generally employed is a diamond-shaped duodenostomy. Simple
duodenojejunostomy without a Roux-en-Y is occasionally necessary
for lesions in the distal duodenum.

Diamond-shaped duodenoduodenostomy.

39. A 1500-gram, 30-week gestation neonate is fed at 2 weeks of age.

He develops abdominal distention, bilious vomiting and guaiac-
positive stool. A plain film of the abdomen demonstrates
pneumotosis intestinalis. Which of the following related statements
are true?

a. An emergency barium upper GI series should be done to rule out

malrotation
b. The child should have a nasogastric tube placed, broad spectrum
intravenous antibiotics begun, and sequential abdominal films
obtained.
c. The likelihood of intestinal perforation is in excess of 50%
d. The expected survival rate is in the range of 70%
Answer: b, d

This clinical history is classical for neonatal necrotizing enterocolitis

(NEC), an idiopathic clinical condition characterized by mucosal
intestinal injury that may progress to transmural bowel necrosis.
Typically it occurs in critically ill, preterm infants and is characterized
by abdominal distention, bilious vomiting and either occult or gross
blood in the stool. In this setting, pneumotosis intestinalis is
pathognomonic. When the diagnosis is suspected on clinical grounds
and the plain film, no contrast imaging should be done as this may
complicate or contribute to the problems of perforation. In particular,
the child presented here has an unequivocal history and an upper GI
series would be inappropriate.
Half of all infants with NEC have birth weights less than 1500 g, and
80% of these infants weigh less than 2500 g at birth. The incidence is
approximately 1 to 2 in 1000 live births. It is the most common
surgical emergency in neonates in North America. The initial
management of infants with NEC consists of nasogastric
decompression; broad-spectrum antibiotic administration; and
correction of hypoxemia, hypotension, acidosis, fluid or electrolyte
disorders, and other reversible medical problems. Up to 90% of
infants with NEC can be managed successfully nonoperatively, but this
is widely variable among institutions because of differences in referral
and practice patterns. Intestinal perforation is characterized by
pneumoperitoneum and is an indication for operation. Although the
incidence of perforation is variable, it is generally less than 20% to
40%.
The overall survival rate for neonates with NEC is about 60% to 70%
for both operative and nonoperative management groups. This
represents a substantial improvement from the 20% to 30% survival
probability when the entity was first recognized 30 to 40 years ago.

40. The most common cause of pyogenic liver abscess in children

today is which of the following?

a. Perforated appendicitis
b. Blunt liver injury
c. Immunocompromised host
d. Percutaneous liver biopsy
e. Omphalitis
Answer: c
In the preantibiotic era, pyogenic hepatic abscesses occurred most
frequently after perforated appendicitis. This complication is rarely
seen today. Chronic granulomatous disease of childhood is a principle
condition associated with hepatic abscess. This disease is the result of
deficient oxidant-mediated bacterial killing by circulating granulocytes.
In the pediatric age group, 40% of pyogenic liver abscesses occur in
children with chronic granulomatous disease, and another 30% occur
in children with other immunodeficiencies, most commonly leukemia.
Other rare causes of liver abscesses in pediatric patients are umbilical
vein catheter-induced infection, omphalitis and other biliary disease.
Pyogenic liver abscesses following blunt liver injury or percutaneous
liver biopsy are distinctly rare events.

41. Which of the followings statements regarding an infant with

meconium ileus are true?

a. The probability is 100% that he will have cystic fibrosis

b. Nonoperative therapy resolves this problem in approximately
two-thirds of patients
c. The average life expectancy is approximately 26 to 28 years for this
infant at present
d. The finding illustrated below on plain film is an absolute operative
indication (Figure 103-23A)
Answer: a, b, c, d

Meconium ileus refers to the characteristic obstruction of the small

intestine in neonates with cystic fibrosis (CF). Some 10% to 20% of
infants with CF present initially with meconium ileus. All infants with
meconium ileus have cystic fibrosis. CF is characterized by a transport
defect of epithelium that results in impermeability of the chloride ion
and therefore water. Inspissated secretions in the pancreas and gut
lead to obturator obstruction of the terminal ileum from meconium in
the neonate. Approximately two-thirds of these infants have simple
meconium ileus, the remainder have complications such as proximal
volvulus, perforation or atresia. These latter problems may be
associated with the development of a meconium cyst. In this instance,
speckled calcifications on plain radiograph or ultrasound are
diagnostic. The film above is that of a meconium pseudocyst
consistent with intraperitoneal spillage of meconium from intestinal
perforation. This finding requires surgical exploration.
Sixty to 70% of infants with simple meconium ileus can be treated by
enema installation of one several irrigation solutions into the
obstructed terminal ileum. Saline, hyperosmolar contrast agents,
dilute N-acetylcysteine and a variety of other solutions have been
used successfully. Following resolution of the obstruction, most
institutions now report survival rates as high as 70% to 100%.
The average life expectancy for CF patients is now well into the third
decade of life. It is primarily determined by the course of the
pulmonary disease rather than GI problems. A number of important
medical advances, including the realistic prospect of gene therapy are
foreseeable for these infants.

42. You are asked to recommend therapy for an asymptomatic 2 year

old who swallowed a small alkaline watch battery 4 hours ago. A plain
film shows the intact battery in the intestine beyond the stomach. The
best course of therapy is?

a. Immediate laparotomy, enterotomy and removal of the battery

b. Enteroscopy with extraction
c. Laparoscopy with ultrasound localization and extraction
d. Cathartics and a follow-up plain film in 48 hours if the child remains
asymptomatic
Answer: d

Passage of an ingested foreign body beyond the gastroesophageal

junction is associated with a 95% probability of uneventful distal
transit. The character of the foreign body is largely irrelevant but
batteries, particularly alkaline disk batteries, present a potentially
serious hazard to children and may require an aggressive approach. A
number of reports have documented the unique risk of intestinal
perforation resulting from disruption of the battery casing and
associated spillage of the toxic contents. Although some have
advocated routine immediate removal, this does not appear
necessary. If the battery is endoscopically accessible in the esophagus,
it should be removed when recognized. Cathartics and enemas may
help expedite passage of batteries discovered when already in the
small or large bowel. Delay for more than a few days, casing rupture
on plain radiograph, or symptoms of any kind require extraction.
Despite the risks, most batteries pass without these sequelae.

43. A jaundiced 6 week old infant has biliary atresia. Which of the
following statements are true?

a. Portoenterostomy is the initial procedure of choice

b. Primary liver transplantation using either a reduced sized cadaveric
graft or a living related graft is now the procedure of choice
c. Approximately two-thirds of patients managed with
portoenterostomy will develop chronic liver disease sufficient to
indicate liver transplantation
d. Because biliary atresia has pathogenic components of acute and
chronic inflammation, antiinflammatory therapy is known to delay
onset of liver failure
Answer: a, c

Biliary atresia is an idiopathic process in which the extrahepatic biliary

ducts are replaced in whole or in part with dense fibrous tissue
containing evidence of both acute and chronic inflammation. There is
an intrahepatic component as well. Although antiinflammatory
therapy is of some theoretical interest, there are no data to suggest
that antiinflammatory pharmocologic therapy will influence the
natural history of liver disease associated with biliary atresia.
The approach for the usual infant in whom biliary atresia is discovered
within the first 90 days of life is to confirm the suspected diagnosis by
operative cholangiogram at laparotomy and then proceed with
portoenterostomy. In general terms, one-third of these infants do well
on a long-term basis, one-third have prompt failure, and the
remainder have chronic liver disease that becomes problematic more
slowly. Therefore, approximately two-thirds of these patients develop
chronic liver disease for which liver transplantation is a reasonable
alternative. Hepatic transplantation is best considered a necessary
and complementary approach to portoenterostomy for infants with
biliary atresia. Data support its use in infants with failed
portoenterostomy or in older infants with established cirrhosis at the
time of presentation. Growth failure, hepatic synthetic failure and
sequelae of portal hypertension are indications to proceed with
transplantation.

44. Of the following, which is the most likely cause of

hemodynamically significant lower gastrointestinal bleeding in a 6
month old male child?

a. Meckel diverticulum
b. Henoch-Schonlein purpura
c. Intussusception
d. Crohn’s colitis
e. Hemolytic uremic syndrome
Answer: a, c

All of the choices are possible causes of lower GI bleeding in a 6

month old; intussusception and a bleeding Meckel diverticulum are
the most common. Upper GI hemorrhage with passage of blood distal
should also be considered, but is not discussed further here as
sampling nasogastric aspirate is a relatively easy and reliable means
of differentiating these problems. The magnitude of blood loss
associated with intussusception is usually minor, but the associated
vomiting and bowel obstruction may lead to significant volume
depletion with hemoconcentration. The magnitude of the hemorrhage
is usually more significant with a Meckel diverticulum.
Infectious diarrheas also occur in this age group. Typically, signs and
symptoms include fever and ileus with bloody diarrhea. The diagnosis
is confirmed with stool examination for leukocytes and cultures for
specific pathogens.

45. Which of the followings statement regarding Hirschprung’s disease

are true?

a. Suction rectal biopsy is virtually always diagnostic if the specimen

includes submucosa
b. Hirschprung’s disease is the result of a sex linked dominant gene
c. The endorectal pullthrough is demonstrably superior to other forms
of surgical construction
d. Ninety percent or more of patients have an excellent or good
functional result following reconstructive surgery
e. The important cause of mortality in contemporary practice is
enterocolitis
Answer: a, d, e

The incidence of Hirschprung’s disease is about 1 per 5000 live births,

with no racial predilection, but with a marked male-to-female (4:1)
preponderance. Most cases are sporadic, but long-segment or total
colonic aganglionosis and female gender are strongly associated with
familial disease. Recent data suggest an association with the RET
protooncogene. The genetic basis of Hirschprung’s disease is under
active investigation and it appears that several genes including those
located on chromosomes 10, 13, 22 and possibly others are involved.
It is neither sex linked nor dominant. There is a rare association with
the MEN syndromes, particularly medullary carcinoma of the thyroid.
The accuracy of suction rectal biopsy is 100% with a correctly done
biopsy that includes submucosa and experienced pediatric pathology
in several large series. This requires both a search for ganglion cells
and evaluation of the axons of the myenteric neurons using either
conventional staining techniques or histochemical staining for
acetylcholinesterase.
Definitive operations for congenital aganglionosis all depend on
resection or bypass of the distal aganglionic rectum with a low rectal
anastomosis to normally innervated pulled-through proximal
intestine. Selection among the several described operations depends
more on a surgeon’s individual training and preference rather than
upon demonstrable differences in outcome. Although the endorectal
pull-through is one of the widely practiced and popular procedures, it
is not demonstrably superior to the procedures described by
Duhamel or Swenson (see text). Eighty to 90% or more of patients
have excellent or normal bowel function following reconstructive
surgery for Hirschprung’s disease when evaluated after 5 years,
regardless of the procedure employed.
The primary remaining cause of mortality directly attributable to
Hirschprung’s disease itself is enterocolitis. When it occurs, this is
typically found in infants or neonates for whom the diagnosis has
been delayed. Postoperative enterocolitis does occur, but it tends to
be substantially less virulent. Undiagnosed neonatal Hirschprung’s
enterocolitis can lead to death in 12 to 24 hours from overwhelming
sepsis.

46. The operative procedure of choice for managing the most

common type of choledochal cyst is which of the following?

a. Cyst gastrostomy
b. Cyst jejunostomy
c. Excision with Roux-en-Y hepaticojejunostomy
d. Transduodenal marsupialization
e. Endoscopic sphincterotomy
Answer: c

The most common choledochal cyst is a type I cyst; 80% to 90% of the
total in most reports. These are characterized by fusiform dilation of
the choledochus itself. These cysts typically involve the entire
common bile duct with only mild dilation of the common hepatic duct
and a normal intrahepatic system. The treatment of this lesion is
always surgical. Internal drainage procedures without cyst resection
(e.g., cyst duodenostomy, cyst gastrostomy and cyst jejunostomy)
were routinely performed for type I choledochal cysts until the 1970s.
The rate of failure (e.g., stricture, recurrent cholangitis, stone
formation, pancreatitis) ranged from 30% to 75%, depending on the
length of follow-up and the type of procedure. As these late
complication rates became apparent, the risk of bile duct
adenocarcinoma in the residual cyst also became widely recognized.
Therefore, the preferred operative treatment of a type I choledochal
cyst is total transmural excision with Roux-en-Y hepaticojejunostomy.
Occasionally, adults with severe inflammation and fibrosis may
require intramural cyst dissection, leaving the posteriomedial (outer)
wall of the cyst in situ to protect the adjacent portal vein and hepatic
artery.

47. Which of the following is the most common liver tumor of

childhood?

a. Hemangioma and hemangioendothelioma

b. Hepatoblastoma
c. Hepatocellular carcinoma
d. Mesenchymal hamaratoma
Answer: b

Primary liver tumors are uncommon in children. Of these, about

one-third are benign and two-thirds are malignant. The most common
presenting feature for a liver tumor is an asymptomatic abdominal
mass. The diagnostic evaluation is generally an ultrasound initially
followed by either computed tomography or magnetic resonance
imaging, for definitive diagnosis. The relative incidence of liver tumors
is illustrated in the table below.
Hepatoblastoma is the most common liver tumor of childhood. Most
hepatoblastomas are discovered within the first two years of life.
Although these are large and may require primary chemotherapy, a
65%–75% survival is achievable with resection that yields histologically
clear resection margins.

48. The risk of biliary tract adenocarcinoma developing in a patient

with a choledochal cyst left in situ is approximately which of the
following?

a. Less than 1%
b. 3% to 5%
c. 10% to 15%
d. Greater than 25%
Answer: b

Adenocarcinoma of the biliary tract develops in 3% to 5% of patients

who have choledochal cysts. Although this represent a small number
of patients, the total number reported exceeds 50 and the incidence is
about 1000 times that of the normal population. In addition, the
carcinoma may develop as early as the adolescent years, a marked
contrast to the normal population in which a fifth or sixth decade of
life presentation is typical. Neoplastic transformation of the dysplastic
biliary cyst epithelium may result from chronic inflammation.
Consideration of this potential problem has contributed significantly
to the current emphasis on surgical excision of these cysts.

49. The most common cause of acute pancreatitis in childhood is

which of the following?

a. Pancreas divisum
b. Cholelithiasis
c. Trauma
d. Valproic acid
e. Annular pancreas
Answer: c

Although adult pancreatitis is usually related to cholelithiasis or

alcohol ingestion, pediatric causes are considerably more varied in
etiology. Fifty to 70% of childhood acute pancreatitis is either
idiopathic or posttraumatic in origin. Trauma is the single most
common cause of acute pancreatitis in childhood. Cholelithiasis is an
important etiology in the adolescent population and in children with
hemotologic disorders. Annular pancreas normally is associated with
duodenal atresia and produces neonatal duodenal obstruction, but
not acute pancreatitis. Pancreas divisum is an anatomic variation
present in 10% to 15% of normal children that is occasionally the
cause of acute pancreatitis. Valproic acid is an important
anti-convulsant used in pediatric neurology. One of its possible
complications is the development of acute pancreatitis or necrotizing
pancreatitis. Fortunately, this is rare as it is often lethal.

50. Which of the following statements regarding gastroschisis are

true?

a. Primary fascial closure can be achieved in only about 25% of these

infants
b. These infants have an incidence of approximately 40% to 50% of
associated anomalies
c. Overall survival is approximately 80% to 90%
d. When the diagnosis is known prenatally, planned cesarean section
is the safest method of delivery
Answer: c

Gastroschisis is a full-thickness defect of the abdominal wall with

herniation of a variable amount of uncovered intestine. Prenatal
diagnosis has enabled the diagnosis of gastroschisis to be made prior
to delivery. Prospective evaluation comparing vaginal delivery with
elective cesarean section has demonstrated no difference in outcome.
Therefore, careful vaginal delivery generally remains the birthing
method of choice. Unlike omphalocele which is commonly associated
(50%) with other anomalies, other structural anomalies are associated
with gastroschisis in approximately 10% of patients.
Primary fascial closure after reduction of the herniated viscera is the
best surgical option and this is possible in 60% to 70% of infants. Care
must be taken not to generate excessive intraabdominal pressure
when performing a primary abdominal wall closure. Generally, an
intraabdominal pressure less than 20 cm H2O is well tolerated. If the
herniated viscera cannot be reduced primarily, a silastic pouch
constructed to temporarily contain the extra abdominal bowel and a
series of partial reductions are begun. This approach combined with
adequate nutritional support by total parenteral nutrition yields
survival rates of at least 80% to 90% in most contemporary series of
gastroschisis.

51. Which of the following are typical causes of neonatal intestinal

obstruction?

a. Intussusception
b. Meconium ileus
c. Hirschprung’s disease
d. Meckel’s diverticulum
e. Incarcerated hernia
Answer: b, c, e

Neonatal bowel obstruction is defined as intestinal obstruction

developing in the first 30 days of life. The cardinal manifestation is
bilious vomiting often in conjunction with abdominal distention.
Meconium ileus and Hirschprung’s disease are classical causes of
neonatal intestinal obstruction outlined in the table. Incarcerated
inguinal hernias are the most common cause of neonatal intestinal
obstruction. Hernias usually do not present a diagnostic dilemma, as
simple inspection of the groin yields an obvious diagnosis.
Intussusception, while a cause of distal bowel obstruction in infants,
does not usually become a consideration until at least 3 to 6 months
of age. Intestinal obstruction related to Meckel’s diverticulum is
generally related to either intussusception or volvulus associated with
a band from the Meckel’s diverticulum to the abdominal wall. Both of
these events tend to occur later than the neonatal period.

52. A 6-week-old child presents with generalized seizures, a serum

glucose of 30 mg/dL and concurrent hyperinsulinemia. This child’s
first priority is which of the following?

a. Permanent central venous access and glucose infusion

b. Administration of cortisone and adrenocorticotropic hormone
c. Computerized tomographic scan of the abdomen to look for an islet
cell adenoma
d. Urgent pancreatic resection
Answer: a

Most cases of hyperinsulinemia in the first 2 years of life are due to

nesidioblastosis, a condition associated with excessive and diffuse
formation of neoislets from primitive pancreatic ductal cells.
Hyperinsulinemia secondary to islet cell adenoma or carcinoma or
islet cell hyperplasia is more common in the older child. Infants with
nesidioblastosis, such as the one described here, typically present
with symptomatic hypoglycemia, seizures and hyperinsulinemia. An
insulin-to-glucose ratio (insulin in IU/ml divided by glucose in mg/dL)
that is greater than 0.5 with fasting is highly suggestive.
Infants with nesidioblastosis are managed initially medically with
maintenance of blood glucose levels above 40 mg/dL. This is best
carried out by the infusion of hypertonic glucose solutions through a
permanent central venous catheter. In addition, diazoxide, cortisone
and adrenocorticotropic hormone and streptozocin have been used
to treat the hypoglycemia.
Definitive management of nesidioblastosis may require pancreatic
resection. This requires a pancreatectomy usually estimated at
approximately 95% to 99% with splenic and duodenal preservation.
Following 90% to 95% pancreatectomy, over 90% of infants with
nesidioblastosis are rendered permanently euglycemic. A CT scan of
the abdomen to search for an adenoma is an appropriate diagnostic
maneuver, but it is not the first priority.

53. At what age is surgical orchiopexy recommended for a child with a

unilateral undescended testis?

a. Promptly upon discovery, regardless of age

b. 1 year
c. 5 to 6 years
d. Any time prior to puberty
Answer: b

Undescended testis occurs in 30% of premature boys, 3.4% of

full-term boys, 0.8% of 1-year olds, and 0.8% of adults. The implication
is that if spontaneous testicular descent has not occurred by 1 year of
age, it is unlikely to occur subsequently. Therefore, orchiopexy is
deferred until the patient reaches 1 year of age. Testes that remain
undescended during childhood have a significant reduction in the
number of germ cells and have little function after puberty.
Demonstrable histologic changes are present by the age of 2 years.
For this reason, delay beyond 1 year is not recommended.

54. An infant is noted to have a left flank mass shortly after birth and
an ultrasound examination demonstrates left hydronephrosis. The
most common cause of this finding is which of the following?

a. Neonatal Wilm’s tumor

b. Congenital ureteropelvic junction obstruction
c. Multicystic dysplastic kidney
d. Vesicoureteral reflux
Answer: b

The most common cause of neonatal hydronephrosis is congenital

ureteropelvic junction obstruction. It is important to rule out bilateral
ureteropelvic junction obstruction when this is found as this can
present in 20% to 30% of neonatal cases. The management requires
establishment of dependent drainage of the renal pelvis, and this is
usually accomplished by a dismembered pyeloplasty with resection of
the obstructing segment. A successful outcome is expected in over
90% of patients.
Neonatal Wilm’s tumor is exceedingly rare and does not ordinarily
present with obstruction of a functioning kidney, but rather as a solid
mass. Multicystic dysplastic kidney occurs in about 1 in 4000 births
and is usually unilateral. It is the most common form of renal cystic
disease in infants, but these cysts do not communicate with a
functional renal pelvis and there is little or no functional renal cortex.
Vesicoureteral reflux, while common, does not present in the
newborn period with hydronephrosis and a palpable kidney.

55. The medical indications for circumcision include which of the

following?

a. Infants with a history of urinary tract infection

b. Hypospadias
c. Phimosis
d. Enuresis
e. Vesicoureteral reflux
Answer: a, c, e

The American Academy of Pediatrics has published guidelines on the

indications for circumcision. Circumcision should be encouraged in
infants with a history of urinary tract infection or vesicoureteral reflux
to decrease the chances of ascending infection. On the contrary, in an
infant with hypospadias, chordee, significant penoscrotal webbing, or
other congenital problems, circumcision should be discouraged to
preserve the foreskin for use in later reconstruction. In normal
infants, circumcision is a matter of family choice and not an important
issue for medical debate. Phimosis is a fibrotic contraction of the
preputial aperture so that retraction is impossible. Circumcision or
dorsal slit are the most effective solutions to phimosis. Enuresis is
unrelated to the preputial skin and has no bearing on the decision for
circumcision.

56. Which of the following statements regarding neuroblastoma are

true?

a. Neuroblastoma is the most common abdominal malignancy of

childhood
b. Approximately 80% of neuroblastoma patients are diagnosed prior
to age 4 years
c. N-myc oncogene copy number in neuroblastoma tissue is inversely
related to survival probability
d. Trk proto-oncogene expression in neuroblastoma tissue is inversely
related to survival probability
e. All of the above
Answer: a, b, c

Neuroblastoma is the most common extracranial solid tumor and the

most common abdominal malignancy of childhood. The incidence is
approximately 8 to 10 per million children under the age of 15 years.
The incidence is uniform throughout the world. This results in
approximately 500 new cases reported each year in the United States.
The median age at diagnosis is about 2 years and 80% of children are
less than four years of age at diagnosis. The N-myc oncogene, whose
function and mechanism of action remain the subject of investigation,
was empirically shown to be a useful predictor of survival and risk. It
was found that patients with increased copies of the N-myc gene had
a much worsened prognosis. Currently, most authorities consider a
copy number of more than ten to be significant. The trk proto-
oncogene is a component of the high-affinity nerve growth factor
receptor and is expressed in human neuroblastoma tissue. Trk-A
expression is inversely correlated with N-myc amplification and is
associated with lower stage at diagnosis and improved prognosis.

57. Which of the following are considered low risk features for
neuroblastoma patients?

a. Age less than one year

b. Stage 2A and 2B disease (International Staging Criteria)
c. Stage 4S disease (International Staging Criteria)
d. Neuron specific enolase plasma level less than 100 ng/ml
e. None of the above
Answer: a, b, c, d

Risk status is determined in neuroblastoma patients by a number of

characteristics which have been elaborated over the last twenty years.

58. A one month old female infant is brought to you for evaluation of
afriable polypoid mass prolapsing through the vaginal introitus. Your
presumptive diagnosis is which of the following?

a. Ectopic ureterocele
b. Rectal prolapse
c. Congenital adrenal hyperplasia with ambiguous genitalia
d. Embryonal rhabdomyosarcoma
Answer: d

Vaginal and cervical primary rhabdomyosarcomas often prolapse

through the vaginal orifice as a friable polypoid mass and may
hemorrhage. Botyroid tumors are really of the embryonal subtype but
grow into a hollow space such as the vagina or bladder so they
undertake a characteristic “grape-like” appearance. Other patterns of
rhabdomyosarcoma include alveolar and pleomorphic morphology.
The incidence of rhabdomysarcoma is biphasic with one peak in
infancy followed by the second in the adolescent years. The nature of
presentation is site dependent. This patient has a classical
presentation for a vaginal botyroid rhabdomyosarcoma. The
emphasis in evaluation is to perform a thorough pretreatment
workup that defines completely local tumor extent and evaluates the
regional and distant sites of metastases.

59. Which of the following approaches is considered standard care for

most Wilms’ tumor patients in the United States today?

a. Adriamycin and vincristine therapy followed by surgical resection

b. Needle biopsy followed by either chemotherapy or resection
depending upon the histology
c. Primary surgical resection followed by chemotherapy
d. Radiation therapy if judged unresectable on CT or MRI imaging
Answer: c

The standard of care for Wilms’ tumor patients in the United States is
initial surgical resection. Exceptions to this rule include extensive
intracaval tumors which require cardiopulmonary bypass for
extraction, obviously unresectable tumors with documented invasion
of contiguous structures, and possibly bilateral tumors, especially if it
is unclear which side is most heavily involved. All resectable Wilms’
tumor patients receive postoperative chemotherapy with the possible
exception of Stage 1 favorable histology patients who are younger
than 24 months of age at diagnosis and have tumors less than 250
grams in weight at resection. Chemotherapy followed by surgical
resection is practiced in Europe with roughly equal outcomes to those
in the United States but this approach has the disadvantage of
changing the surgical and pathologic staging which are the basis for
the National Wilms’ Tumor Studies and the cornerstone of treatment
in the United States. Needle biopsy has a very limited role for unusual
presentations of Wilms’ tumor as the diagnosis is generally apparent
with modern imaging techniques. Radiation therapy is not a primary
mode of therapy for Wilms’ tumor under contemporary National
Wilms’ Tumor treatment protocols.

60. Which of the following statements regarding rhabdomyosarcoma

are true?

a. Surgical resection of the primary tumor results in cure of

approximately 80 to 90% of all patients
b. Currently recommended therapy includes complete resection of
primary tumors prior to chemotherapy for small noninvasive lesions,
or after documented response with more formidable primary tumors
c. Alveolar histology is a favorable prognostic finding
d. Overall survival of all patients is now approximately 50%
Answer: b, d

Surgical resection of the primary tumor was the mainstay of

treatment 30 years ago for rhabdomyosarcoma but only resulted in
overall survival rates in the range of 20%. This improved to
approximately 50% with the addition of chemotherapy. Survival is
stage dependent and if all cases (both high and low risk) are included,
the overall survival from rhabdomyosarcoma is now approximately
50%. Presently it is recommended that complete resection of primary
tumors should be undertaken either before chemotherapy for small
noninvasive lesions or after documented response with more
formidable primary tumors. In certain situations where chemotherapy
results in a complete or very good tumor regression, external beam
radiation may be employed as a primary means of local control.
Debilitating or disfiguring surgery is only performed if residual tumor
is present after both chemotherapy and therapeutic irradiation.
Alveolar histology is associated with a particularly poor prognosis for
rhabdomyosarcoma.

61. Patients with Wilms’ tumors most frequently present with which of
the following?

a. Bilateral metachronous lesions

b. Bilateral synchronous lesions
c. An extrarenal primary
d. A multicentric primary lesion
e. A unifocal, unilateral lesion
Answer: e
It is currently hypothesized that Wilms’ tumor arises from primitive
metanephric blastema and individual tumors often contain not only
primitive metanephric cells but also cartilage, skeletal muscle and
squamous epithelium. Most tumors arise unifocally within the kidney
but approximately seven percent of unilateral Wilms’ tumors are
multicentric. The proportion of synchronous bilateral tumors among
all nephroblastoma patients ranges from 4.4 to 7.0% while that of
metachronous tumors is 1.0–1.9%. Wilms’ tumors are equally
distributed with regard to the left and right side and may occur with
no apparent connection to the kidneys. Usually, extrarenal Wilms’
tumor occurs in the retroperitoneal area but other reported sites
include pelvis, scrotum and inguinal region.

62. Hepatoblastomas are childhood liver tumors characterized by

which of the following features?

a. Multicentricity
b. Cirrhosis in the uninvolved liver
c. Unresectable tumors subjected to cytoreductive chemotherapy may
be resected with long-term survival
d. Jaundice
Answer: c

Children with hepatoblastoma most commonly present with an

abdominal mass or diffuse abdominal swelling. The child is typically in
good health and the lesion may be observed by an observant parent
or clinician on routine examination. Weight loss and other symptoms
are unusual. Liver function tests are usually normal or nonspecifically
altered. Jaundice is uncommon. The most useful tumor marker is the
serum (a-fetoprotein (AFP)) level which is elevated in approximately
90% of the cases.
Hepatoblastoma usually presents as a single, pseudo-encapsulated
lesion often reaching large proportions before becoming apparent.
The umbilical fissure is generally not breached. Multicentricity occurs
in less than 20% and cirrhosis of the surrounding liver is unusual.
Multicentricity and associated cirrhosis are typical of hepatocellular
carcinoma. Complete surgical resection remains the major objective
of therapy for hepatoblastoma. At presentation approximately 60% of
patients with hepatoblastoma have resectable tumors. Chemotherapy
is the major treatment option available for unresectable tumors. Over
the last decade it has become apparent that some of these patients
may be rendered resectable by preoperative chemotherapy.

63. Common sites of neuroblastoma metastasis are which of the

following?
a. Lung
b. Regional lymph nodes
c. Bone marrow
d. Cortical bone
Liver
Answer: b, c, d

Neuroblastoma metastasizes to both regional lymph nodes and

distant sites, most frequently bone marrow and/or cortical bone. The
liver and lungs are rarely the site of metastatic spread. Cortical bone
involvement as manifested by a positive bone scan is a particularly
poor prognostic indicator. The majority of patients present with locally
advanced disease at the time of diagnosis.

64. Which of the following statements regarding renal tumors of

childhood and adolescence are true?

a. Clear cell sarcoma is presently considered a variant of Wilms’ tumor

with a poor prognosis
b. Clear cell sarcoma of the kidney has a high rate of metastasis to
bone
c. Rhabdoid tumors may arise in the kidney, mediastinum or brain
d. Childhood rhabdoid tumors of the kidney carry an excellent
prognosis
Answer: b, c

Clear cell sarcoma of the kidney is presently considered a distinct

histopathologic and clinical entity from Wilms’ tumor. It has a similar
age distribution as that observed in Wilms’ tumor, but a markedly
worsened prognosis. It is characterized by a proclivity to metastasize
to bones and indeed has been called the bone metastasizing renal
tumor of childhood. Relapse and death occur in 75% of patients with
over half dying within one year of diagnosis. Aggressive systemic
chemotherapy is recommended for all stages of the disease. Likewise,
postoperative radiation to the tumor bed is recommended regardless
of stage.
Rhabdoid tumors are rare malignancies that most commonly involve
the kidney in childhood but may also occur primarily in the
mediastinum or brain. Outcome is particularly poor and there is no
proven chemotherapy regimen. Rhabdoid tumors of the kidney occur
in infancy with a median age at presentation of 13 months. Survival
rates are almost zero and even Stage I patients fare poorly. Aggressive
therapy is warranted including surgical resection, local radiation
therapy and systemic chemotherapy.
65. Which of the following syndromes are associated with the
development of Wilms’ tumor?

a. Beckwith-Wiedemann Syndrome (hemi-hypertrophy, macroglossia,

aniridia)
b. Neurofibromatosis
c. Denys-Drash syndrome (pseudohermaphroditism, glomerulopathy)
d. Gonadal dysgenesis
e. Hemolytic uremic syndrome
Answer: a, b, c, d

A number of syndromes are associated with the development of

Wilms’ tumors and when present, require routine periodic screening.
There is no known association of the hemolytic uremic syndrome with
the development of Wilms’ tumor.

MedCosmos at 7:01 PM

No comments:

Post a Comment

‹ Home ›
View web version

Powered by Blogger