CHAPTER 71
Pediatric Surgery
Brad W. Warner, MD
Newborn Physiology
Fluids, Electrolytes, and Nutrition
Extracorporeal Life Support
Trauma
Lesions of the Neck
Alimentary Tract
Abdominal Wall
Genitourinary
Congenital Diaphragmatic Hernia
Congenital Chest Wall Deformities
Bronchopulmonary Malformations
Hepatobiliary
Childhood Solid Tumors
Fetal Surgery
Pediatric surgery is a subspecialty that is both exciting
and rewarding for multiple reasons. First, the range of
problems encountered may be quite dramatic and is not
limited to specific anatomic boundaries. Further, the
pathogenesis of many significant pediatric surgical
conditions remains unknown. As such, the challenge for
intense, active investigation is ever-present. Finally, the
approach to the child, interactions with concerned
parents, and lifelong consequences of operative interven-
tions demand a unique sensitivity and attention to detail,
the impact of which is often profound.
This chapter seeks to emphasize the most important
components of the more common pediatric surgical con-
ditions. A discussion of several common pediatric condi-
tions (e.g., appendicitis, inflammatory bowel disease) is
intentionally omitted to avoid redundancy with other
chapters.
Ch071-X3675.indd 2047
NEWBORN PHYSIOLOGY
General
The newborn infant is both physically and physiologi-
cally distinct from the adult patient in several respects.
The smaller size, immature organ systems, and differing
volume capacities present unique challenges toward
perioperative management. In utero, the cardiovascular
system essentially pumps blood from the placenta and
bypasses the lungs through the patent foramen ovale and
the ductus arteriosus. With clamping of the umbilical cord
at the time of delivery, the foramen ovale closes, and
there is an abrupt fall in pulmonary arterial pressure. The
ductus arteriosus begins to close soon thereafter. These
factors serve to promote pulmonary blood flow. Persis-
tent pulmonary hypertension, which is associated with
hypoxemia, acidosis, or sepsis, may contribute to ductal
patency, and right-to-left shunting may occur. In addition,
prematurity is a risk factor for failure of the ductus arte-
riosus to close. As such, attempts to close the ductus
pharmacologically using indomethacin or by direct surgi-
cal ligation may be necessary. Before the ductus is closed,
there may be a higher partial pressure and saturation of
oxygen in the blood when sampled from the right arm
(preductal), as compared with the other extremities
(postductal), owing to the flow of unoxygenated blood
from the pulmonary artery through the ductus into the
aorta.
Cardiovascular
Cardiac perfusion is best monitored clinically by capillary
refill, which ideally is less than 1 second. A capillary refill
longer than 1 to 2 seconds is associated with significant
shunting of blood from the skin to the central organs as
may occur with cardiogenic shock or significantly reduced
2047
8/28/2007 11:37:44 AM
 2048 Section XIII Specialties in General Surgery
intravascular volume from dehydration or bleeding. In
neonates, the size of the liver is a reasonable gauge of
intravascular volume. Finally, cardiac output in the
newborn period is rate dependent, and the heart has a
limited capacity to increase stroke volume to compensate
for bradycardia.
Pulmonary
The lungs are not completely developed at birth and
continue to form new terminal bronchioles and alveoli
until about 8 years of age. In premature infants, lung
immaturity is one of the greatest contributors to morbidity
and mortality. In addition to reduced alveoli, immature
lungs have reduced production of surfactant, which is
critical for maintaining surface tension within the alveoli
and gas exchange. A major contribution in the manage-
ment of premature infants has therefore been the ability
to provide exogenous surfactant. This has resulted in
improved survival and less bronchopulmonary dysplasia
(defined as the need for supplemental oxygen beyond
the first 28 days of life). In addition to pulmonary paren-
chymal issues, the airway of the newborn is quite small
(tracheal diameter, 2.5-4 mm) and easily plugged with
secretions. The respiratory rate for a normal newborn
may range from 40 to 60 breaths/min. Respiratory distress
is heralded by nasal flaring, grunting, intercostal and
substernal retractions, and cyanosis. Finally, infants pref-
erentially breathe through their nose and not their
mouth.
Cold Stress
Newborn infants must be maintained in a neutral thermal
environment because they are at great risk for cold stress.
The major risk factors for the development of hypother-
mia in infants include their relatively large body surface
area, lack of hair and subcutaneous tissue, and increased
insensible losses. Neonates who are cold stressed respond
by nonshivering thermogenesis. Metabolic rate and
oxygen consumption are augmented by brown fat mobi-
lization. Continued cold exposure leads to decreased
perfusion and acidosis. Radiant heat warmers are usually
necessary in very small premature infants, and this may
contribute to further insensible water losses.
Infection
The neonate is relatively immunodeficient, with reduced
levels of immunoglobulins and the C3b component of
complement. As such, premature infants are at signifi-
cantly increased risk for severe infection. Sepsis may
result from multiple interventions that are necessary to
care for these premature infants, including prolonged
endotracheal intubation and central vein or bladder cath-
eterization. Empiric antibiotic therapy to prevent over-
whelming sepsis may be lifesaving and may be based on
simple clinical judgment of subtle alterations in factors
such as reduced tolerance of enteral feeding, tem-
perature instability, reduced capillary refill, tachypnea, or
irritability.
Ch071-X3675.indd 2048
FLUIDS, ELECTROLYTES, AND NUTRITION
Several basic principles must be understood before
assuming the responsibility for the metabolic needs for
an infant or child. First, the margin for error is narrower
when compared with adults. The consequences of too
much or too little intravenous (IV) glucose in a neonate
may be devastating and even life threatening. As such,
the fluid, electrolyte, and parenteral nutrition orders are
considered as important as writing for any medication
that has the potential for serious side effects. Second, on
a body weight basis, protein and energy requirements
are much greater in younger children, and decrease with
age. Not only are calories utilized for higher baseline
metabolic rates, but also a significant proportion of
energy in the diet must be allocated for growth. Third,
in contrast with adults, daily weight gain in neonates is
an important indicator of providing sufficient calories.
Infants who are losing weight, or even failing to gain
weight, mandate a careful reassessment of metabolic
needs and amount of nutrition provided.
Fluid Requirements
Because of increased insensible water losses through
thinner, less mature skin, the fluid requirements for pre-
mature infants are substantial. Insensible water losses are
directly related to gestational age and range from 45 to
60 mL/kg/day for premature infants weighing less than
1500 g to 30 to 35 mL/kg/day for term infants. In contrast,
insensible water losses in an adult are roughly 15 mL/kg/
day. Other factors such as radiant heat warmers, photo-
therapy for hyperbilirubinemia, and respiratory distress
further increase losses.
At 12 weeks’ gestation, 94% of the fetal body weight
is composed of water. This amount declines to roughly
78% by term (40 weeks’ gestation) and reaches adult
levels (60%) by 11/2 years of age. In the first 3 to 5 days
of life, there is a physiologic water loss of up to 10% of
the body weight of the infant. This is the singular excep-
tion to the general principle that infants are expected to
gain weight each day. As such, fluid replacement volumes
are less over the first several days of life. Recommended
fluid volume replacements are shown in Table 71-1.
These fluid volumes are regarded as estimates and may
change given differing environmental or patient factors.
Table 71-1 Daily Fluid Requirements for Neonates
and Infants
WEIGHT VOLUME
Premature <2.0 kg 140-150 mL/kg/day
Neonates and infants 100 mL/kg/day for first 10 kg
2-10 kg
Children 10-20 kg 1000 mL + 50 mL/kg/day for
weight 10-20 kg
Children >20 kg 1500 mL + 20 mL/kg/day for
weight >20 kg
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 Chapter 71 Pediatric Surgery 2049

40 40

35 35

ENERGY FOR GROWTH (% of total)

30 30

25 25

Weight gain (g/day) 20 20

15 15

10 10

5 5

0 0
0 2 4 6 8 10 12 14 16 18 20 22 24
Age (months)

Figure 71-1 Daily gain in body weight and percentage of energy intake utilized for growth at 2 weeks to 2 years
of age. Solid line, weight gain (g/day); dashed line, % of energy utilized for growth. (From Anderson TA: Birth
through 24 months. In Rudolph AM [ed]: Pediatrics, 18th ed. Norwalk, CT, Appleton & Lange, 1987, p 158.)

The best two indicators of sufficient fluid intake are Table 71-2 Average Energy Requirement by Age
urine output and osmolarity. The minimum urine output AGE (mo) AVERAGE ENERGY REQUIREMENT* (kcal/kg/d)
in a newborn and young child is 1 to 2 mL/kg/day.
Although adults can concentrate urine in the 1200 mOsm/ 0-1 124
kg range, an infant responding to water deprivation is 1-2 116
only able to concentrate urine to a maximum of
2-3 109
700 mOsm/kg. Clinically, this means that greater fluid
intake and urine output are necessary to excrete the 3-4 103
solute load presented to the kidney during normal 4-5 99
metabolism. 5-6 96.5
Electrolyte Requirements 6-7 95
7-8 94.5
In general, the daily requirements for sodium are 2 to
4 mEq/kg and for potassium are 1 to 2 mEq/kg. These 8-9 95
requirements are usually met with a solution of 5% 9-10 99
dextrose in 0.2% normal saline with 20 mEq KCl added
10-11 100
per liter at the calculated maintenance rate, as noted
previously. 11-12 104.5

*Based on measured intakes.

Caloric Requirements From Beaton GH: Nutritional needs during the first year of life: Some
concepts and perspectives. Pediatr Clin North Am 32:275, 1985.
Energy requirements from birth through childhood are
partitioned into maintenance of existing body tissues,
growth, and physical activity. As depicted in Figure 71-1,
the amount of energy from the diet required for growth
alone may be as high as 40% in neonates. The parameter
in infants. This protein requirement is reduced in half by
that is most indicative of sufficient provision of calories
age 12 years and approaches adult requirement levels
in neonates is daily weight gain. As such, infants gain
(1 g/kg/day) by 18 years of age. The provision of greater
roughly 30 g/day. The expected daily weight gain
amounts of protein, relative to nonprotein, calories will
decreases with age. Total daily caloric requirements
result in rising blood urea nitrogen levels. The nonprotein
also decrease with age, and guidelines are listed in
calorie (carbohydrate plus fat calories) to protein calorie
Table 71-2.
ratio (when expressed in grams of nitrogen) therefore is
not less than 150 : 1. For infants receiving parenteral nutri-
Protein tion, the amount of protein provided is usually begun
The average intake of protein makes up about 15% of at 0.5 g/kg/day and advanced in daily increments of
the total daily calories and ranges from 2 to 3.5 g/kg/day 0.5 g/kg/day to the target goal.

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 2050 Section XIII Specialties in General Surgery
Carbohydrate
When oral nutrition cannot be provided to infants, it is
critical that IV fluids are provided to supply water, elec-
trolytes, and glucose. Failure to provide glucose for pro-
longed periods will result in the rapid (within hours)
development of hypoglycemia. In turn, this may lead to
seizures, neurologic impairment, or even death. The
absolute minimum IV glucose infusion rate for neonates
is 4 to 6 mg/kg/min. This rate needs to be calculated
daily for every neonate receiving parenteral nutrition.
During total parenteral nutrition, the amount of glucose
provided is increased daily to a maximum of 10 to 12 mg/
kg/min. These are general guidelines and must be tai-
lored to each individual patient. The amount of weight
gain will dictate the need to continue advancing glucose
calories. Further, hyperglycemia from either too rapid
advancement or underlying sepsis needs to be avoided
because it leads to rapid hyperosmolarity and dehydra-
tion. In contrast with adults, the addition of insulin to the
parenteral nutrition solution in children is very high risk
and is generally not indicated in routine practice.
Fat
In adults, parenteral fat is provided as a daily infusion as
a source of calories or is given intermittently to prevent
the development of essential fatty acid deficiency. In the
pediatric population, fat is always provided as a daily
infusion for both purposes. The lipid requirements for
growth are significant, and fat is a robust caloric source.
Similar to protein, fat infusions are started at 0.5 g/kg/day
and advanced up to 2.5 to 3 g/kg/day. In infants with
unconjugated hyperbilirubinemia, fat administration is
done with caution because fatty acids may displace bili-
rubin from albumin. The free unconjugated bilirubin may
then cross the blood-brain barrier and lead to kernicterus
and resultant mental retardation.
EXTRACORPOREAL LIFE SUPPORT
Extracorporeal life support (ECLS), formerly referred to
as extracorporeal membrane oxygenation (ECMO), is a
type of heart-lung bypass that provides short-term (days
to weeks) support for the critically ill patient with acute
life-threatening respiratory or cardiac failure. ECLS is a
purely supportive, nontherapeutic intervention that main-
tains adequate gas exchange and circulatory support
while resting the injured lungs or heart. Although the use
of ECLS has been described in both pediatric and adult
populations, the greatest experience has been reported
in neonatal respiratory failure.1 Since the mid-1970s when
the first neonatal survival was reported, ECLS has become
the standard of care for neonatal respiratory failure unre-
sponsive to maximum conventional medical manage-
ment. It is practiced in more than 90 centers worldwide,
with an overall survival rate of 80%.
Indications
The major indications for initiation of neonatal ECLS
include meconium aspiration, respiratory distress syn-
Ch071-X3675.indd 2050
drome, persistent pulmonary hypertension, sepsis, and
congenital diaphragmatic hernia. Occasionally, neonates
with congenital cardiac anomalies may be supported with
ECLS until surgical repair can be accomplished. Meco-
nium aspiration syndrome is the most common indication
for neonatal ECLS and is associated with the highest
survival rate (>90%).
Selection Criteria
Selection criteria for initiation of neonatal ECLS vary
slightly from institution to institution and are usually
derived from historical controls. Generally, an infant must
have at least 80% predicted mortality with continued
conventional medical management to justify this high-risk
therapy. Two formulas have been historically used as a
means to predict survival without ECLS. One formula is
the alveolar-arterial oxygen gradient (AaDO2) and is cal-
culated as (atmospheric pressure − 47) − (PaO2 + PaCO2). An
AaDO2 that is greater than 620 for 12 hours or an AaDO2
greater than 620 for 6 hours associated with extensive
barotrauma and severe hypotension requiring inotropic
support are considered to be criteria for ECLS. The other
formula is the oxygen index (OI) and is calculated as the
fraction of inspired oxygen (usually 1.0) multiplied by
the mean airway pressure times 100 ÷ PaO2. If the OI is
more than 40, 80% mortality may be assumed. Additional
inclusion criteria include gestational age greater than 34
weeks, birth weight more than 2 kg, and a reversible
pulmonary process. Exclusion criteria include the pres-
ence of cyanotic congenital heart disease or other major
congenital anomalies that preclude survival, intractable
coagulopathy or hemorrhage, sonographic evidence of a
significant intracranial hemorrhage (>grade I intraven-
tricular hemorrhage), and more than 10 to 14 days of
high-pressure mechanical ventilation. Before initiation of
ECLS, all infants must undergo a cardiac echocardiogram
to rule out congenital heart disease and a cranial ultra-
sound to exclude the presence of significant intracranial
hemorrhage.
Extracorporeal Life Support Circuit
The basic concept of ECLS is to drain venous blood,
remove carbon dioxide, and add oxygen through the
artificial membrane lung, and then return warmed blood
to the arterial (venoarterial) or venous (venovenous) cir-
culation. Venoarterial bypass provides both cardiac and
respiratory support, whereas venovenous bypass pro-
vides only respiratory support. Venoarterial bypass is
used most commonly, and the right internal jugular vein
and common carotid artery are typically chosen for can-
nulation because of their large size, accessibility, and
adequate collateral circulation. The ECLS circuit (Fig.
71-2) is composed of a silicone rubber collapsible
bladder (which collapses if venous return is diminished),
a roller pump, a membrane oxygenator, a heat exchanger,
tubing, and connectors. Venous blood from the right
atrium drains through the venous cannula to the bladder
and is pumped to the membrane oxygenator, where
carbon dioxide is removed and oxygen is added. The
8/28/2007 11:37:46 AM

Aortic
arch
Fluids Heparin
Blood
drainage
Bridge
Servo-
regulation
Pump
Figure 71-2 Diagrammatic representation of venoarterial extracorporeal life support circuit. (From Shanley CJ,
Bartlett RH: Extracorporeal life support: Techniques, indications, and results. In Cameron JL [ed]: Current Surgical
Therapy [4th ed]. St Louis, Mosby–Year Book, 1992, pp 1062-1066.)
oxygenated blood then passes through the heat ex-
changer and is returned to the patient through the arterial
cannula.
To prevent clotting of the ECLS circuit, systemic anti-
coagulation is maintained. The fully heparinized patient
is at risk for serious bleeding complications. As such,
hematocrit levels are followed closely. Similarly, platelet
counts and fibrinogen level must be monitored and
normal levels maintained. Daily cranial ultrasound evalu-
ations are obtained to monitor for hemorrhage.
Weaning From Extracorporeal Life Support
Extracorporeal flow is gradually weaned as native cardiac
or pulmonary function improves. Indicators of lung
recovery include an increasing PaO2, improved lung com-
pliance, and clearing of the chest x-ray. Once the extra-
corporeal flow rates are at minimal levels, the venous
and arterial cannulas may be clamped to give the patient
a trial off bypass. If the patient remains hemodynamically
stable with adequate oxygenation and ventilation while
the cannulas are clamped, the cannulas are surgically
removed, and conventional ventilatory support is contin-
ued. The mean duration of ECLS in neonates is roughly
5 to 6 days. Patients with congenital diaphragmatic hernia
and sepsis tend to have the longest required duration of
bypass.
Ch071-X3675.indd 2051
Chapter 71 Pediatric Surgery 2051
Right
atrium
Blood
return
Heat
exchanger
Membrane lung
Complications
In addition to bleeding, ECLS is associated with signifi-
cant morbidity and mortality. Multiple factors contribute
to the risk of this technology and include ligation and
cannulation of the right common carotid artery and right
internal jugular vein, systemic heparinization, exposure
to multiple blood products, and potential for mechanical
failure of the circuit. Bleeding is the most common com-
plication and may be either medical (e.g., too few plate-
lets, too much heparin, intracranial) or surgical (e.g., neck
cannulation site, intrathoracic, gastrointestinal). Birth
weight and gestational age are the most significant cor-
relates of intracranial hemorrhage on ECLS, and infants
weighing less than 2.2 kg and younger than 35 weeks’
gestational age are at highest risk. Other significant com-
plications associated with ECLS include seizures, neuro-
logic impairment, renal failure requiring hemofiltration or
hemodialysis, hypertension, infection, and mechanical
malfunction (e.g., failure of the membrane oxygenator,
pump, heat exchanger).
TRAUMA
In children between 1 and 15 years of age, trauma is the
leading cause of death. Although motor vehicle crashes
account for most traumatic deaths, falls, bicycle accidents,
8/28/2007 11:37:46 AM
 2052 Section XIII Specialties in General Surgery

and child abuse constitute a substantial component as glutamic-oxaloacetic transaminase or serum glutamic-
well. Violence-related penetrating injury from firearms pyruvic transaminase levels are higher than 200 or 100 IU/
is becoming increasingly common. Much emphasis on L, respectively. A significant amount of peritoneal fluid
trauma research is directed toward prevention. Because in the absence of solid organ injury raises the suspicion
a large percentage of motor vehicle crash–related injury of a small bowel injury and prompts further investigation.
occurs because of absent or improper use of child restraint Although significant spleen and liver injuries are fre-
devices, community outreach education programs are quently identified, the need for operative intervention is
vital. Along these lines, programs for distribution and rare. The major indications for laparotomy in these cir-
education on the use of safety helmets for bike riding cumstances include obvious hemodynamic instability, the
and skateboarding and on the safest location within the need for blood transfusion in amounts greater than half
car for children2 are extremely important in reducing the child’s calculated blood volume (40 mL/kg) within
injury severity. Finally, active participation by pediatric the first 24 hours after injury, or obvious extravascular
surgeons in legislative efforts toward factors such as blush of IV administered contrast material. Specific treat-
firearm safety locks and use of all-terrain vehicles by ment guidelines based on CT grade of liver or spleen
young children are critical. injury have been prospectively validated by the Liver/
The management of trauma in children is similar to Spleen Trauma Study Group of the American Pediatric
that of adults and beyond the scope of the chapter. Surgical Association and are more focused to the pediat-
However, several caveats are important to consider. Just ric population.3 According to these guidelines, a patient
as in adults, the priorities during the resuscitation phase with an isolated grade I liver or spleen injury may be
are airway, breathing, and circulation. In general, a child managed without the need for admission to an intensive
who is crying on arrival to the emergency department is care unit (ICU), require no more than 2 days of hospi-
reassuring because the airway and breathing are more talization, and resume full activities and contact sports
than likely to be intact. If endotracheal intubation is after 3 weeks. At the other end of the spectrum, patients
required, an uncuffed endotracheal tube is used in chil- with isolated grade IV injuries are carefully monitored in
dren younger than 8 to 10 years of age because of the an ICU for at least the first 24 hours and remain hospital-
small size of the trachea. The appropriate endotracheal ized for no less than 5 days. Return to full activities does
tube size can be estimated visually as being equivalent not take place until 6 weeks after injury. Regardless of
to the diameter of the child’s little finger. Alternatively the injury grade and in the absence of specific indica-
the appropriate endotracheal tube inner diameter can be tions, follow-up imaging either at the time of discharge
calculated by the following formula: 4 + (patient’s age in or before resumption of normal activities is not
years) ÷ 4. Because of the soft and easily injured trachea indicated.
of young children, surgical cricothyroidotomy must never
be attempted in a child who is younger than 12 years of
age. LESIONS OF THE NECK
With regard to fluid resuscitation, crystalloid is given
as a rapid IV bolus in increments of 20 mL/kg. The ability Cystic Hygroma
to secure reliable IV access in young children may be
A cystic hygroma is a lymphatic malformation that occurs
quite challenging. In children younger than 6 years of
as a result of a maldeveloped localized lymphatic network,
age in whom an IV line cannot be secured within a rea-
which fails to connect or drain into the venous system.
sonable period, intraosseous access is considered. This
Most (75%) involve the lymphatic jugular sacs and present
is accomplished with a specially designed needle placed
in the posterior neck region (Fig. 71-3). Another 20%
under sterile conditions through the flat, anteromedial
surface of the tibia, 1 to 2 cm below the anterior tibial
tuberosity. Virtually any IV drug or fluid that may be
required during resuscitation can be safely administered
by the intraosseous route. Blood transfusion is warranted
in pediatric trauma patients who demonstrate persistent
evidence of hypovolemic shock after two boluses (total
of 40 mL/kg) of crystalloid fluid. An estimate of a child’s
entire blood volume is roughly 80 mL/kg. As a general
rule, if the need for blood transfusion within the first 24
hours after blunt abdominal trauma exceeds half the
estimated blood volume, active hemorrhage is presumed
and is usually an indication for laparotomy.
Imaging of the pediatric blunt trauma patient is pre-
dominately by computed tomography (CT) of the
abdomen and pelvis. The indications for CT include the
presence of a distracting injury such as an associated
arm or leg fracture, significant closed head injury, if the
examination is unclear or cannot be obtained because
of an uncooperative or very young child, or if the serum Figure 71-3 Cystic hygroma.

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 Chapter 71 Pediatric Surgery 2053

occur in the axilla, and the remainder are found through-

out the body, including the retroperitoneum, mediasti-
num, pelvis, and inguinal area. Roughly 50% to 65% of
hygromas present at birth, and most become apparent
by the second year of life.
Because hygromas are multiloculated cystic spaces
lined by endothelial cells, they usually present as soft,
cystic masses that distort the surrounding anatomy. The
indications for therapy are obviously cosmetic. In addi-
tion, the hygroma may expand to compress the airway,
resulting in acute airway obstruction. Prenatal recognition
of a large cystic mass of the neck is associated with sig-
nificant risk to the airway, greater association with chro-
mosomal abnormalities, and higher mortality rates.4
Improved fetal imaging modalities may allow for inter-
vention at the time of delivery based on principles of
pharmacologic maintenance of placental circulation until
endotracheal intubation is achieved. This technique is
referred to as the ex utero intrapartum therapy (EXIT)
procedure5 and is discussed later in this chapter. In addi- Figure 71-4 Branchial cleft fistula. The original site of the fistula
tion to accumulating lymph fluid, hygromas are prone to in the lower neck (arrow) has been elliptically excised, and a
infection and hemorrhage within the mass. Thus, rapid stepladder counterincision has been made higher in the neck to
changes in the size of the hygroma may necessitate more remove the entire tract. A lacrimal probe has been inserted into
urgent intervention. the tract to define its course.
Complete surgical excision is the preferred treatment;
however, this may be impossible because of the hygroma
infiltrating within and around important neurovascular
mass that may become infected. Branchial remnants may
structures. Careful preoperative magnetic resonance
also be palpable as cartilaginous lumps or cords corre-
imaging (MRI) to define the extent of the hygroma is
sponding with a fistulous tract. Dermal pits or skin tags
crucial. Operations are routinely performed with the aid
may also be evident.
of loupe magnification and a nerve stimulator. Because
First branchial remnants are typically located in the
hygromas are not neoplastic tumors, radical resection
front or back of the ear, or in the upper neck in the
with removal of major blood vessels and nerves is not
region of the mandible. Fistulas typically course through
indicated. Postoperative morbidity includes recurrence,
the parotid gland, deep, or through branches of the facial
lymphatic leak, infection, and neurovascular injury.
nerve, and end in the external auditory canal.
Injection of sclerosing agents such as bleomycin or the
Remnants from the second branchial cleft are the most
derivative of Streptococcus pyogenes OK-432 have also
common. The external ostium of these remnants is
been reported to be effective in the management of cystic
located along the anterior border of the sternocleidomas-
hygromas. Intracystic injection of sclerosants appears to
toid muscle, usually in the vicinity of the upper half to
be most effective for macrocystic hygromas, as opposed
lower third of the muscle. The course of the fistula must
to the microcystic variety.
be anticipated preoperatively because stepladder coun-
terincisions are often necessary to excise the fistula com-
Branchial Cleft Remnants pletely (Fig. 71-4). Typically, the fistula penetrates the
platysma, ascends along the carotid sheath to the level
The mature structures of the head and neck are embryo-
of the hyoid bone, and then turns medially to extend
logically derived from six pairs of branchial arches, their
between the carotid artery bifurcation. The fistula then
intervening clefts externally, and pouches internally.
courses behind the posterior belly of the digastric and
Congenital cysts, sinuses, or fistulas result from failure of
stylohyoid muscles to end in the tonsillar fossa.
these structures to regress, persisting in an aberrant loca-
Third branchial cleft remnants usually do not have
tion. The location of these remnants generally dictates
associated sinuses or fistulas and are located in the supra-
their embryologic origin and guides the subsequent oper-
sternal notch or clavicular region. These most often
ative approach. Failure to understand the embryology
contain cartilage and present clinically as a firm mass or
may result in incomplete resection or injury to adjacent
as a subcutaneous abscess.
structures.
By definition, all branchial remnants are present at the
time of birth, although they may not become clinically
Thyroglossal Duct Cyst
evident until later in life. In children, fistulas are more One of the most common lesions in the midline of the
common than external sinuses, which are more common neck is the thyroglossal duct cyst, which most commonly
than cysts. In adults, cysts predominate. The clinical pre- presents in preschool-aged children. Thyroglossal rem-
sentation may range from a continuous mucoid drainage nants are involved with the embryogenesis of the thyroid
from a fistula or sinus to the development of a cystic gland, tongue, and hyoid bone and produce midline

Ch071-X3675.indd 2053 8/28/2007 11:37:47 AM

 2054 Section XIII Specialties in General Surgery
Hyoid bone
Cyst
Figure 71-5 Thyroglossal duct cyst. There are usually branches
from the cyst that are intimate with the hyoid bone and extend
cephalad for variable distances. The Sistrunk procedure, which
involves en bloc removal of the cyst, central portion of the hyoid
bone, and tissue above to the base of the tongue is required to
minimize recurrence. (From Horisawa M, Niinomi N, Ito T:
What is the optimal depth for core-out toward the foramen
cecum in a thyroglossal duct cyst operation? J Pediatr Surg
27:710-713, 1992).
masses extending from the base of the tongue (foramen
cecum) to the pyramidal lobe of the thyroid gland. Com-
plete failure of thyroid migration results in a lingual
thyroid. Ultrasound or radionuclide imaging may there-
fore be useful to identify the presence of a normal thyroid
gland within the neck. This information would be useful
to prevent performing an inadvertent complete thyroid-
ectomy during treatment of a presumed thyroglossal
remnant.
Thyroglossal duct cysts may be located in the midline
of the neck anywhere from the base of the tongue to the
thyroid gland. Most, however, are found at or just below
the hyoid bone. The indications for surgery include
increasing size, the risk for cyst infection, or the presence
(1%-2%) of carcinoma. The classic treatment has remained
unchanged since it was described by Sistrunk in 1928
and involves complete excision of the cyst in continuity
with its tract, the central portion of the hyoid bone, and
the tissue above the hyoid bone extending to the base
of the tongue. Failure to remove these tissues will result
in a high risk for recurrence because multiple sinuses
have been histologically identified in these locations
(Fig. 71-5).
Torticollis
Torticollis simply refers to a twisted neck, which may be
either congenital or acquired. In infants with congenital
torticollis, the head is typically tilted toward the side of
the affected muscle and rotated in the opposite direction.
In many cases, a mass can be palpated within the affected
muscle. Although the true etiology is unknown, birth
Ch071-X3675.indd 2054
trauma is most frequently considered. The typical onset
of congenital torticollis is 4 to 6 weeks of age in an
otherwise healthy infant. The diagnosis is purely
clinical.
Treatment of congenital torticollis is initially conserva-
tive. Range-of-motion exercises consist of passive stretch-
ing of the affected muscle and are curative in most
infants. The average duration of required treatment is 4.7
months. Surgical resection or division of the involved
muscle is rarely necessary, but is indicated if symptoms
persist beyond 1 year.
Congenital torticollis must be distinguished from
acquired torticollis. In the former, the onset is fairly soon
(within weeks) after birth, and associated problems are
rare. Acquired torticollis occurs later and is associated
with a range of conditions, including acute myositis,
brainstem tumors, atlantoaxial subluxation, or infectious
causes such as retropharyngeal abscess, cervical adenitis,
or tonsillitis.
Cervical Lymphadenopathy
Enlarged cervical lymph nodes occur frequently in the
pediatric population, and referral to a surgeon for biopsy
is common. The etiology is overwhelmingly infectious;
however, it is important to be aware of several other
causative factors. Decisions regarding diagnostic testing
and therapy are based largely on clinical judgment and
must be derived from a thoughtful history and physical
examination.6
The distribution of enlarged lymph nodes is important
because most healthy children have small, mobile,
rubbery, palpable lymph nodes in the anterior cervical
triangle. On the other hand, nontender, fixed nodes in
the supraclavicular region are worrisome for malignancy.
Further, concern is raised regarding nodes that are larger
than 2 cm, hard, nontender, and fixed to surrounding
structures. Additional concerns for an underlying neo-
plasm are raised by a history of weight loss, night sweats,
and progressive nodal enlargement.
If a diagnostic lymph node biopsy is indicated, a pre-
operative chest radiograph is performed to exclude
associated mediastinal adenopathy. If enlarged anterior
mediastinal nodes are seen, CT of the chest is done to
determine whether there is airway compression. Failure
to recognize this preoperatively may result in life-
threatening airway obstruction during the induction of
general anesthesia. If significant airway compression is
seen, every attempt is made to perform the biopsy under
local anesthesia. Because this may not be feasible in
some children, preoperative discussion of the CT findings
with the anesthesiologist is critical. Anesthetic caveats for
this patient population include preservation of spontane-
ous ventilation during intubation, induction in the sitting
position, securing IV access in a lower extremity, and
changing the patient’s position whenever cardiorespira-
tory compromise is apparent. Access to fiberoptic and
rigid bronchoscopy, a skilled bronchoscopist, and longer
endotracheal tubes must be immediately available.
Patients with acute, bilateral cervical lymphadenitis are
usually managed conservatively because infection with
8/28/2007 11:37:48 AM

6% 2%
Figure 71-6 The main anatomic variants and incidence of esophageal atresia and tracheoesophageal fistula.
respiratory viruses is so common. These viruses include
adenovirus, influenza virus, and respiratory syncytial
virus and are often associated with symptoms of cough,
rhinorrhea, and sinus congestion. Acute unilateral pyo-
genic lymphadenitis is caused by Staphylococcus aureus
and group A Streptococcus species in more than 80% of
cases. In early cases, an oral antibiotic directed primarily
toward gram-positive organisms is indicated. After the
nodes become fluctuant, needle aspiration or incision
and drainage will be necessary.
Cat-scratch disease is thought to account for as much
as 3% of acute cervical lymphadenopathy and is caused
by the organism Bartonella henselae. A history of expo-
sure to cats is helpful, but not always present. The diag-
nosis may be made by polymerase chain reaction from
nodal tissue. Antibiotic therapy is not recommended
because the disease is self-limited in most cases.
A less common infectious cause for cervical lymphad-
enitis is nontuberculous Mycobacteria infection. Typi-
cally, the nodes are fluctuant, and the overlying skin has
a violaceous appearance, but is not particularly tender.
Occasionally, the nodes will drain spontaneously with
the formation of mature sinus tracts. The diagnosis is
made by positive cultures for nontuberculous acid-fast
bacilli together with a positive tuberculin skin test.
Because most of the nontuberculous Mycobacteria are
resistant to conventional chemotherapy, surgical excision
is the treatment of choice.7 Incision and drainage
alone are associated with a high rate of recurrence and
poor healing of the wound. In contrast with patients
with active tuberculous infections, there is no indica-
tion for isolation of patients with nontuberculous
lymphadenitis.
ALIMENTARY TRACT
Esophageal Atresia and
Tracheoesophageal Fistula
Esophageal atresia (EA) is a congenital interruption or
discontinuity of the esophagus resulting in esophageal
obstruction. Tracheoesophageal fistula (TEF) is an abnor-
Ch071-X3675.indd 2055
Chapter 71 Pediatric Surgery 2055
85% 1% 2%
mal communication (fistula) between the esophagus
and trachea. Esophageal atresia may be present with or
without a TEF. Alternatively, a TEF can occur without EA.
The incidence and range of anatomic variants is depicted
in Figure 71-6. The prevalence of EA or TEF is 2.6 to 3
per 10,000 births, with a slight male predominance.
Associated Anomalies
The etiology of the disturbed embryogenesis is presently
unknown. Roughly one third of infants with EA or TEF
have low birth weight, and two thirds of infants have
associated anomalies. There is a nonrandom, nonheredi-
tary association of anomalies in patients with EA or TEF
that must be considered under the acronym VATER (ver-
tebral, anorectal, tracheal, esophageal, renal or radial
limb). Another acronym that is commonly used is
VACTERL (vertebral, anorectal, cardiac, tracheal, esopha-
geal, renal, and limb).
Clinical Presentation
The diagnosis of EA is entertained in an infant with
excessive salivation along with coughing or choking
during the first oral feeding. A maternal history of poly-
hydramnios is often present. In a baby with EA and TEF,
acute gastric distention may occur as a result of air enter-
ing the distal esophagus and stomach with each inspired
breath. Reflux of gastric contents into the distal esopha-
gus will traverse the TEF and spill into the trachea, result-
ing in cough, tachypnea, apnea, or cyanosis. The
presentation of isolated TEF without EA may be more
subtle and often beyond the newborn period. In general,
these infants have choking and coughing associated with
oral feeding.
Diagnosis
The inability to pass a nasogastric tube into the stomach
of the neonate is a cardinal feature for the diagnosis of EA.
If gas is present in the gastrointestinal tract below the dia-
phragm, an associated TEF is confirmed (Fig. 71-7A). On
the other hand, inability to pass a nasogastric tube in an
infant with absent radiographic evidence for gastrointesti-
nal gas is virtually diagnostic of an isolated EA without TEF
(see Fig. 71-7B). These simple rules provide the correct
8/28/2007 11:37:48 AM
 2056 Section XIII Specialties in General Surgery
A B
Figure 71-7 A, Plain chest radiograph of infant with pure esophageal atresia. Note the inability to pass the naso-
gastric tube (arrow) into the stomach and absence of gas within the abdomen. B, Plain chest radiograph of infant
with esophageal atresia and tracheoesophageal fistula (TEF). The esophageal atresia is suggested by the inability
to pass the nasogastric tube into the stomach and the surrounding gas-filled proximal esophagus (arrows). The
TEF is verified by the presence of gas within the abdomen.
diagnosis in most cases. Occasionally, a small amount of
isotonic contrast may be given by mouth to demonstrate
the level of the proximal EA pouch or the presence of a
TEF, but this is rarely necessary. In fact, the risk for aspira-
tion with studies of this type is generally high.
Preoperative Evaluation and Management
The immediate care of an infant with EA or TEF includes
decompression of the proximal EA pouch with a sump
type of tube placed to continuous suction. This will
prevent spillover of oral secretions into the trachea. The
presence of the TEF may be life threatening; positive-
pressure ventilation may be inadequate to inflate the
lungs because air is directed into the TEF through the
path of least resistance. Ventilation may further be com-
pounded by the resultant gastric distention. In these cir-
cumstances, manipulation of the endotracheal tube distal
to the TEF (e.g., right main-stem intubation) may mini-
mize the leak and permit adequate ventilation. Further,
placement of an occlusive balloon (Fogarty) catheter into
the fistula through a bronchoscope may be useful. Finally,
urgent thoracotomy with direct ligation of the fistula, but
without repair of the EA, may be required. In these cases,
performance of a gastrostomy to decompress the dis-
tended stomach is avoided because it may result in the
abrupt inability to ventilate the patient.
In the preoperative period, it is necessary to perform
a thorough physical examination with particular attention
to the aforementioned VACTERL anomalies. A preopera-
tive echocardiogram is essential to evaluate the presence
or absence of congenital heart disease as well as to define
the side of the aortic arch. A right thoracotomy is typically
done for the repair of EA or TEF in patients with a normal
left-sided aortic arch. However, for infants with a right-
Ch071-X3675.indd 2056
sided arch, a left thoracotomy would be preferred, and
a higher incidence of aortic arch anomalies (vascular
rings) and postoperative complications must be antici-
pated.8 Additional preoperative imaging studies include
ultrasonography of the spine and kidneys.
Surgical Management
The traditional surgical treatment of the most common
EA or TEF involves an extrapleural thoracotomy through
the fourth intercostal space. More recently, thoracoscopic
repair has been described by several pediatric centers
with advanced minimally invasive expertise.9 A bronchos-
copy is performed to identify the relative site of the
fistula, exclude the presence of a second fistula, and
delineate the bronchial anatomy. On the right side, the
azygos vein is divided to reveal the underlying TEF. The
TEF is dissected circumferentially and then ligated using
interrupted, nonabsorbable sutures. The proximal esoph-
ageal pouch is then mobilized as high as possible to
afford a tension-free esophageal anastomosis. The blood
supply to the upper esophageal pouch is generally robust
and based on arteries derived from the thyrocervical
trunk. On the other hand, the blood supply to the lower
esophagus is more tenuous and segmental, originating
from intercostal vessels. As such, significant mobilization
of the lower esophagus is not done, so as to avoid isch-
emia at the site of the esophageal anastomosis. The
anastomosis is performed using either a single- or double-
layer technique. The rates of anastomotic leak are slightly
higher with the single-layer anastomosis, whereas the
rates of esophageal stricture are higher with the double-
layer technique.
If the two ends of the esophagus cannot be joined
without significant tension, there are several options. The
8/28/2007 11:37:48 AM

first is to suture the divided end of the distal esophagus
to the prevertebral fascia, mark its location with a metal
clip, and close the thoracotomy. Over time (2-3 months),
the proximal esophageal pouch may grow such that a
subsequent thoracotomy may permit a primary esopha-
geal anastomosis. A circular or spiral esophagomyotomy10
of the upper pouch may also be done to gain esophageal
length and facilitate a primary anastomosis. Another tech-
nique involves placement of traction sutures through the
proximal and distal ends of the esophagus, brought out
through the chest. These sutures are progressively tight-
ened, and a primary esophageal anastomosis is per-
formed after several days.11 Alternatively, a cervical
esophagostomy may be constructed and a formal esopha-
geal replacement performed at a later date.
In patients with pure EA, the gap between the two
esophageal ends is frequently wide, thus preventing a
primary anastomosis in the newborn period. In these
patients, the traditional approach is to perform a cervical
esophagostomy for drainage of oral secretions and inser-
tion of a gastrostomy for enteral feeding. An esophageal
replacement using the stomach, small intestine, or colon
is then performed at about 1 year of age. More recently,
it has become apparent that the two ends of the esopha-
gus may spontaneously grow such that a primary anas-
tomosis may be accomplished by 4 months of age.12
Thus, insertion of a gastrostomy in the neonatal period
for feeding may be the only necessary intervention. The
swallowing of saliva may actually promote elongation of
the upper pouch, and an esophagostomy is therefore
avoided.
In patients with pure TEF without EA, the site of the
TEF is usually in the region of the thoracic inlet. As such,
the surgical approach is through a cervical incision. After
the induction of anesthesia, but before making the inci-
sion, it is often helpful to cannulate the TEF with a
guidewire to facilitate identification of the TEF.
Outcome
The mortality rate of EA or TEF is directly related to the
associated anomalies—particularly cardiac defects and
chromosomal abnormalities. In the absence of these
factors, survival of more than 95% of patients is expected.13
Postoperative complications unique to EA or TEF include
esophageal motility disorders, gastroesophageal reflux
(25%-50%), anastomotic stricture (15%-30%), anastomotic
leak (10%-20%), and tracheomalacia (8%-15%).
Gastroesophageal Reflux
Vomiting during infancy is a common occurrence and
can be difficult to distinguish from chronic gastroesopha-
geal reflux (GER) that ultimately requires surgical correc-
tion. Although the diagnosis and surgical management of
GER are similar between adults and children, there are
several major differences that must be understood.
Clinical Presentation
Although the symptoms of GER can often be obtained
easily in adults, the recognition of symptoms in young
children may be more subtle. Rather than complain of
Ch071-X3675.indd 2057
Chapter 71 Pediatric Surgery 2057
heartburn, children tend to associate pain with eating. As
such, they may be irritable during or after feeding, or
limit their formula intake altogether. This may be identi-
fied by failure to thrive (FTT). Another cause of FTT in
infants with GER is the nutritional consequences of
reduced caloric intake due to protracted emesis. Other
unique symptoms include life-threatening episodes of
apnea, termed near-miss sudden infant death syndrome
(SIDS). In a child with documented GER, an episode of
near-miss SIDS is an absolute indication for antireflux
surgery. Respiratory symptoms of GER in children may
manifest as chronic cough, hoarseness, recurrent pneu-
monias, or asthma. Persistent asthma may be due to GER
in up to 75% of children, a significant proportion of
whom have no other apparent symptoms of GER. Acid
suppression alone has not been shown to alleviate the
asthma symptoms, and operative therapy in these patients
may be considered sooner.14
Many children referred for antireflux surgery are neu-
rologically impaired, usually secondary to such factors as
metabolic conditions, head trauma, or birth asphyxia. As
such, most of these patients require permanent feeding
access in the form of a gastrostomy tube. Thus, antireflux
surgery is often entertained at the time of the gastrostomy
tube insertion, especially in patients who are unable to
reliably protect their airway, or who already have signifi-
cant vomiting associated with intragastric tube feeding.
Preoperative Evaluation
The evaluation of the child with GER involves several
studies, each designed to provide different information.
The initial study includes an upper gastrointestinal (UGI)
radiographic series. The UGI does not correlate well with
the presence or absence of GER but is important to
exclude other causes of vomiting in children. These
would include malrotation, antral web, foregut duplica-
tion cysts, and pyloric or duodenal stenosis. The gold
standard test for delineating pathologic from physiologic
GER is continuous (18-24 hour) esophageal pH monitor-
ing.15 Risk assessment for the development of esophagitis
can be derived from a reflux index, which takes into
account the percentage of time the lower esophageal pH
is less than 4. A reflux index of greater than 11% in infants
up to 1 year of age, or greater than 6% in older children,
is considered pathologic. The limitation of this study is
that it nicely delineates risk for acid injury to the esopha-
gus but may fail to detect pathologic GER in patients who
have symptoms related to pulmonary aspiration. In these
circumstances, the episode of reflux may be of sufficient
magnitude to cause pneumonia or bronchospasm, but
cleared efficiently from the esophagus so as to be inter-
preted as normal. Multichannel intraluminal impedance
studies have been demonstrated to be equivalent to pH
probe testing and may add benefit for the detection of
reflux of gastric contents that are nonacidic.16 Nuclear
scintigraphy involves labeling food or formula with a
radioisotope and then measuring the number of post-
prandial episodes of GER and aspiration events. The
advantages of this technique include the ability to identify
nonacid GER events and to quantitate gastric emptying.
A drawback is that it is extremely sensitive and therefore
8/28/2007 11:37:48 AM
 2058 Section XIII Specialties in General Surgery
is unable to distinguish between pathologic and non-
pathologic GER. Further, normative data for the pediatric
population are lacking. Finally, endoscopic visualization
of the esophagus, larynx, and trachea are all complemen-
tary studies to confirm the presence of acid injury.
Conservative Management
Nonoperative measures to reduce GER include thicken-
ing of formula with cereal, reducing the volume of
feeding, and postural maneuvers. In addition, pharmaco-
logic acid suppression may be useful. Indications for
surgical intervention include severe GER that is unre-
sponsive to aggressive medical management. In addition,
surgery is generally warranted in patients with life-
threatening near-miss SIDS episodes, FTT, or esophageal
stricture. Other relative indications include those requir-
ing complex surgical airway reconstruction,17 neurologic
impairment requiring permanent feeding access, or a
history of recurrent pneumonias or persistent asthma.
Operative Management
As in adults, multiple operations have been designed for
children with GER. The gold standard procedure remains
Nissen’s fundoplication. In the pediatric population, this
can be done open or laparoscopically with similar results.
In severely neurologically impaired patients, a complete
esophagogastric disconnection with Roux-en-Y esoph-
agojejunostomy has been proposed but is associated with
significant morbidity.18
Outcome
The overall results for antireflux surgery in children
are excellent. The risk for recurrent GER is elevated in
the neurologically impaired population, in the presence
of severe underlying lung disease, or with a history of
prematurity.19
Hypertrophic Pyloric Stenosis
Hypertrophic pyloric stenosis (HPS) is one of the most
common gastrointestinal disorders during early infancy,
with an incidence of 1 in 3000 to 4000 live births. This
condition is most common between the ages of 2 and 8
weeks. In HPS, hypertrophy of the circular muscle of the
pylorus results in constriction and obstruction of the
gastric outlet. Gastric outlet obstruction leads to nonbil-
ious, projectile emesis; loss of hydrochloric acid with the
development of hypochloremic, metabolic alkalosis; and
ultimately dehydration. Treatment of this condition is by
surgical mechanical distraction of the pyloric ring. There
is currently no place for medical management of HPS.
Etiology
The cause for pyloric stenosis is unknown, and multiple
factors have been implicated. Ethnic origin is important
because the highest incidence is found among whites of
Scandinavian decent and lowest risk among African
Americans and Chinese. Males outnumber females in
every series by a ratio of 4 : 1 or 5 : 1. There is a higher
risk for developing HPS in offspring of parents with this
Ch071-X3675.indd 2058
condition, and in many series, first-born males are fre-
quently encountered.
Clinical Presentation
Infants with HPS typically present with projectile emesis
or frequent episodes of nonbilious emesis. Occasionally,
the vomitus may be brown or blood streaked, but it is
always nonbilious. Visible gastric peristalsis may be seen
as a wave of contraction from the left upper quadrant to
the epigastrium. The infants usually feed vigorously
between episodes of vomiting.
Diagnosis
Palpation of the pyloric tumor (also called the olive) in
the epigastrium or right upper quadrant by a skilled
examiner is pathognomonic for the diagnosis of HPS. If
the olive is palpated, no additional diagnostic testing is
necessary. When the olive cannot be palpated, the diag-
nosis of HPS can be made with an ultrasound exam or
fluoroscopic UGI series. These imaging tests are similar
in terms of sensitivity and specificity for the diagnosis of
HPS. The UGI is useful for the evaluation of other causes
of vomiting, whereas the absence of radiation exposure
and cost make the ultrasound the usual preferred study.
A persistent pyloric muscle thickness of more than 3 to
4 mm or pyloric length of more than 15 to 18 mm in the
presence of functional gastric outlet obstruction is gener-
ally considered diagnostic.20 Recently, it was demon-
strated that preoperative palpation of an olive did not
affect the frequency of ultrasound imaging.21
Treatment
Treatment of HPS is by a pyloromyotomy. This consists
of cutting across the abnormal pyloric musculature while
preserving the underlying mucosa (Fig. 71-8). This can
be done through a traditional right upper quadrant inci-
sion, through a periumbilical incision, or laparoscopically
Figure 71-8 Pyloromyotomy for hypertrophic pyloric stenosis.
The thickened pyloric musculature has been cut and then spread
apart to reveal the underlying mucosa.
8/28/2007 11:37:49 AM

with similar outcomes.22 Before surgery, it is important
that the infant is hydrated with IV fluids to establish a
normal urine output. It is important that the underlying
metabolic alkalosis is slowly corrected with normal saline.
Potassium is not given until the intravascular volume has
been restored and normal urine output has resumed.
Because the infant with underlying metabolic alkalosis
will compensate with respiratory acidosis, postoperative
apnea may occur. Thus, the serum HCO3− needs to be
normalized before surgery.
Postoperatively, infants are usually allowed to resume
enteral feedings. Vomiting after surgery occurs frequently
but is usually self-limited. Complications specific to the
pyloromyotomy include incomplete myotomy, mucosal
perforation (usually at the duodenal end), and wound
infection.
Intestinal Atresia
Duodenal Atresia
In contrast with more distal intestinal atresias, duodenal
atresia (DA) is thought to occur as a result of failure of
vacuolization of the duodenum from its solid cord stage.
The range of anatomic variants includes duodenal steno-
sis, mucosal web with intact muscular wall (so-called
windsock deformity), two ends separated by a fibrous
cord, or complete separation with a gap within the
duodenum.
Associated Anomalies
DA is associated with several conditions, including pre-
maturity, Down syndrome, maternal polyhydramnios,
malrotation, annular pancreas, and biliary atresia. Other
anomalies, such as cardiac, renal, esophageal, and ano-
rectal anomalies, are also common. In most cases, the
duodenal obstruction is distal to the ampulla of Vater,
and infants present with bilious emesis in the neonatal
period. In patients with a mucosal web, the symptoms
of postprandial emesis may occur later in life.
Diagnosis
The classic plain abdominal radiograph of DA is termed
the double-bubble sign (air-filled stomach and duodenal
bulb; Fig. 71-9). In cases in which there is no distal air,
the diagnosis is secured, and no further studies are neces-
sary. On the other hand, if distal air is present, an upper
gastrointestinal contrast study is performed fairly rapidly.
This study is important not only to confirm the diagnosis
of duodenal stenosis or atresia but also to exclude midgut
volvulus, which would constitute a surgical emergency.
Treatment
The management of DA is by surgical bypass of the
duodenal obstruction as either a side-to-side or proximal
transverse–to–distal longitudinal (diamond-shaped) duo-
denoduodenostomy. When the proximal duodenum is
markedly dilated, a tapering duodenoplasty may be per-
formed to reduce the duodenal caliber and may improve
postoperative gastric emptying. In patients with a duo-
denal mucosal web, the web is excised transduodenally.
The ampulla is often associated with the web itself and
Ch071-X3675.indd 2059
Chapter 71 Pediatric Surgery 2059
Figure 71-9 Plain abdominal radiograph demonstrating the
typical double-bubble appearance of duodenal atresia. The large
gas-filled stomach is visualized along with the dilated proximal
duodenum. There is no gas beyond the duodenum.
must therefore be identified and preserved during the
web excision.
Jejunoileal Atresia
Although several mechanisms have been proposed to
explain the findings of jejunoileal atresia (JIA), the pre-
vailing theory is that of an intrauterine focal mesenteric
vascular accident. The spectrum of gross pathologic find-
ings includes simple stenosis, complete interruption of
the intestinal lumen with or without a fibrous cord
attached to the distal bowel, a missing segment of bowel
and mesentery, or multiple atresias. One final type is
referred to as the apple-peel or Christmas tree deformity
(Fig. 71-10). This atresia is unique from the standpoint
that the obstruction is usually in the proximal jejunum,
which is supplied by the entire superior mesenteric artery
(SMA). There is then a gap in the mesentery, and the
remainder of the small intestine is coiled around the
ileocolic branch of the SMA, which is perfused retrograde
from the middle colic artery. This tenuous blood supply
has obvious implications for reanastomosis and the
potential for ischemic necrosis due to an antenatal vol-
vulus. As such, many of these infants with this type of
atresia are born with reduced intestinal length.
Clinical Presentation
The clinical presentation is typically dependent on the
level of obstruction. In proximal atresia, abdominal dis-
tention is less frequent, and bilious emesis is usually
present. Plain abdominal radiographs typically reveal air-
fluid levels with absent distal gas. If the atresia is distal,
abdominal distention may be present. A preoperative
barium enema may be useful to exclude multiple atresias,
which may be present in 10% to 15% of cases. In contrast
8/28/2007 11:37:49 AM
 2060 Section XIII Specialties in General Surgery
Figure 71-10 Proximal jejunal atresia of the apple-peel or Christ-
mas tree variant. The dilated proximal jejunum (arrows) is sup-
plied by the superior mesenteric artery (SMA). There is a gap in
the mesentery, and the remainder of the small intestine is coiled
around the ileocolic branch of the SMA, which is perfused ret-
rograde from the middle colic artery.
with DA, JIA is usually not associated with other anoma-
lies. One exception is cystic fibrosis, which may be
present in roughly 10% of cases.
Management
The treatment of JIA is to reestablish intestinal continuity.
In the presence of multiple atresias, it is imperative to
preserve as much intestinal length as possible. This may
require multiple anastomoses over an endoluminal stent.
If the proximal intestine is significantly dilated, peristalsis
will be perturbed. As such, on the one hand a tapering
enteroplasty of the dilated bowel is performed if the
remnant intestinal length is short. On the other hand, the
dilated bowel needs to be resected if the remnant small
bowel length is normal. The overall survival for infants
with JIA atresia is more than 90%23 and unrelated to the
type of atresia encountered. The most significant associ-
ated morbidity is the short gut syndrome.
Anomalies of Intestinal Rotation and Fixation
Most intestinal rotation and fixation abnormalities become
clinically evident during infancy and childhood. The true
incidence of rotational anomalies of the midgut is difficult
to determine and has been reported to occur with a fre-
quency of 1 in 6000 live births. An understanding of
the embryology of the intestine is essential in the recogni-
tion and appropriate surgical management of these
conditions.
Normal Intestinal Rotation
The midgut normally herniates out of the abdominal
cavity through the umbilical ring at about the fourth week
of development in all human fetuses. By the 10th week
of gestation, the intestine returns to the abdominal cavity
and rotates around the axis of the SMA for 270 degrees
Ch071-X3675.indd 2060
in a counterclockwise direction. The final position of the
ligament of Treitz is in the left upper quadrant and the
cecum in the right lower quadrant of the abdomen. Inter-
ruption or reversal of any of these coordinated move-
ments permits an embryologic explanation for the range
of anomalies seen.
Abnormal Intestinal Rotation
Complete nonrotation of the midgut is the most fre-
quently encountered anomaly and occurs when neither
the duodenojejunal limb nor the cecocolic limb under-
goes rotation. As a result, there is no duodenal C-loop,
and the ligament of Treitz is located on the right side of
the abdomen. Likewise, the cecum has failed to rotate
and is present in the left side of the abdomen. In non-
rotation, the proximal jejunum and ascending colon are
fused together as one pedicle, through which the blood
supply to the entire midgut (SMA) is located. It is this
pedicle on which a midgut volvulus occurs, leading to
ischemic necrosis of the entire midgut.
Nonrotation of the duodenojejunal limb followed by
normal rotation and fixation of the cecocolic limb will
result in duodenal obstruction due to mesenteric (Ladd’s)
bands originating from the colon and extending over the
duodenum to end in the retroperitoneum. In this situa-
tion, a midgut volvulus is less likely because the base of
the mesentery is relatively wide and fixed to the posterior
abdomen. Duodenal obstruction from Ladd’s bands is
usually heralded by bilious emesis. Several other abnor-
malities are possible with any combination of incomplete,
absent, or reverse rotation of the duodenojejunal limb
followed by varied rotation patterns of the cecocolic
limb.
Clinical Presentation
Rotational anomalies may manifest clinically in several
different ways; however, the main symptom complexes
may be grouped together as those related to volvulus,
duodenal obstruction, or intermittent or chronic abdomi-
nal pain, or as an incidental finding in an otherwise
asymptomatic patient. Most patients develop symptoms
during the first month of life.
Midgut volvulus is a true surgical emergency because
delay in operative correction is associated with a high
risk for intestinal necrosis and subsequent death. The
sudden appearance of bilious emesis in a newborn is the
classic presentation. Although bilious emesis is most
often due to other causes, it is critical to exclude midgut
volvulus. When clinical signs of intestinal compromise
begin to appear, the ability to salvage the patient or any
significant length of small bowel may be gone.
Midgut volvulus may also be incomplete or intermit-
tent. Patients may have chronic abdominal pain, intermit-
tent episodes of emesis (which may be nonbilious), early
satiety, weight loss, failure to thrive, or malabsorption
and diarrhea. With partial volvulus, the resultant mesen-
teric venous and lymphatic obstruction may impair nutri-
ent absorption and produce protein loss into the gut
lumen as well as mucosal ischemia and melena as a result
of arterial insufficiency.
8/28/2007 11:37:49 AM

Diagnosis
The preoperative evaluation of a child with a suspected
rotational anomaly of the intestine includes plain abdomi-
nal radiographs and a UGI contrast series. Occasionally,
plain abdominal radiographs may reveal evidence of
intestinal obstruction; however, the most common find-
ings are nonspecific. The UGI contrast series remains the
gold standard for the diagnosis. A key element in the
diagnosis of rotational abnormalities of the intestine is
the position of the ligament of Treitz. This is normally
located to the left of midline and at the level of the gastric
antrum. In the presence of a volvulus, the site of obstruc-
tion is usually the third portion of the duodenum and
has the appearance of a bird’s beak.
In the acutely ill child with midgut volvulus and
obstruction, urgent operative correction is indicated, and
little time is available for IV fluid resuscitation, placement
of a nasogastric tube and Foley catheter, type and
crossmatch for blood, and administration of broad-
spectrum antibiotics. Time is critical in terms of intestinal
salvage.
Operative Management: Ladd’s Procedure
Surgical management of most rotational anomalies of the
intestine is Ladd’s procedure. On entering the peritoneal
cavity, the entire bowel is immediately exposed. If a
volvulus is encountered, it needs to be remembered that
in most cases, the volvulus twists in a clockwise direction
and thus needs to be untwisted in a counterclockwise
manner. After detorsion, the intestine may be congested
and edematous, and some areas may appear necrotic.
Placement of warm sponges and observation for a period
of time may improve the appearance of the intestine
when the vascular integrity has been compromised. If
areas of the bowel are obviously necrotic, resection
with creation of a stoma is performed. Because it is
imperative to preserve as much intestine as possible,
marginal or questionable segments of bowel are left in
place and a second-look procedure performed within 24
to 36 hours. Next, Ladd’s bands are divided as they
extend from the ascending colon across the duodenum
and attach to the posterior aspect of the right upper
quadrant. To prevent extramural compression of the duo-
denum and recurrent obstruction, the bands must be
lysed completely on both lateral and medial aspects of
the duodenum. In dividing the medial bands, the distance
between the duodenum and ascending colon is increased.
Broadening this mesenteric base will reduce the inci-
dence of volvulus. There has been no demonstrated
benefit to pexing the cecum or duodenum to the abdomi-
nal wall. In neonates, a balloon catheter may be passed
through the mouth and advanced beyond the pylorus
into the distal duodenum to exclude an intraluminal
obstruction. An incidental appendectomy is then per-
formed because the cecum will ultimately lie on the left
side of the abdomen after this procedure. The intestine
is replaced into the abdominal cavity with the small
bowel lying entirely on the right side while the colon is
positioned on the left.
Ch071-X3675.indd 2061
Chapter 71 Pediatric Surgery 2061
Outcome
Recurrent volvulus is relatively infrequent but must be of
prime concern in patients presenting with obstructive
symptoms at any time postoperatively. More commonly,
the cause for postoperative obstruction is adhesive bands.
Gastrointestinal motility disturbances are also frequent.
Midgut volvulus accounts for roughly 18% of cases of
short gut syndrome in the pediatric population. Urgent
recognition and management is the most important factor
in preventing this complication.
Necrotizing Enterocolitis
Necrotizing enterocolitis (NEC) is the most common gas-
trointestinal emergency in the neonatal period. Prematu-
rity is the single most important risk factor, although other
factors such as ischemia, bacteria, cytokines, and enteral
feeding are all likely significant. The advent of exogenous
surfactant and improved methods of mechanical ventila-
tion are contributing to greater numbers of premature
infants at risk for developing NEC. Despite the tremen-
dous impact of NEC on neonatal morbidity and mortality,
progress in understanding this condition is hampered by
the fact that a reliable animal model for NEC does not
exist.
Clinical Presentation
The development of NEC is unusual in the first few days
of life. About 80% of cases, however, occur within the
first month of life. The clinical presentation of NEC is
often nonspecific and unpredictable. Clinical signs include
irritability, temperature instability, poor feeding, or epi-
sodes of apnea or bradycardia. More specific signs include
abdominal distention, vomiting, feeding intolerance, or
passage of a bloody stool. As NEC progresses, systemic
sepsis develops with cardiorespiratory deterioration,
coagulopathy, and death. The radiographic hallmark of
NEC is pneumatosis intestinalis (Fig. 71-11). Pneumatosis
is composed of hydrogen gas generated by bacterial fer-
mentation of luminal substrates. Other radiographic find-
ings may include portal venous gas, ascites, fixed loops
of small bowel, or free air. The distal ileum and ascend-
ing colon are the usual sites affected, although the entire
gastrointestinal tract (NEC totalis) may also be involved.
Conservative Management
After the diagnosis of NEC has been established, initial
management consists of bowel rest with nasogastric tube
decompression, fluid resuscitation, blood and platelet
transfusion, and administration of broad-spectrum antibi-
otics. Medical management continues for 7 to 10 days
and is successful in roughly half of cases. The absolute
indication for operative management of NEC is the pres-
ence of intestinal perforation as revealed by the identifi-
cation of free air on plain abdominal radiographs. Other
relative indications for surgery include overall clinical
deterioration, abdominal wall cellulitis, worsening acido-
sis, falling white blood cell or platelet count, palpable
abdominal mass, or a persistent fixed loop on repeated
abdominal radiographs. The decision to proceed with
8/28/2007 11:37:50 AM
 2062 Section XIII Specialties in General Surgery
Figure 71-11 Plain abdominal radiograph of an infant with nec-
rotizing enterocolitis demonstrating diffuse pneumatosis intesti-
nalis. In addition to the typical ground-glass appearance, linear
gas corresponding with the submucosal plane of the bowel wall
is easily visualized (arrows).
surgery can be difficult and must be weighed against the
risks of laparotomy in an already compromised prema-
ture infant.
Surgical Management
The general principles of surgical management of NEC
include resection of all nonviable segments of intestine
with creation of a stoma. All efforts need to be made to
preserve as much intestinal length as possible. As such,
it may be necessary to resect multiple sites of necrotic
bowel, preserve intervening segments of viable intestine,
and create multiple stomas. In cases in which the bowel
is ischemic, but not frankly necrotic, a second-look oper-
ation may be performed after 24 hours. Bowel resection
with primary reanastomosis may be considered in the
rare infant with focal involvement of NEC and minimal
peritoneal contamination who is very stable in the operat-
ing room. The risks for anastomotic leak and stricture
formation have tempered widespread enthusiasm for this
approach.
Another, more recent operative approach to the man-
agement of the infant with NEC whose intestine has
perforated is bedside placement of peritoneal drains
under local anesthesia. Drainage of the contaminated
peritoneal fluid may improve ventilation and halt the
progression of sepsis in select very ill, preterm infants.
Surprisingly, drainage of the peritoneum may be the only
necessary intervention in a few patients. The data to
support peritoneal drainage as an accepted mode of
treatment for NEC were recently established in a multi-
center, randomized prospective clinical trial.24 In this
study, survival, need for parenteral nutrition, and length
Ch071-X3675.indd 2062
of hospital stay were similar for NEC infants weighing
less than 1500 g treated by either peritoneal drainage or
laparotomy.
Outcome
The overall mortality rate for surgically managed NEC
ranges from 10% to 50%. NEC is currently the single most
common cause of short gut syndrome in children.25 Intes-
tinal strictures may develop after either medical or surgi-
cal management of NEC in roughly 10% of infants. The
most common site of involvement is the splenic flexure
of the colon. Because of the risk for stricture, a radio-
graphic contrast study of the distal intestine needs to be
done before elective stoma closure. Neurodevelopmental
delay is also a frequent long-term problem in these
infants.
Meconium Syndromes
The meconium syndromes of infancy represent a complex
group of gastrointestinal conditions associated with cystic
fibrosis (CF), with considerable overlap in clinical pre-
sentation and management. Cystic fibrosis results from a
mutation within the cystic fibrosis transmembrane regula-
tor (CFTR) gene and is autosomal recessive. Therefore,
both parents must be carriers. It is estimated that 3.3%
of the white population in the United States are asymp-
tomatic carriers of the mutated CF gene. The abnormal
chloride transport in patients with CF results in tenacious,
viscous secretions affecting a wide variety of organs,
including the intestine, pancreas, lungs, salivary glands,
reproductive organs, and biliary tract. The clinical pre-
sentation of the meconium syndromes ranges from a
meconium plug to simple and complicated meconium
ileus.
Meconium Plug
Meconium plug syndrome is a frequent cause of neonatal
intestinal obstruction and associated with multiple condi-
tions, including Hirschsprung’s disease, maternal diabe-
tes, hypothyroidism, and CF. Although most children with
meconium plug syndrome are normal, further studies to
exclude Hirschsprung’s disease and CF are warranted.
Typically, affected infants are often preterm and present
with signs and symptoms of distal intestinal obstruction.
Abdominal distention is a prominent feature. Plain
abdominal radiographs reveal multiple dilated loops of
intestine. The diagnostic and therapeutic procedure of
choice is a water-soluble contrast enema. This often
results in the passage of a plug of meconium and relief
of the obstruction (Fig. 71-12).
Simple Meconium Ileus
Meconium ileus in the newborn represents the earliest
clinical manifestation of CF and affects roughly 15% of
patients with this inherited disease. In North America,
virtually all white neonates with meconium ileus have
CF. In simple meconium ileus, the terminal ileum is
dilated and filled with thick, tarlike, inspissated meco-
nium. Smaller pellets of meconium are found in the more
distal ileum, leading into a relatively small colon. In
8/28/2007 11:37:51 AM

Figure 71-12 Meconium plug. This plug of meconium was passed
following a contrast enema performed in an infant with abdomi-
nal distention and obstipation for 48 hours. After passage of the
plug, the infant began to pass stool normally.
patients with simple meconium ileus, important plain
abdominal radiographic findings include dilated, gas-
filled loops of small bowel, absence of air-fluid levels,
and a mass of meconium within the right side of the
abdomen mixed with gas to give a ground-glass or soap
bubble appearance.
The initial diagnostic study of choice is a contrast
enema using water-soluble, ionic contrast solution. The
exact type of contrast material is inconsistent among
pediatric radiologists; however, Gastrografin is probably
the most frequently used. Because the contrast agents are
typically hypertonic relative to serum, it is important that
the infants are well hydrated and electrolytes and vital
signs carefully monitored following the procedure. It is
important that the contrast reach the ileum into the area
of inspissated meconium. This is successful in relieving
the obstruction in up to 75% of cases, with a bowel per-
foration rate of less than 3%.
Operative Management
The operative management of simple meconium ileus is
required when the obstruction cannot be relieved with
contrast enema. Historically, the dilated terminal ileum
was resected, and various types of stomas were created.
This allowed for a very sick neonate to recover who
would have otherwise died. More recently, simple evacu-
ation of the luminal meconium without the need to create
a stoma is all that is necessary in most cases. This is ac-
complished by open laparotomy, and a small enterotomy
is made in the dilated terminal ileum. A red rubber cath-
eter is used to irrigate the proximal and distal bowel with
either warmed saline solution or 4% N-acetylcysteine.
The latter solution serves to break the disulfide bonds
within the meconium and facilitate separation from the
bowel mucosa. The meconium is either manipulated into
the distal colon or removed through the enterotomy, with
care taken to avoid peritoneal contamination. After the
obstruction is relieved, the procedure is concluded by
closure of the enterotomy in two layers. In cases in which
Ch071-X3675.indd 2063
Chapter 71 Pediatric Surgery 2063
the meconium evacuation is incomplete, a T tube may
be left in place within the ileum to facilitate continued
postoperative irrigation.
Complicated Meconium Ileus
Meconium ileus is considered complicated when perfora-
tion of the intestine has taken place. This may occur in
utero or the early neonatal period. Meconium within the
peritoneal cavity results in severe peritonitis with a dense
inflammatory response and calcification. The presenta-
tion of complicated meconium ileus is variable and
includes formation of a meconium pseudocyst, adhesive
peritonitis with or without secondary bacterial infection,
or ascites.
The diagnosis of CF is usually confirmed in the post-
operative period. The pilocarpine iontophoresis sweat
test revealing a chloride concentration greater than
60 mEq/L is the most reliable and definitive method to
confirm the diagnosis of CF. This test may not be reliable
in infants and is usually performed later. A more immedi-
ate test includes detection of the mutated CFTR gene.
This test, coupled with a careful family history and clini-
cal presentation, permits confirmation of the diagnosis in
most infants.
Outcome
The long-term outcome of patients with CF with or
without meconium ileus is probably not different,
although gastrointestinal complications continue
throughout life. A meconium ileus equivalent (distal ileal
obstructive syndrome) may develop as a consequence of
noncompliance with oral enzyme replacement therapy or
bouts of dehydration. This is managed nonoperatively in
most patients with enemas or oral polyethylene glycol
purging solutions. Other diagnoses must also be consid-
ered, including simple adhesive intestinal obstruction.
Further, with the introduction of enteric-coated, high-
strength pancreatic enzyme replacement therapy, a fibros-
ing cholangiopathy has been described. Resection of the
inflammatory colon stricture may be necessary.
Intussusception
Intussusception is the telescoping of one portion of the
intestine into the other and is the most common cause
of intestinal obstruction in early childhood. In most pedi-
atric intussusceptions, the cause is unknown, the location
is at the ileocecal junction, and there is no identifiable
pathologic lead point. Invariably, there is marked swell-
ing of the lymphoid tissue within the region of the ileo-
cecal valve. It is unknown whether this represents the
cause or the effect of the intussusception. Evidence to
implicate a role for lymphoid swelling in the pathogene-
sis of intussusception is suggested by the association of
this condition with a history of recent episodes of viral
gastroenteritis, upper respiratory infections, and recently,
administration of rotavirus vaccine.
Etiology
The incidence of a pathologic lead point is up to 12% in
most pediatric series and increases directly with age. The
8/28/2007 11:37:51 AM
 2064 Section XIII Specialties in General Surgery
most common lead point for intussusception is Meckel’s
diverticulum; however, other causes must be considered,
including polyps, the appendix, intestinal neoplasm, sub-
mucosal hemorrhage associated with Henoch-Schönlein
purpura, foreign body, ectopic pancreatic or gastric
tissue, and intestinal duplication. Intussusception may
also occur within the small bowel in the absence of a
lead point in children who undergo abdominal surgery
for a variety of reasons. This diagnosis is entertained in
a child with crampy abdominal pain and emesis in the
early postoperative period.
Clinical Presentation
Intussusception classically produces severe, cramping
abdominal pain in an otherwise healthy child. The child
often draws the legs up during the pain episodes and is
usually quiet during the intervening periods. After some
time, the child becomes lethargic. Vomiting is almost
universal. Although frequent bowel movements may
occur with the onset of pain, the progression of the
obstruction results in bowel ischemia with passage of
dark blood clots mixed with mucus commonly referred
to as currant jelly stool. An abdominal mass may be
palpated.
Diagnosis
In about half of cases, the diagnosis of intussusception
can be suspected on plain abdominal radiographs. Sug-
gestive radiographic abnormalities include the presence
of a mass, sparse gas within the colon, or complete distal
small bowel obstruction. In cases in which there is a low
index of suspicion for intussusception based on clinical
findings, an abdominal ultrasound may be the initial
diagnostic test. The characteristic sonographic findings of
intussusception include the target sign of the intussus-
cepted layers of bowel on transverse view or the pseu-
dokidney sign when seen longitudinally.
Nonoperative Management
When the clinical index of suspicion for intussusception
is high, hydrostatic reduction by enema using contrast or
air is the diagnostic and therapeutic procedure of choice.
Contraindications to this study include the presence of
peritonitis or hemodynamic instability. Further, an intus-
susception that is located entirely within the small intes-
tine is unlikely to be reached by enema and more likely
to have an associated lead point. Hydrostatic reduction
using barium has been the mainstay of therapy; however,
more recently, the use of air enema has become more
widespread. Successful reduction is accomplished in
more than 80% of cases and confirmed by resolution of
the mass, along with reflux of air into the proximal
ileum. To avoid radiation exposure altogether, intussus-
ception reduction by saline enema under ultrasound sur-
veillance may be employed. Recurrence rates after
hydrostatic reduction are roughly 11% and usually occur
within the first 24 hours. Recurrence is usually managed
by another attempt at hydrostatic reduction. A third
recurrence is usually an indication for operative
management.
Ch071-X3675.indd 2064
Operative Management
The indications for operation in patients with intussus-
ception include the presence of peritonitis or a clinical
exam consistent with necrotic bowel. The presence of
complete small bowel obstruction, small bowel location,
failure of hydrostatic complete reduction, or history of
several recurrences also directs surgical intervention.
Laparoscopy may be useful as a first step to confirm the
presence of an incompletely reduced intussusception and
to facilitate reduction, thus avoiding a larger incision.26
The intussusceptum is delivered through a transverse
incision in the right side of the abdomen and reduced
by squeezing the mass retrograde from distal to proximal
until completely reduced. Warm lap pads may be placed
over the bowel and a period of observation may be war-
ranted in cases of questionable bowel viability. Adhesive
bands around the ileocecal junction are divided, and an
appendectomy is then performed. Invariably, the lym-
phoid tissue within the ileocecal valve region is thick-
ened and edematous and may be mistaken for a tumor
within the small bowel. Experience with this condition
may prevent an unnecessary bowel resection. The recur-
rence rates are very low after surgical reduction. Bowel
resection is required in cases in which the intussuscep-
tion cannot be reduced, the viability of the bowel is
uncertain, or a lead point is identified. An ileocolectomy
with primary reanastomosis is the usual procedure
performed.
Hirschsprung’s Disease
Hirschsprung’s disease occurs in 1 out of every 5000 live
births and is characterized pathologically by absent gan-
glion cells in the myenteric (Auerbach’s) and submucosal
(Meissner’s) plexus. This neurogenic abnormality is asso-
ciated with muscular spasm of the distal colon and inter-
nal anal sphincter resulting in a functional obstruction.
Hence, the abnormal bowel is the contracted, distal
segment, whereas the normal bowel is the proximal,
dilated portion. The area between the dilated and con-
tracted segments is referred to as the transition zone. In
this area, ganglion cells begin to appear, but in reduced
numbers. The aganglionosis always involves the distal
rectum and extends proximally for variable distances.
The rectosigmoid is affected in roughly 75% of cases,
splenic flexure or transverse colon in 17%, and the entire
colon with variable extension into the small bowel in 8%.
The risk for Hirschsprung’s disease is greater if there is
a positive family history and in patients with Down
syndrome.
Clinical Presentation
Most infants with Hirschsprung’s disease have symptoms
within the first 24 hours of life with progressive abdomi-
nal distention and bilious emesis. Failure to pass meco-
nium in the first 24 hours is a highly significant and
cardinal feature of this condition. In some infants, diar-
rhea may develop as a result of enterocolitis. The diag-
nosis of Hirschsprung’s disease may also be overlooked
for prolonged periods. In these cases, older children may
present with a history of poor feeding, chronic abdominal
8/28/2007 11:37:51 AM

Figure 71-13 Hirschsprung’s disease. A barium enema demon-
strating the zone of transition (arrows) from the dilated proxi-
mal normal colon to the reduced caliber of the distal aganglionic
colon.
distention, and a history of significant constipation.
Because constipation is a common problem among
normal children, referral for surgical biopsy to exclude
Hirschsprung’s disease occurs relatively frequently.
Enterocolitis is the most common cause of death in
patients with uncorrected Hirschsprung’s disease and
may manifest as diarrhea alternating with periods of
obstipation, abdominal distention, fever, hematochezia,
and peritonitis.
Diagnosis
The initial diagnostic step in a newborn with radio-
graphic evidence of a distal bowel obstruction is a barium
enema. Before this study, rectal examination and enemas
are avoided so that they do not interfere with the identi-
fication of a transition zone. In a normal barium enema
study, the rectum is wider than the sigmoid colon. In
patients with Hirschsprung’s disease, spasm of the distal
rectum usually results in a smaller caliber when com-
pared with the more proximal sigmoid colon. Identifica-
tion of a transition zone may be quite helpful (Fig. 71-13);
however, determination of the location of the transition
zone is considered to be relatively inaccurate. Failure to
completely evacuate the instilled contrast material after
24 hours would also be consistent with Hirschsprung’s
disease and may provide additional diagnostic yield. An
important goal of this study is to exclude other causes
of constipation in the newborn, such as meconium plug,
small left colon syndrome, and atresia.
Anorectal manometry may also suggest the diagnosis
of Hirschsprung’s disease. The classic finding is failure
of the internal sphincter to relax when the rectum is
distended with a balloon. The advantage of this method
is that it can be done in an outpatient setting, without
Ch071-X3675.indd 2065
Chapter 71 Pediatric Surgery 2065
the need for general anesthesia. This is more often useful
in an older patient and is seldom used in neonates.
A rectal biopsy is the gold standard for the diagnosis
of Hirschsprung’s disease. In the newborn period, this is
done at the bedside with minimal morbidity using a
special suction rectal biopsy instrument. It is important
to obtain the sample at least 2 cm above the dentate line
to avoid sampling the normal transition from ganglion-
ated bowel to the paucity or absence of ganglia in the
region of the internal sphincter. In older children, because
the rectal mucosa is thicker, a full-thickness biopsy is
obtained under general anesthesia. Absent ganglia, hyper-
trophied nerve trunks, and robust immunostaining for
acetylcholinesterase are the pathologic criteria to make
the diagnosis.
Surgical Management
Multiple surgical options exist for the management of
Hirschsprung’s disease. Traditionally, a leveling proce-
dure is done, followed by proximal diversion. This con-
sists of a formal laparotomy, which is usually performed
through a small incision in the left lower quadrant of the
abdomen. The location of the transition zone is then
identified and confirmed by multiple seromuscular biop-
sies. A diverting colostomy is then performed in the
region of normal ganglionated bowel. A definitive pro-
cedure is then performed later.
The definitive management of Hirschsprung’s disease
involves variations among three main procedures. In the
Swenson procedure, the aganglionic bowel is removed
down to the level of the internal sphincters and a colo-
anal anastomosis is performed on the perineum. In the
Duhamel procedure, the aganglionic rectal stump is left
in place, and the ganglionated, normal colon is pulled
behind this stump. A GIA stapler is then inserted through
the anus with one arm within the normal, ganglionated
bowel posteriorly and the other in the aganglionic rectum
anteriorly. Firing of the stapler therefore results in forma-
tion of a neorectum that empties normally because of the
posterior patch of ganglionated bowel. Finally, the Soave
technique involves an endorectal mucosal dissection
within the aganglionic distal rectum. The normally gan-
glionated colon is then pulled through the remnant mus-
cular cuff and a coloanal anastomosis performed. More
recently, these procedures have been performed in the
newborn period as a primary procedure and without an
initial ostomy. Further, the same procedure has been
described in infants completely through a transanal
approach with or without laparoscopic guidance.27 The
overall survival of patients with Hirschsprung’s disease is
excellent; however, long-term stooling problems are not
infrequent. Constipation is the most frequent postopera-
tive problem followed by soiling, incontinence, and
enterocolitis.
Imperforate Anus
The spectrum of anorectal malformations ranges from
simple anal stenosis to the persistence of a cloaca; inci-
dence ranges from 1 in 4000 to 5000 live births and is
slightly more common in boys. The most common defect
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 2066 Section XIII Specialties in General Surgery
Table 71-3 Classification of Congenital
Anomalies of the Anorectum
Female
High Anorectal agenesis with or without
rectovaginal fistula
Rectal atresia
Intermediate Anorectal agenesis with or without
rectovaginal fistula
Anal agenesis
Low Anovestibular or anocutaneous fistula
(anteriorly displaced anus)
Anal stenosis
Cloaca
Male
High Anorectal agenesis with or without
rectoprostatic urethral fistula
Rectal atresia
Intermediate Anorectal agenesis with or without
rectobulbar urethral fistula
Anal agenesis
Low Anocutaneous fistula (anteriorly
displaced anus)
Anal stenosis
is an imperforate anus with a fistula between the distal
colon and the urethra in boys or the vestibule of the
vagina in girls.
Anorectal Embryology
By 6 weeks of gestation, the urorectal septum moves
caudally to divide the cloaca into the anterior urogenital
sinus and posterior anorectal canal. Failure of this septum
to form results in a fistula between the bowel and urinary
tract (in boys) or the vagina (in girls). Complete or partial
failure of the anal membrane to resorb results in an anal
membrane or stenosis. The perineum also contributes to
development of the external anal opening and genitalia
by formation of cloacal folds, which extend from the
anterior genital tubercle to the anus. The perineal body
is formed by fusion of the cloacal folds between the anal
and urogenital membranes. Breakdown of the cloacal
membrane anywhere along its course results in the exter-
nal anal opening being anterior to the external sphincter
(i.e., anteriorly displaced anus).
Classification of Anorectal Anomalies
An anatomic classification of anorectal anomalies
is based on the level at which the blind-ending rectal
pouch ends in relationship to the levator ani musculature
(Table 71-3). Historically, the level of the end of the rectal
pouch was determined by obtaining a lateral pelvic radio-
graph (i.e., invertogram) after the infant is held upside-
down for several minutes to allow air to pass into the
rectal pouch. This examination is very subjective and no
longer used. Inspection of the perineum alone deter-
mines the pouch level in 80% of boys and 90% of girls.
Clinically, if an anocutaneus fistula is seen anywhere on
Ch071-X3675.indd 2066
the perineal skin of a boy or external to the hymen of a
girl, a low lesion can be assumed, which allows a primary
perineal repair procedure to be performed, without the
need for a stoma. Most other lesions are high or inter-
mediate, and they require proximal diversion by a sigmoid
colostomy. This is followed by a definitive repair proce-
dure at a later date. If required, the level of the rectal
pouch can be detailed more definitively by ultrasonog-
raphy or MRI.
Rectal atresia refers to an unusual lesion in which the
lumen of the rectum is either completely or partially
interrupted, with the upper rectum being dilated and the
lower rectum consisting of a small anal canal. A persistent
cloaca is defined as a defect in which the rectum, vagina,
and urethra all meet and fuse to form a single, common
channel. In girls, the type of defect may be determined
by the number of orifices at the perineum. A single orifice
would be consistent with a cloaca. If two orifices are
seen (i.e., urethra and vagina), the defect represents
either a high imperforate anus or, less commonly, a per-
sistent urogenital sinus comprising one orifice and a
normal anus as the other orifice.
Associated Anomalies
Congenital anorectal anomalies often coexist with other
lesions, and the VATER or VACTERL association must be
considered. Bony abnormalities of the sacrum and spine
occur in about one third of patients and consist of absent,
accessory, or hemivertebrae or an asymmetric or short
sacrum. Absence of two or more vertebrae is associated
with a poor prognosis for bowel and bladder continence.
Occult dysraphism of the spinal cord also may be present
and consists of tethered cord, lipomeningocele, or fat
within the filum terminale.
Preoperative Evaluation
Clinical evaluation includes plain radiographs of the
spine as well as an ultrasound of the spinal cord. Geni-
tourinary abnormalities other than the rectourinary fistula
occur in 26% to 59% of patients. Vesicoureteral reflux and
hydronephrosis are the most common, but other findings
such as horseshoe, dysplastic, or absent kidney, as well
as hypospadias or cryptorchidism, also must be consid-
ered. In general, the higher the anorectal malformation,
the greater the frequency of associated urologic abnor-
malities. In patients with a persistent cloaca or rectovesi-
cal fistula, the likelihood of a genitourinary abnormality
is about 90%. In contrast, the frequency is only 10% in
children with low defects (i.e., perineal fistula). Radio-
graphic evaluation of the urinary tract includes renal
ultrasonography and voiding cystourethrography; a rec-
tourinary fistula (if present) likely will be demonstrated
by the latter procedure.
In addition to the other tests described previously, a
plain chest radiograph and careful clinical evaluation of
the heart are conducted. If a cardiac defect is suspected,
echocardiography is performed before any surgical pro-
cedure. Before feeding, a nasogastric tube is placed and
its presence within the stomach confirmed to exclude
esophageal atresia.
8/28/2007 11:37:52 AM

Low Lesions
The newborn infant with a low lesion can have a primary,
single-stage repair procedure without need for a colos-
tomy. Three basic approaches may be used. For anal
stenosis in which the anal opening is in a normal loca-
tion, serial dilation alone is usually curative. Dilations are
performed daily by the caretaker, and the size of the
dilator is increased progressively (beginning with 8-9
French and increased slowly to 14 to 16 French). If the
anal opening is anterior to the external sphincter (i.e.,
anteriorly displaced anus) with a small distance between
the opening and the center of the external sphincter, and
the perineal body is intact, a cutback anoplasty is per-
formed. This consists of an incision extending from the
ectopic anal orifice to the central part of the anal sphinc-
ter, thus enlarging the anal opening. Alternatively, if there
is a large distance between the anal opening and the
central portion of the external anal sphincter, a transposi-
tion anoplasty is performed in which the aberrant anal
opening is transposed to the normal position within the
center of the sphincter muscles, and the perineal body
is reconstructed.
High or Intermediate Lesions
Infants with intermediate or high lesions traditionally
require a colostomy as the first part of a three-stage
reconstruction. The colon is completely divided in the
sigmoid region, with the proximal bowel as the colos-
tomy and the distal bowel as a mucous fistula. Complete
division of the bowel minimizes fecal contamination into
the area of a rectourinary fistula, and it may lessen the
risk for urosepsis. Furthermore, the distal bowel can be
evaluated radiographically to determine the location of
the rectourinary fistula. The second-stage procedure
usually is performed 3 to 6 months later and consists of
surgically dividing the rectourinary or rectovaginal fistula
with pull-through of the terminal rectal pouch into the
normal anal position. A posterior sagittal approach as
championed by Peña is the procedure most frequently
performed.28 This consists of determination of the loca-
tion of the central position of the anal sphincter by elec-
trical stimulation of the perineum. An incision is then
made in the midline extending from the coccyx to the
anterior perineum and through the sphincter and levator
musculature until the rectum is identified. The fistula
from the rectum to the vagina or urinary tract is divided.
The rectum is then mobilized and the perineal muscula-
ture reconstructed. The third and final stage is closure of
the colostomy, which is performed a few months later.
Anal dilations are begun 2 weeks after the pull-through
procedure and continue for several months after the
colostomy closure.
More recently, a single-stage procedure using a trans-
abdominal laparoscopic approach has been described for
treatment of intermediate and high imperforate anus
anomalies.29 This technique offers the theoretic advan-
tages of placement of the neorectum within the central
position of the sphincter and levator muscle complex
under direct vision and avoids the need to cut across
Ch071-X3675.indd 2067
Chapter 71 Pediatric Surgery 2067
these structures. The long-term outcome of this new
approach when compared with the standard posterior
sagittal method is presently unknown.
Most of the morbidity in patients with anorectal
malformations is related to the presence of associated
anomalies. Fecal continence is the major goal regarding
correction of the defect. Prognostic factors for continence
include the level of the pouch and whether the sacrum
is normal. Globally, 75% of patients have voluntary bowel
movements. Half of this group still soil their underwear
occasionally while the other half are considered totally
continent.30 Constipation is the most common sequela. A
bowel management program consisting of daily enemas
is an important postoperative plan to reduce the fre-
quency of soilage and improve the quality of life for these
patients.
ABDOMINAL WALL
Abdominal Wall Defects
Defects of the anterior abdominal wall are a relatively
frequent anomaly managed by pediatric surgeons. During
normal development of the human embryo, the midgut
herniates outward through the umbilical ring and contin-
ues to grow. By the 11th week of gestation, the midgut
returns back into the abdominal cavity and undergoes
normal rotation and fixation, along with closure of the
umbilical ring. If the intestine fails to return, the infant is
born with abdominal contents protruding directly through
the umbilical ring, termed an omphalocele (Fig. 71-14A).
Most commonly, a sac is still covering the bowel, thus
protecting it from the surrounding amniotic fluid. Occa-
sionally, the sac may be torn at some point in utero,
creating confusion with the other major type of abdomi-
nal wall defect termed gastroschisis (see Fig. 71-14B). In
contrast with omphalocele, the defect seen with gastros-
chisis is always on the right side of the umbilical ring
with an intact umbilical cord, and there is never a sac
covering the abdominal contents. Major morbidity and
mortality with either anomaly are not as high with surgi-
cal repair of the abdominal defect as with the associated
abnormalities. In the absence of other major anomalies,
long-term survival is excellent.31
Omphalocele
The abdominal contents with an omphalocele are covered
with a membrane composed of the peritoneum on the
inside and amnion on the outside. The size of the defect
is variable, ranging from a small opening through which
a small portion of the intestine is herniated to a large one
in which the entire bowel and liver are included. In
contrast with gastroschisis, karyotype abnormalities are
present in roughly 30% of infants, including trisomies 13,
18, and 21. More than half of infants with omphalocele
have other major or minor malformations, with cardiac
being the most common, followed by musculoskeletal,
gastrointestinal, and genitourinary. There is also a close
8/28/2007 11:37:52 AM
 2068 Section XIII Specialties in General Surgery

B
Figure 71-14 The two major abdominal wall defects. An omphalocele (A) originates in the center of the umbilical
ring and contains a sac covering the bowel. There is a high incidence of other associated anomalies in the infant.
In contrast, a gastroschisis (B) defect originates on the right side of the umbilical ring, there is no sac covering
the viscera, and associated anomalies are relatively infrequent.

association with Beckwith-Wiedemann syndrome (ompha-
locele, hyperinsulinemia, and macroglossia).
Preoperative Evaluation and Management
The immediate treatment of an omphalocele consists of
nasogastric or orogastric tube decompression for preven-
tion of visceral distention due to swallowed air. An IV
line is secured for administration of fluids and broad-
spectrum antibiotics. The sac is covered with a sterile,
moist dressing and the infant transported to a tertiary care
pediatric surgery facility. Before operative repair, the
infant is evaluated for potential chromosomal and devel-
opmental anomalies by a careful physical examination,
plain chest radiograph, echocardiography if the physical
examination suggests underlying congenital heart disease,
and renal ultrasonography. Because the viscera are
covered by a sac, operative repair of the defect may be
delayed to allow thorough evaluation of the infant.
Surgical Management
Several options exist for the surgical management of an
omphalocele and are largely dictated by the size of the
defect. In most cases, the contents within the sac are
reduced back into the abdomen, the sac is excised with
care to individually ligate the umbilical vessels, and the
fascia and skin are closed. Fascial closure may be facili-
tated by stretching the anterior abdominal wall as well
as milking out the contents of the bowel proximally and
distally.
In giant omphaloceles, the degree of visceroabdominal
disproportion prevents primary closure, and the opera-
tive management becomes more challenging. Construc-
tion of a Silastic silo allows for gradual reduction of the
viscera into the abdominal cavity over a several-day
period. Monitoring of intra-abdominal pressure during
reduction may prevent the development of an abdominal
compartment syndrome. After the abdominal contents are
returned to the abdomen, the infant is taken back to the
operating room for formal fascia and skin closure. Occa-
sionally, closure of the fascia may be impossible. In these
cases, the skin is closed, and a large hernia is accepted.
This is repaired after 1 or 2 years. When the skin cannot
be closed over the defect, several options exist, including
the topical application of an antimicrobial solution to the
outside of the sac, such as silver nitrate or silver sulfadia-
zine. Over time, this will result in granulation tissue and
subsequent epithelialization of the sac. A repair of the
large hernia is then performed a few years after this.

Ch071-X3675.indd 2068 8/28/2007 11:37:52 AM


Peritoneal cavity
Internal ring
External ring Scrotal hernia or
communicating
Normal hydrocele
Hydrocele
of cord
Figure 71-15 Anatomic variants of inguinal hernia and hydrocele. (From Cox JA: Inguinal hernia of childhood.
Surg Clin North Am 65:1331-1342, 1985).
Gastroschisis
In contrast to patients with an omphalocele, the risk for
associated anomalies with gastroschisis is low. One major
exception to this general rule is the association of gas-
troschisis with intestinal atresia, which may be present in
up to 15%. Atresias may involve the small and large
intestine. The cause of gastroschisis is presently unknown,
but a prevailing theory is that it results from an abdominal
wall defect associated with normal involution of the
second umbilical vein. In addition, babies with gastros-
chisis are more often small for gestational age and born
to mothers with a history of cigarette, alcohol, and rec-
reational drug use and intake of aspirin, ibuprofen, and
pseudoephedrine during the first trimester; there is also
an 11-fold increase in risk in mothers younger than 20
years.
The surgical management of gastroschisis is similar to
omphalocele. Considerations for third-space fluid losses
from the exposed intestine and risk for infection dictate
more expedient coverage. The presence of atresia in a
patient with gastroschisis may be managed in a number
of ways. The bowel can simply be placed into the
abdomen with a planned reoperation after several weeks.
Another approach would be to perform a proximal divert-
ing stoma. Finally, a primary anastomosis may be
attempted. This is rarely advised because of the possibil-
ity of other atresias as well as the overall condition of
the bowel.
In patients with gastroschisis, the intestine is often
thickened, edematous, matted together, and foreshort-
ened. It is unclear whether this represents damage from
Ch071-X3675.indd 2069
Chapter 71 Pediatric Surgery 2069
Hernia
Hydrocele
the amniotic fluid or ischemia from the small, constricting
abdominal wall defect. The short gut syndrome may be
a consequence of the attenuated intestinal length. Even
with adequate length, the remnant bowel may be damaged
to the point that motility, digestion, and absorption are
markedly impaired. This prenatal intestinal injury accounts
for most of the postoperative morbidity and mortality.
Virtually all infants have a prolonged postoperative ileus.
Parenteral nutrition is lifesaving but is also associated
with the development of cholestasis, cirrhosis, portal
hypertension, and ultimate liver failure.
Inguinal Hernia
Repair of an inguinal hernia (IH) represents one of the
most common surgical procedures performed in the
pediatric age group. Virtually all IHs in children are indi-
rect and congenital in origin. The variable persistence of
the embryonic processus vaginalis offers a spectrum of
abnormalities, including a scrotal hernia, communicating
hydrocele, hydrocele of the cord, or simple hydrocele
(Fig. 71-15).
Incidence
The incidence of IH has been reported to range between
0.8% and 4.4%, which roughly translates into 10 to 20
per 1000 live births. In preterm infants, the incidence may
be as high as 30%. About one third of children with IH
are younger than 6 months, and males are affected about
six times more often than females. The right side is
involved in 60% and the left in 30% of patients; bilateral
8/28/2007 11:37:53 AM
 2070 Section XIII Specialties in General Surgery
hernias are seen in 10%. The higher incidence on the
right side compared with the left is probably related to
the later descent and obliteration of the processus vagi-
nalis of the right testis.
Diagnosis
Most IHs present as a bulge in the region of the external
ring extending downward for varying distances to the
scrotum or labia. Often, the hernia is detected by a
pediatrician during a routine physical examination or
observed by the parents. Inguinal pain may also be a
presenting complaint. Incarceration and possible strangu-
lation are the most feared consequences of IH and occur
more frequently in premature infants. Because of the risk
for these complications, all IHs in children need to be
repaired.
Hydroceles represent fluid around the testicle or cord.
A hydrocele that fills with fluid from the peritoneum is
termed communicating. This is distinguished from a
noncommunicating hydrocele by the history of variation
in size throughout the day and palpation of a thickened
cord above the testicle on the affected side. A commu-
nicating hydrocele is basically a small IH in which fluid,
but not peritoneal structures, traverses the processus
vaginalis. As such, all communicating hydroceles are
repaired in the same manner as an indirect IH. In con-
trast, noncommunicating hydroceles are common in
infants and can be observed for several months. The
indications for repair of a noncommunicating hydrocele
include failure to resolve and increase in size to one that
is large and tense. The acute development of a hydrocele
might be associated with the onset of epididymitis, tes-
ticular tumor, trauma, and torsion of a testicular append-
age. An ultrasound of the scrotum may provide important
diagnostic information in cases of an acute hydrocele in
which examination of the testicle is difficult.
The timing of IH repair in premature infants is con-
troversial. Early repair may be associated with a higher
risk for injury to the cord structures, greater recurrence
rate, and anesthetic-related apnea. These factors must be
weighed against the higher risk for incarceration and
strangulation, the potential for losing the patient during
follow-up, and the development of a larger IH with loss
of domain in the abdominal cavity. Taking these factors
into account, most pediatric surgeons perform hernior-
rhaphy before the neonate is discharged home from the
nursery.32 If the infant has already been discharged home,
most pediatric surgeons wait until the infant is older than
50 weeks postconception (gestational age + postnatal
age). After this age, the risk for postoperative apnea is
diminished.
The timing of repair of incarcerated IH is another
important point and depends on the sex of the patient
and contents within the hernia sac. In girls, the most
common structure present in an IH that cannot be reduced
is an ovary. The ovary within the sac is at significant risk
for torsion and strangulation. Although this is not a true
surgical emergency, IH repair needs to be done relatively
soon (within a few days).
Ch071-X3675.indd 2070
In patients with incarcerated IH containing bowel,
attempts must be made to reduce the hernia, unless there
is clinical evidence of peritonitis. This may require IV
sedation and careful monitoring. If the reduction is suc-
cessful, the child is admitted and observed for 24 to 48
hours. The IH repair is done after the period of observa-
tion to allow for tissue edema to subside. On the other
hand, if the IH cannot be reduced, the child is promptly
taken to the operating room for inguinal exploration. If
an intestinal resection is required, it can usually be done
through the opened hernia sac before IH repair.
There is much controversy over the management of
the opposite groin of the child with a unilateral IH. The
major advantage of contralateral exploration is that it
determines the presence of a patent processus vaginalis.
Although a patent processus is not the same as an IH,
an indirect IH cannot occur without it. Because there is
a higher incidence of a contralateral patent processus
within the first year of life, many surgeons restrict explo-
ration of the other side to children younger than 1 year.
In addition, many surgeons believe that contralateral
exploration must be performed in all girls presenting with
a clinically obvious unilateral IH because the likelihood
of injury to reproductive structures is rare. Laparoscopic
evaluation of the contralateral groin through the opened
sac at the time of repair may be a safe and accurate
method of identifying the presence of a patent processus
vaginalis.33
The technical details of IH repair in infants have been
well described and consist of high ligation of the hernia
sac at the level of the internal ring. A repair of the floor
of the inguinal canal is usually not necessary. In most
cases, this is an outpatient procedure with minimal mor-
bidity. Recurrence, injury to the vas deferens, wound
infection, and postoperative hydrocele are recognized
complications associated with IH repair but occur with a
frequency of less than 1%.
Umbilical Hernia
An umbilical hernia (UH) occurs as a result of persistence
of the umbilical ring. Complete closure of this ring can
be anticipated by the age of 4 to 6 years in up to 80%
of cases. In contrast with IH, a UH is rarely associated
with significant complications. As such, most pediatric
surgeons defer UH repair until the child is old enough
to begin kindergarten. Exceptions to this general rule are
a large UH defect (>2 cm) because the likelihood for
spontaneous resolution is lower. History of incarceration,
a large skin proboscis, and a ventriculoperitoneal shunt
are other relative indications for repair. The technique
for UH repair generally involves an infraumbilical semi-
circular incision, separation of the hernia sac from the
overlying umbilical skin, repair of the fascial defect,
pexing the base of the umbilical skin to the fascia, and
skin closure.
Epigastric Hernia
Epigastric hernias (EH) represent the third most common
hernia in children. These are found anywhere along the
8/28/2007 11:37:54 AM

midline of the abdomen between the umbilicus and
xiphoid process. Not to be confused with a broad defect
of a diastasis rectus, the fascial defect of an EH is quite
small but allows herniation of properitoneal fat through
the defect. Although this does not pose significant risk to
the patient, strangulation of the fat often results in pain,
redness, and swelling. This scenario often directs urgent
operative exploration to exclude incarceration of other,
more important structures. Because of this and the likeli-
hood for continued enlargement, most pediatric surgeons
recommend elective repair. This is accomplished through
a small transverse incision overlying the palpable mass.
The herniated fat is excised and the fascia repaired.
Umbilical Abnormalities
The most significant abnormalities of the umbilicus are
generally associated with an abnormal communication
with the gastrointestinal tract or urinary bladder. In the
newborn period, failure of separation of the umbilical
cord, prolapsed mucosa or granulation tissue at the base
of the umbilicus, or drainage of urine or stool from the
umbilicus suggest these abnormalities.
Communication of the gastrointestinal tract to the
umbilicus occurs through a patent omphalomesenteric
duct. When the remnant of this duct is not connected to
the abdominal wall, it is referred to as Meckel’s diverticu-
lum. Stool may be identified in the base of the umbilicus
when the communication is open. The connection
between the ileum and umbilicus may also be nonpatent,
in which the remnant band may serve as a nidus for an
intestinal volvulus or obstruction. If the connection is
patent and stool is noted, umbilical exploration is war-
ranted, and no further imaging is needed. The omphalo-
mesenteric duct remnant is excised down to the
antimesenteric side of the ileum, where it is divided.
Communication of the urinary bladder to the umbili-
cus occurs through a patent urachus. A urachal connec-
tion extends from the dome of the bladder in a
retroperitoneal plane along the midline of anterior
abdominal wall, where it connects to the base of the
umbilicus. As such, the diagnosis may be easily made by
ultrasonography of the infraumbilical abdominal wall.
This test is warranted if there is clear umbilical drainage
to suggest urine or prolapsed mucosa or granulation
tissue after the umbilical stalk has separated. Treatment
involves an umbilical exploration with dissection of the
urachus down to the top of the bladder, where it is
divided and removed. Occasionally, there is only a sinus,
which extends for variable distances from the umbilicus
to the bladder, but without a frank communication.
Because of the propensity for infection, abnormal uro-
thelium contained in the sinus, and mucoid drainage
from the umbilicus, it needs to be excised in the same
manner as for a patent urachus. Finally, a cyst may be
contained within a urachal remnant that does not com-
municate with either the umbilicus or the bladder. These
cysts most commonly present as urachal abscess. After
percutaneous or open drainage of the abscess for initial
treatment, the entire urachal remnant is excised.
Ch071-X3675.indd 2071
Chapter 71 Pediatric Surgery 2071
GENITOURINARY
Cryptorchidism
Cryptorchidism (undescended testes [UDT]) is the most
common disorder of sexual differentiation and occurs in
about 2% of human male births. The consequences of
cryptorchidism include degeneration of the testes with
impaired fertility and an increased risk for germ cell tes-
ticular tumors. Despite the significance of this problem,
the cause of UDT has not been clarified in most patients.
It is presently unclear whether the peculiar pathologic
change in the cryptorchid testes occurs as a result of a
primary defect in testicular development or that a normal
testis fails to descend with the high temperature inducing
a secondary change.
A true UDT must be distinguished from a retractile
testis. Retractile testes can usually be manipulated from
the inguinal canal to a scrotal position. These testes can
be expected to spontaneously descend into a normal
scrotal position over time. Thus, no specific therapy is
needed beyond careful long-term follow-up.
When a unilateral gonad is palpated in the inguinal
canal, but cannot be manipulated into a scrotal position,
an orchidopexy is performed. In most pediatric centers,
this is done when patients have reached about 6 months
of age. This early intervention may permit postnatal germ
cell development to proceed normally. The orchidopexy
is done through an inguinal incision. In about 90% of
cases of UDT, there is an associated inguinal hernia,
which is repaired at the same time. The testicle and its
blood supply are mobilized into the scrotal position. A
pouch is created within the dartos fascia through a sepa-
rate scrotal incision, and the testicle is sutured to this
fascia.
If the testicle is unable to be brought into a scrotal
position after mobilization alone, a Fowler-Stephens tech-
nique may be applied. In this procedure, the tethering
spermatic blood vessels are divided. At a second stage,
the testicle may be placed into the scrotum after collateral
blood supply to the testicle has developed. Alternatively,
the testicular artery and vein can be divided and reanas-
tomosed using microvascular techniques to permit place-
ment of the gonad into the scrotum.
In the event that the gonad cannot be palpated on
one side, ultrasonography or MRI may be used to locate
the testicle. This localization is typically followed by
laparoscopy to either facilitate the orchidopexy or to
remove the atrophic gonad. When congenital UDT pres-
ents beyond puberty, removal of the gonad is usually
indicated to prevent the development of malignancy.
Intersex Abnormalities
In all mammalian embryos, both male and female pri-
mordial reproductive ducts coexist for a short period of
time. In the absence of testes, the uterus, fallopian tubes,
and upper third of the vagina form autonomously. Simi-
larly, the external genitalia develop autonomously into a
clitoris, labia majora, and labia minora. The development
8/28/2007 11:37:54 AM
 2072 Section XIII Specialties in General Surgery
of male external genitalia requires the reduction of tes-
tosterone by the enzyme 5α-reductase into dihydrotes-
tosterone. Although autonomous female development
can occur without ovaries, the development of a male
phenotype requires a functional Y chromosome and
androgenic steroids.
A baby born with ambiguous genitalia mandates an
expeditious and thoughtful evaluation to establish an
optimal gender assignment. This dictates a multidisci-
plinary approach with input from experienced physicians
in endocrinology, neonatology, genetics, and pediatric
surgery. Inaccurate and hasty gender assignment can be
psychologically devastating to the parents and child with
lifetime consequences. Significant advances in genitouri-
nary reconstruction have provided multiple acceptable
options to harmonize the appearance and function of the
external genitalia with the sex of rearing. Three major
categories of developmental abnormalities account for
ambiguous genitalia in newborns and are discussed
next.
Female Pseudohermaphroditism
In the first category, genetic females become masculin-
ized because of an overproduction of androgenic ste-
roids. The most common cause of this is an enzyme
defect involved in the conversion of progesterones to
glucocorticoids and mineralocorticoids. Mutations in the
CYP21 gene (previously referred to as the gene encoding
21-hydroxylase) are noted in 90% of cases. This genetic
defect results in the syndrome known as congenital
adrenal hyperplasia, also known as adrenogenital syn-
drome, also referred to a female pseudohermaphroditism.
Although this syndrome may be seen in both males and
females, ambiguous genitalia are only observed in female
infants. Because of timing of internal organ formation
relative to adrenal function in the developing embryo,
the external genitalia of female infants are most signifi-
cantly masculinized, whereas the internal structures
(uterus, fallopian tubes, and ovaries) are normal.
Male Pseudohermaphroditism
The second group of developmental abnormalities results
from deficient production, response to, or conversion of
androgen in genetic males. Defective synthesis of at least
five different enzymes needed for the successful conver-
sion of cholesterol to testosterone has been characterized
as leading to the development of ambiguous genitalia in
males. Androgen insensitivity results from point muta-
tions in both coding and noncoding regions of the andro-
gen receptor gene leading to a variable phenotype. The
complete form of androgen insensitivity is known as the
testicular feminization syndrome. This condition most
commonly presents at the time of inguinal hernia repair
in a presumed female patient. The presence of bilateral
inguinal hernia sacs containing palpable, reducible
gonads suggests this diagnosis. Intraoperatively, testicles
are discovered, and vaginoscopy reveals a shortened,
blind-ending vagina and no cervix. In these cases of
complete androgen insensitivity, the child continues to
be raised as a female and will ultimately require testicular
Ch071-X3675.indd 2072
removal to avoid the potential for malignancy. Although
these children do not have a uterus, exogenous estrogen
is typically required at the age of puberty to facilitate
secondary sexual development. Finally, defective produc-
tion of the 5α-reductase type 2 gene prevents the conver-
sion of testosterone to dihydrotestosterone in genital
tissues and permits feminization of the developing genetic
male.
Defective Gonadal Differentiation
The third and final set of abnormalities involves gene
mutations causing absent, incomplete, or asymmetric
gonad differentiation. In these conditions, gonadal dys-
genesis may involve both gonads (pure) or one gonad
on one side combined with a streak remnant on the other
(mixed). A true hermaphrodite is represented typically
with a normal testis on one side and a gonad containing
both testicular and ovarian tissue (ovotestes) on the other
side. Various other gonadal combinations have been
described.
CONGENITAL DIAPHRAGMATIC HERNIA
Congenital diaphragmatic hernia (CDH) represents one
of the most enigmatic diseases encountered in pediatric
surgery. The reported incidence of CDH is in the range
of 1 in 2000 to 5000 live births. Most CDH defects are
on the left side (80%); however, up to 20% may occur
on the right side. A CDH may also be bilateral, but this
is distinctly rare. Despite multiple innovative treatment
strategies, including in utero diaphragm repair, fetal tra-
cheal occlusion, high-frequency oscillation or partial
liquid ventilation, ECLS, exogenous surfactant, and
inhaled nitric oxide, survival rates for this condition have
not been significantly impacted. The exact survival rate
for CDH is difficult to determine but in the range of 70%
to 90%.34 Calculation of true survival is complicated by
the fact that many infants with CDH are stillborn, and
many reports tend to exclude infants with complex asso-
ciated anomalies from survival calculations.35
Pathogenesis
The cause for CDH is unknown, but it is thought to result
from failure of normal closure of the pleuroperitoneal
canal in the developing embryo. As a result, abdominal
contents herniate through the resultant defect in the
posterolateral diaphragm and compress the ipsilateral
developing lung. The posterolateral location of this hernia
is known as Bochdalek’s hernia and is distinguished from
the congenital hernia of the anteromedial, retrosternal
diaphragm, which is known as Morgagni’s hernia. Com-
pression of the lung results in pulmonary hypoplasia
involving both lungs, with the ipsilateral lung being the
most affected. In addition to the abnormal airway devel-
opment, the pulmonary vasculature is distinctly abnormal
in that the medial muscular thickness of the arterioles
is excessive and extremely sensitive to the multiple
local and systemic factors known to trigger vasospasm.
Thus, the two main factors that affect morbidity and
8/28/2007 11:37:54 AM
 Chapter 71 Pediatric Surgery 2073

mortality are pulmonary hypoplasia and pulmonary

hypertension.

Clinical Presentation
The most frequent clinical presentation of CDH is respira-
tory distress due to severe hypoxemia. The infant appears
dyspneic, tachypneic, and cyanotic, with severe retrac-
tions. The anteroposterior diameter of the chest may be
large, and the abdomen may be scaphoid. There are three
general presentations of infants with CDH. In the first
scenario, signs of severe respiratory distress are present
immediately at the time of birth. As such, if the diagnosis
is known prenatally, delivery within an institution capable
of providing ECLS, high-frequency ventilation, and
sophisticated neonatal care is crucial. In these infants,
pulmonary hypoplasia may be severe enough to be
incompatible with life. The infant may also have a revers-
ible cause for immediate hypoxia such as hypovolemia
or severe pulmonary vasospasm. Unfortunately, there are
no known criteria for distinguishing infants with severe
lung hypoplasia from those with reversible conditions.
As such, many infants with irreversible lung hypoplasia
are placed on ECLS for prolonged periods before it Figure 71-16 Congenital diaphragmatic hernia. The tip of the
becomes apparent that their underlying lung condition is nasogastric tube and obvious loops of gas-filled bowel are located
incurable. in the left hemithorax.
In the second and most common presentation, the
infant does well for several hours after delivery (the so-
called honeymoon period) and then begins to deteriorate
failed to demonstrate any survival advantage.36 Subse-
from a respiratory standpoint. Patients in this category
quent to this was the realization that occlusion of the
may benefit from therapy to reduce pulmonary hyperten-
fetal trachea might result in accumulation of lung fluid
sion and hypoxemia. Theoretically, these patients are
with stimulation of lung growth. Although several tech-
ideal candidates for ECLS because their lung develop-
niques for occlusion of the trachea have been described,
ment has progressed enough to sustain life. Unfortu-
including the use of balloons, sponges, or external clip
nately, this is not always the case because many infants
application, the overall result is larger, but persistently
in this group do not survive, even with ECLS support.
abnormal lungs and survival are not significantly
The third and final clinical presentation of CDH is
improved.37 Currently, there appears to be no rationale
beyond the first 24 hours of life, which occurs in about
for fetal intervention in the diagnosis of CDH.
10% to 20% of cases. Many of these children present with
The postnatal management of CDH is complex, but all
feeding difficulties, chronic respiratory disease, pneumo-
efforts need to be directed toward stabilization of the
nia, or intestinal obstruction. This group of patients enjoys
cardiorespiratory system, while minimizing iatrogenic
the best prognosis.
injury from therapeutic interventions. Endotracheal intu-
bation is critical to optimize ventilation. Placement of a
Diagnosis nasogastric tube is also important to prevent gastric dis-
The diagnosis of CDH is frequently made at the time of tention, which may worsen the lung compression, medi-
a prenatal ultrasound during an otherwise unremarkable astinal shift, and ability to ventilate. Acute deterioration
pregnancy. The postnatal diagnosis is relatively straight- of an infant with CDH may be due to a number of factors,
forward; a plain chest radiograph demonstrates the gastric including inadvertent extubation. However, it is impor-
air bubble or loops of bowel within the chest (Fig. tant to remember that a pneumothorax may develop
71-16). There may also be a mediastinal shift away from during aggressive attempts at ventilation. As such, it is
the side of the hernia or polyhydramnios from the important to remember that the pneumothorax in patients
obstructed stomach. Rarely is an upper gastrointestinal with CDH always occurs on the side contralateral to the
contrast study necessary. side of the CDH. Needle decompression of the contralat-
eral chest during an acute deterioration event may be
Preoperative Management lifesaving and necessary before a chest radiograph can
be obtained.
The management of CDH that has been detected in utero
Although used traditionally, pharmacologic pulmonary
has directed open fetal surgery as a strategy to remove
vasodilators (tolazoline), surfactant, high-frequency ven-
the compression of the abdominal viscera and allow for
tilation, and inhaled nitric oxide have all demonstrated
improved lung development. Unfortunately, this inter-
inconsistent success. One of the more important recent
vention is high risk to both the mother and fetus and has
contributions to the management of infants with CDH

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 2074 Section XIII Specialties in General Surgery

has been the concept of gentle ventilation with permis-

sive hypercapnia and stable hypoxemia (tolerance of
preductal oxygen saturations above 80%.). Using this
strategy, Stolar and colleagues reported a survival rate of
75.8%.38

Surgical Management
Historically, the surgical repair of a CDH was considered
to be a surgical emergency because it was thought that
the abdominal viscera within the chest prevented the
ability to ventilate. More recently, it has become evident
that the physiologic stress associated with early repair
probably adds more insult and that survival is not
improved when compared with delayed repair. Thus,
most pediatric surgeons wait for a variable period of time
(24-72 hours) to allow for stabilization of the infant
before embarking on surgical repair.
Most pediatric surgeons repair a posterolateral CDH
through an abdominal subcostal incision, although a A
thoracotomy will also provide adequate exposure. The
viscera are reduced into the abdominal cavity, and the
posterolateral defect in the diaphragm is closed using
interrupted, nonabsorbable sutures. In most cases
(roughly 80%-90%), a hernia sac is not present. If identi-
fied, however, it is excised at the time of repair. Occa-
sionally, the defect is too large to permit primary closure,
and a number of reconstructive techniques are available,
including various abdominal or thoracic muscle flaps.
The use of prosthetic material such as Gore-Tex has
become more widespread. The advantage of a prosthetic
patch is that a tension-free repair can be frequently
obtained. The major problems with prosthetic patches
are the risk for infection and recurrence of the hernia.
Occasionally, the abdominal compartment may be too
small to accommodate the viscera, which have developed
within the thoracic cavity. In these circumstances, an
abdominal silo may need to be constructed, as in the
management of congenital abdominal wall defects.
Beyond the early postoperative period, many infants
with CDH have continued morbidity, which demands
careful long-term follow-up.39 Many children who survive
aggressive management of severe respiratory failure man-
ifest neurologic problems, such as abnormalities in both
motor and cognitive skills, developmental delay, seizures,
and hearing loss. Other problems include a high inci-
dence of foregut gastroesophageal reflux and foregut
dysmotility. Other morbidity associated with CDH survi-
vors includes chronic lung disease, scoliosis, growth B
retardation, and pectus excavatum deformities. Figure 71-17 Pectus excavatum (A) and pectus carinatum (B).

times more frequent in males and is identified in the first

CONGENITAL CHEST WALL DEFORMITIES
Although several categories of congenital chest wall
deformities exist, the two major types are pectus excava-
tum and pectus carinatum (Fig. 71-17). Pectus excavatum
is also referred to as a funnel or sunken chest and is the
most common deformity encountered (roughly five times
more common than carinatum deformities). It is three
year of life in roughly 90% of cases.
Etiology
Although the etiology is unknown, abnormalities of costal
cartilage development have been most frequently impli-
cated. Several conditions are known to be associated with
pectus excavatum and must be considered in the preop-

Ch071-X3675.indd 2074 8/28/2007 11:37:54 AM


erative evaluation. Roughly 15% of patients have scolio-
sis. In addition, the possibility of Marfan syndrome must
be considered, and ophthalmologic evaluation, along
with an echocardiogram, obtained. Mitral valve prolapse
may be seen in about half of patients and structural con-
genital heart disease in roughly 2%. Asthma is also
common, but it is unknown whether asthma contributes
to the development of the defect or occurs as a result
of it.
Indications for Surgery
The most common indication for surgery in patients with
pectus deformities is cosmetic. This is not a minor issue,
particularly for adolescents with significant concerns
regarding body image and development of self-esteem.
Theoretically, correction of a severe excavatum deformity
will significantly improve cardiopulmonary function.
However, notwithstanding many decades of experience
with this condition, no appreciable consensus has been
reached regarding the degree of cardiopulmonary impair-
ment, if any, that this common chest wall deformity pro-
duces. Despite this, it is important to screen for underlying
cardiopulmonary conditions before embarking on opera-
tive correction.
Preoperative Evaluation
A standard anteroposterior and lateral chest radiograph
is essential to serve as a baseline of the degree of defor-
mity as well as to detect the presence of thoracic scolio-
sis. Pulmonary function studies are important to document
either restrictive or obstructive abnormalities. The latter
is particularly important if this component is reversible
with bronchodilators. If a heart murmur is detected on
physical examination, an echocardiogram is indicated.
Finally, a CT scan permits the calculation of a Haller
index by dividing the measured transverse diameter of
the chest by the anteroposterior diameter to more objec-
tively document the severity of the defect. This measure-
ment is affected by both age and gender.40
Surgical Treatment
The surgical correction of a pectus excavatum is not done
before the age of 5 years because a severe, postoperative
restrictive chest wall deformity may result. Presently,
there are two main methods for operative correction. The
first technique was originally described in 1949 by Ravitch
and remains the standard to which all other procedures
are compared. This procedure is applied to patients with
either excavatum or carinatum deformities and consists
of a transverse skin incision overlying the deformity,
bilateral subchondral resection of abnormal costal carti-
lages, sternal osteotomy, and anterior fixation of the
sternum with a retrosternal stainless steel strut. The strut
is removed as a secondary procedure in 6 months to a
year. The results with this operation are excellent.
More recently, a minimally invasive technique has
been described for excavatum defects in which a C-
shaped bar is passed in a retrosternal plane from one
hemithorax into the other through two lateral intercostal
Ch071-X3675.indd 2075
Chapter 71 Pediatric Surgery 2075
incisions. The bar is then “flipped” such that the convex-
ity is outward, and the chest wall defect is immediately
corrected. As described by Nuss,41 this technique avoids
the creation of pectoral flaps, cartilage resection, and
sternal osteotomy. The bar must be left in place for 2
years after which it is removed. Although this new tech-
nique has gained considerable popularity among the lay
public, the advantages of this technique over the standard
Ravitch procedure has yet to be conclusively demon-
strated. A multicenter, prospective trial to address this
issue is currently ongoing. Treatment of pectus carinatum
is done by open costal cartilage resection and sternal
fixation in the same way as for pectus excavatum. More
recently, success has been described with external brace
application to correct carinatum deformities.42
BRONCHOPULMONARY MALFORMATIONS
Dramatic improvements in prenatal ultrasonography have
led to a more frequent recognition of developmental
abnormalities of the lungs and major bronchi. Some
lesions may be associated with in utero death unless fetal
intervention is performed, some infants may have respira-
tory compromise at birth, and some patients may present
later in life with a persistent infection or neoplasm.
Bronchogenic Cyst
These cysts are usually solitary and lined by cuboidal or
columnar ciliated epithelium and mucus glands. Roughly
two thirds of cysts are within the lung parenchyma, and
the remainder are found within the mediastinum. Cysts
within the pulmonary parenchyma typically communi-
cate with a bronchus, whereas those in the mediastinum
usually do not. Although up to one third of patients are
asymptomatic and the diagnosis is made on a routine
chest radiograph, many patients present with respiratory
complaints, including recurrent pneumonia, cough,
hemoptysis, or dyspnea. Because of these symptoms, as
well as the reports of neoplasm occurring within these
cysts, treatment for all bronchogenic cysts is resection.
Frequently, mediastinal cysts may be amenable to resec-
tion using minimally invasive techniques.
Pulmonary Sequestration
Sequestrations represent malformations of the lung in
which there is usually no bronchial communication and
there is frequently an aberrant systemic blood supply.
Sequestrations are discriminated on the basis of being
either intralobar, in which they reside within the lung
parenchyma, or extralobar, in which they are surrounded
by a separate pleural covering. Intralobar sequestrations
are infrequently associated with other anomalies and are
found within the medial or posterior segments of the
lower lobes, with about two thirds occurring on the left
side. In about 85% of cases, the intralobar sequestration
is supplied by an anomalous systemic vessel arising from
the infradiaphragmatic aorta and located within the infe-
rior pulmonary ligament. Anticipation of this structure is
therefore critical during attempted resection of this
8/28/2007 11:37:57 AM
 2076 Section XIII Specialties in General Surgery
malformation. The venous drainage is usually through
the inferior pulmonary vein, but may also occur by way
of systemic veins. Because of the risk for infection and
bleeding, intralobar sequestrations are usually removed,
either by segmentectomy or lobectomy. Historically, angi-
ography was considered an important preoperative study
before embarking on resection of a sequestration. More
recently, CT and MRI have replaced the need for angi-
ography and provide excellent mapping of the blood
supply.
In contrast with those that are intralobar, extralobar
sequestrations occur predominantly in males (3 : 1 male-
to-female ratio) and are found three times more fre-
quently on the left side. In roughly 40% of cases, multiple
other anomalies are encountered, including posterolat-
eral diaphragmatic hernia, eventration of the diaphragm,
pectus excavatum and carinatum, enteric duplication
cysts, and congenital heart disease. Extralobar sequestra-
tions are usually asymptomatic, and because there is
usually no bronchial communication, the risk for infec-
tion is low. As such, many of these malformations may
be observed. Frequently, their discovery during other
procedures or inability to make the correct diagnosis by
noninvasive imaging dictates their removal.
Congenital Lobar Emphysema
Congenital lobar emphysema (CLE) results from overdis-
tention of one or more lobes within a histologically
normal lung due to abnormal cartilaginous support of the
feeding bronchus. This focal area of bronchial collapse
results in a check-valve with air trapping and a progres-
sive increase in lobar distention. Most often, the cartilage
within the bronchus is abnormal; however, extrinsic com-
pression of the bronchus from an aberrant vessel may
also cause the same findings. The left upper lobe is
involved in roughly half of cases, with the remainder
evenly distributed between the right middle and lower
lobes.
The symptoms of CLE range from none to severe
respiratory distress within the neonatal period. Asymp-
tomatic patients are often identified during a routine
chest radiograph as an area of hyperlucency. In these
cases, observation without pulmonary resection may be
prudent. Occasionally, CLE is identified in a patient with
recurrent of persistent pneumonia or with progressive
dyspnea. Resection of the involved lung is therapeutic
and well tolerated. The presentation of CLE in a neonate
may include severe respiratory distress. In these cases,
the clinical and radiographic picture may mimic a tension
pneumothorax with severe mediastinal shift. Inadvertent
placement of a chest tube into the distended lung would
be catastrophic. Immediate thoracotomy with resection
of the involved lobe may be lifesaving.
Congenital Cystic Adenomatoid Malformation
A congenital cystic adenomatoid malformation (CCAM)
typically involves a single lobe and represents a multicys-
Ch071-X3675.indd 2076
tic mass of pulmonary tissue in which there is proliferation
of bronchial structures at the expense of alveoli. Unlike
sequestrations, a CCAM typically has a bronchial commu-
nication, and the arterial and venous drainage is classi-
cally from the normal pulmonary circulation. There are
three general types segregated on the basis of cyst size. A
type I CCAM is considered the macrocystic variety and
includes single or multiple cysts greater than 2 cm. Type
I lesions account for roughly 50% of all cases and usually
have no associated anomalies. A type II CCAM contains
respiratory epithelium–lined cysts, but less than 1 cm in
size. Type II lesions are associated with other anomalies
such as renal agenesis, cardiac malformations, CDH, or
skeletal abnormalities. The outcome of patients with type
II CCAM depends on the associated conditions. A type III
CCAM is considered microcystic and on gross inspection
may appear solid, but microscopic analysis shows multi-
ple cysts. Type III CCAMs are often associated with medi-
astinal shift, the development of nonimmune hydrops,
and a generally poor prognosis. In utero surgery has been
applied with some success in the management of large
CCAMs. The development of nonimmune hydrops is one
of the main predictors of survival and in utero interven-
tion (thoracoamniotic shunting, fetal thoracotomy) may
be indicated.43 The postnatal management of the symp-
tomatic patient is relatively straightforward by pulmonary
resection in the newborn period. In asymptomatic patients
with small lesions detected by fetal ultrasound, the ra-
tionale for resection becomes less clear. Because there
have been reports of malignancy developing within these
lesions, as well as the potential for infection and enlarge-
ment, they probably all need to be resected.
HEPATOBILIARY
Neonatal Jaundice
Many children with chronic or life-threatening liver
disease present in the neonatal period. As such, early
recognition is important for apt diagnosis, treatment, and
improved outcome. Jaundice is the most common
symptom associated with neonatal liver disease. Physio-
logic (unconjugated) jaundice of the newborn is the most
common cause of jaundice and seen in up to 15% of
term infants by 2 weeks of age. Cholestasis is defined as
a pathologic state of reduced bile formation or flow.
Because of the relative immaturity of enzymes involved
with bile formation and transport, the neonate is particu-
larly prone to develop cholestasis in response to a wide
variety of insults that would not normally be associated
with cholestasis in adults. As such, the differential diag-
nosis of cholestasis is broader in neonates when com-
pared with adults.
Definition of Pathologic Hyperbilirubinemia
The possibility of liver disease is considered in any infant
who is jaundiced beyond 2 weeks of age. Hyperbilirubi-
nemia is considered pathologic if the direct (conjugated
fraction) serum bilirubin is greater than 1.0 mg/dL or
8/28/2007 11:37:57 AM

Table 71-4 Most Common Causes of Cholestasis in Infants Younger Than 2 Months
OBSTRUCTIVE CHOLESTASIS HEPATOCELLULAR CHOLESTASIS
Biliary atresia Idiopathic neonatal hepatitis
Choledochal cysts Viral infection
Gallstones or biliary sludge Cytomegalovirus
Alagille syndrome HIV
Inspissated bile Bacterial infection
Cystic fibrosis Urinary tract infection
Neonatal sclerosing cholangitis Sepsis
Congenital hepatic fibrosis Syphilis
(Caroli’s disease)
From Moyer V, Freese DK, Whitington PF, et al: Guideline for the evaluation of cholestatic jaundice: Recommendations of the North American Society of
Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 39:115-128, 2004.
represents more than 20% of the total bilirubin when the
total bilirubin level is great than 5 mg/dL. Any infant
beyond 2 weeks of life with this level of direct hyperbili-
rubinemia needs to undergo a thorough investigation.
Differential Diagnosis
Although the list of conditions associated with neonatal
cholestasis is long, fewer than 15 disorders account for
greater than 95% of neonatal cholestasis. Idiopathic neo-
natal hepatitis accounts for roughly 30% to 40% of cases
and is characterized histologically by identification of
giant cell transformation in the absence of evidence of
other causes of neonatal cholestasis. Treatment is sup-
portive. Biliary atresia is the second most common cause
of neonatal cholestasis. Treatment for this condition is
urgent and requires surgical intervention to reestablish
bile flow (discussed later). The third most common cause
of neonatal cholestasis is α1-antitrypsin deficiency. This
autosomal recessive disorder results in a misfolded
protein that cannot traverse the secretory pathway. Addi-
tional causes of cholestasis in infants younger than 2
months are shown in Table 71-4.
Evaluation
In the assessment of neonatal liver disease, it is important
to distinguish between hepatocellular, physiologic,
medical treatment and obstructive, anatomic, surgical
treatment of cholestasis. In an ill-appearing infant, the
primary cause (e.g., sepsis, hypothyroidism, metabolic
disorder) needs to be investigated and corrected. In the
absence of underlying liver disease, treatment of the
causal condition results in resolution of the cholestasis.
Further testing includes urine for reducing substances,
serum for α1-antitrypsin and albumin, and a complete
blood count with coagulation studies.
A hepatobiliary ultrasound is performed simultane-
ously with the previously mentioned diagnostic tests. An
absent gallbladder would be suggestive of biliary atresia,
whereas a cystic hilar mass would suggest the diagnosis
Ch071-X3675.indd 2077
Chapter 71 Pediatric Surgery 2077
GENETIC AND METABOLIC
DISORDERS TOXIC OR SECONDARY
α1-Antitrypsin deficiency Parenteral nutrition–
Tyrosinemia associated cholestasis
Galactosemia
Hypothyroidism
Progressive familial intrahepatic
cholestasis (PFIC)
Cystic fibrosis
Panhypopituitarism
of a choledochal cyst. Other less common conditions in
which an ultrasound would be useful include gallstones
or biliary sludge or the presence of an obstructing hilar
tumor. The final diagnostic procedure in the evaluation
of the cholestatic infant is a percutaneous liver biopsy.
This modality provides the greatest accuracy for diagnos-
ing neonatal liver disease. Hepatobiliary scintigraphy
scans, although historically useful, add little to the com-
bination of ultrasonography and percutaneous liver
biopsy and are no longer routinely employed.
Biliary Atresia
Biliary atresia (BA) is characterized by progressive (not
static) obliteration of the extrahepatic and intrahepatic
bile ducts. The cause is presently unknown, and the
incidence is about 1 in 15,000 live births. Presently, there
is no medical therapy to reverse the obliterative process,
and patients who are not offered surgical treatment uni-
formly develop biliary cirrhosis, portal hypertension, and
death by 2 years of age.
Pathology
Pathologically, the biliary tracts contain inflammatory and
fibrous cells surrounding miniscule ducts that are proba-
bly remnants of the original ductal system. Bile duct
proliferation, severe cholestasis with plugging, and
inflammatory cell infiltrate are the pathologic hallmarks
of this disease. Over time, these changes progress to
fibrosis with end-stage cirrhosis. This histology is usually
distinct from the giant cell transformation and hepatocel-
lular necrosis that are characteristic of neonatal hepatitis,
the other major cause of direct hyperbilirubinemia in the
newborn. There are variants of BA ranging from fibrosis
of the distal bile ducts with proximal patency (5%, con-
sidered correctable form), fibrosis of the proximal bile
ducts with distal patency (15%), or fibrosis of both proxi-
mal and distal bile ducts (80%).
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 2078 Section XIII Specialties in General Surgery
Diagnosis
A serum direct bilirubin level of more than 2.0 mg/dL or
greater than 15% of the total bilirubin level defines cho-
lestasis, is distinctly abnormal, and further evaluation is
mandatory. Delay in diagnosis of BA is associated with
a worse prognosis. Success with surgical correction is
much improved if undertaken before 60 days of life and
related to the degree of hepatic fibrosis.44 Thus, the initial
opportunity for success in the management of this disease
relies on the early recognition of abnormal direct
hyperbilirubinemia.
The list of potential causes for cholestasis in infants is
relatively long; however, an organized, systematic
approach usually permits the establishment of an accu-
rate diagnosis within a few days. In addition to a careful
history and physical examination, blood and urine are
obtained for bacterial and viral cultures, reducing sub-
stances in the urine to rule out galactosemia, serum
immunoglobulin M (IgM) titers for syphilis, cytomegalo-
virus, herpes, and hepatitis B, serum α1-antitrypsin level
and phenotype, serum thyroxine level, and a sweat chlo-
ride test done to exclude cystic fibrosis.
Ultrasonography of the liver and gallbladder is impor-
tant in the evaluation of the infant with cholestasis. In
BA, the gallbladder is typically shrunken or absent, and
the extrahepatic bile ducts cannot be visualized. The next
diagnostic step is to perform a percutaneous liver biopsy
if the hepatic synthetic function is normal. This is well
tolerated under local anesthesia, and the diagnostic accu-
racy is in the range of 90%.45 In cases in which the
ultrasound and biopsy findings are inconclusive, hepato-
biliary scintigraphy, using iminodiacetic acid analogues,
may demonstrate normal hepatic uptake, but absent
excretion into the intestine. Pretreatment of the infant
with phenobarbital may improve the sensitivity of this
test.
Surgical Management
If the needle biopsy or abdominal ultrasound is consis-
tent with BA, exploratory laparotomy is then performed
expeditiously. The initial goal at surgery is to confirm the
diagnosis. This requires the demonstration of the fibrotic
biliary remnant and definition of absent proximal and
distal bile duct patency by cholecystocholangiography.
The classic technique for correction of BA is the Kasai
hepatoportoenterostomy. In this procedure, the distal bile
duct is transected and dissected proximally up to the
level of the liver capsule, whereby it is excised, along
with the gallbladder remnant (Fig. 71-18). A Roux-en-Y
hepaticojejunostomy is then constructed by anastomosis
of the jejunal Roux limb to the fibrous plate above the
portal vein. Some surgeons prefer to monitor postopera-
tive bile flow by constructing a distal double-barrel stoma.
Although it has been considered that this may lessen
the risk for cholangitis, this has yet to be definitively
established.
Postoperative Management
Postoperatively, the use of oral choleretic bile salts such
as ursodeoxycholic acid may facilitate bile flow.46 In
Ch071-X3675.indd 2078
Porta hepatis
PV
HA
Figure 71-18 Kasai’s hepatoportoenterostomy procedure for
biliary atresia. The extrahepatic bile ducts and gallbladder have
been removed. The fibrous plate of the hepatic duct is tran-
sected above the bifurcation of the portal vein (PV) and hepatic
artery (HA), and a Roux limb of jejunum is sewn to this plate to
achieve drainage of bile. (From Grosfeld JL, Fitzgerald JF,
Predaina R, et al: The efficacy of hepatoportoenterostomy in
biliary atresia. Surgery 106:692-700, 1989).
addition, methylprednisolone is employed as an anti-
inflammatory agent, and trimethoprim sulfamethoxazole
is administered for long-term antimicrobial prophylaxis.
Cholangitis is a serious but common problem after hepa-
toportoenterostomy and may be associated with cessa-
tion of bile flow. Episodes of cholangitis are managed by
hospitalization, rehydration, broad-spectrum IV antibiot-
ics, steroids, and occasionally surgical exploration of the
portoenterostomy.
Outcome
About 30% of infants undergoing hepatoportoenteros-
tomy before 60 days of age will have a long-term suc-
cessful outcome and not require liver transplantation.
Older children and those with preoperative evidence for
bridging fibrosis seen on liver biopsy will predictably do
less well. As such, some surgeons may forgo performing
a portoenterostomy procedure and simply place the
patient on a waiting list for liver transplantation. The
remaining patients undergoing portoenterostomy will
develop progressive hepatic fibrosis with resultant portal
hypertension and progressive cholestasis. In this group,
liver transplantation is lifesaving and associated with a
79% survival rate.47 Biliary atresia currently represents
the most common indication for pediatric liver
transplantation.
Choledochal Cyst
A cystic enlargement of the common bile duct is referred
to as a choledochal cyst. The initial anatomic organization
was proposed by Alonso-Lej in 1959 and has been
updated to the current classification as depicted in Figure
71-19. Type I cysts represent 80% to 90% of cases and
are simply cystic dilations of the common bile duct. Type
II cysts are represented as a diverticulum arising from the
common bile duct. Type III cysts are also referred to as
8/28/2007 11:37:58 AM

I II
IV
Figure 71-19 The anatomic classification of choledochal cyst. (From Sigalet DL: Biliary tract disorders and portal
hypertension. In Ashcraft KW, Sharp RJ, Sigalet DL, Snyder CL [eds]: Pediatric Surgery, 3rd ed. Philadelphia, WB
Saunders, 2000, p 588.)
choledochoceles and are isolated to the intrapancreatic
portion of the common bile duct and frequently involve
the ampulla. Type IV cysts are second in frequency and
represent dilation of both intrahepatic and extrahepatic
bile ducts. In Type V cysts, only the intrahepatic ducts
are dilated.
The pathophysiology of choledochal cysts remains
poorly understood. In one theory, reflux of pancreatic
digestive enzymes into the bile duct through an anoma-
lous pancreaticobiliary ductal junction results in damage
to the duct. In another theory, persistent or transient
obstruction of the distal bile duct may be present.
Clinical Presentation
Although choledochal cysts can produce symptoms in
any age group, most become clinically evident within the
first decade of life. The triad of a right upper quadrant
mass, abdominal pain, and jaundice is highly suggestive
of the diagnosis. In some patients, pancreatitis may be
present. In older children and adults, the presentation
may be more insidious and include choledocholithiasis,
cholangitis, and cirrhosis with progression to portal
hypertension. Malignant degeneration is also found in up
to 16% of adults with choledochal cysts.
Preoperative Evaluation
In addition to routine measurement of serum bilirubin,
alkaline phosphatase, and amylase levels, the most useful
diagnostic test for choledochal cysts is ultrasonography.
Ch071-X3675.indd 2079
Chapter 71 Pediatric Surgery 2079
III
V
Once dilation of the extrahepatic biliary ducts is demon-
strated, no further testing is usually necessary in children.
Although seldom necessary, preoperative endoscopic
retrograde cholangiopancreatography (ERCP) may
provide addition information regarding the pancreati-
cobiliary ductal anatomy to guide intraoperative decision
making.
Operative Management
Total cyst excision with Roux-en-Y hepaticojejunostomy
is the definitive procedure for management of type I and
II choledochal cysts. In cases in which there is significant
inflammation, it may be impossible to safely dissect the
entire cyst away from the anterior surface of the portal
vein. In these circumstances, the internal lining of the
cyst can be excised, leaving the external portion of the
cyst wall intact. Type III cysts are typically approached
by opening the duodenum, resecting the cyst wall with
care to reconstruct and marsupialize the remnant pancre-
aticobiliary ducts to the duodenal mucosa. In type IV
cysts, the bile duct excision is coupled with a lateral hilar
dissection to perform a jejunal anastomosis to the low-
ermost intrahepatic cysts. If the intrahepatic cysts are
confined to a single lobe or segment, hepatic resection
may be indicated. The treatment of type V cysts involving
both lobes is usually palliative with transhepatic or U-
tubes until liver transplantation can be performed. The
postoperative outcomes following excision of chole-
dochal cysts are excellent.48
8/28/2007 11:37:58 AM
 2080 Section XIII Specialties in General Surgery
CHILDHOOD SOLID TUMORS
Neuroblastoma
Neuroblastoma (NBL) is the most common abdominal
malignancy in children, accounting for 6% to 10% of
all childhood cancers and 15% of all pediatric cancer
deaths in the United States. The overall incidence in an
unscreened population is 1 case per 10,000 persons with
roughly 525 new cases diagnosed in the United States
each year.
Sites of Involvement
These tumors are of neural crest origin and as a result
may arise anywhere along the sympathetic ganglia or
within the adrenal medulla. Although these tumors may
occur at any site from the brain to the pelvis, 75% origi-
nate within the abdomen or pelvis, and half of these
occur within the adrenal medulla. Twenty percent of
NBLs originate within the posterior mediastinum, and 5%
are within the neck. The median age at diagnosis is 2
years. Nearly 35% occur in children younger than 1 year,
and less than 5% of cases present after the age of 10
years.
NBL is an enigmatic tumor that is capable of rapid
progression in some children and spontaneous regression
in others, particularly those younger than 1 year. About
25% of patients present with a solitary mass that may be
cured by surgical therapy, whereas most present with
extensive locoregional or metastatic disease. In this latter
group of patients, the prognosis is generally poor, with
an overall survival rate of less than 30%.
Clinical Presentation
The presenting symptoms of NBL are dependent on
several factors, including the site of the primary tumor,
the presence of metastatic disease, the age of the patient,
and the metabolic activity of the tumor. The most common
presentation is a fixed, lobular mass extending from the
flank toward the midline of the abdomen. Although the
abdominal mass may be noted in an otherwise asymp-
tomatic child, patients may complain of abdominal pain,
distention, weight loss, or anorexia. Bowel or bladder
dysfunction may arise from direct compression of these
structures by the tumor. Cervical tumors may be discov-
ered as a palpable or visible mass or be associated with
stridor or dysphagia. Posterior mediastinal masses are
usually detected by plain chest radiographs in a child
with Horner’s syndrome, dyspnea, or pneumonia. Further,
the tumor may extend into the neural foramina and cause
symptoms of spinal cord compression. Neuroblastoma
tends to metastasize to cortical bones, bone marrow, and
liver. As such, patients may present with localized swell-
ing and tenderness, lump, or refusal to walk. Periorbital
metastasis accounts for proptosis and ecchymosis (so-
called panda or raccoon eyes). Marrow replacement by
tumor may result in anemia and weakness. In infants,
liver metastasis may rapidly expand, causing massive
hepatomegaly and respiratory distress requiring mechani-
cal ventilation and surgical decompression. Metastatic
lesions to the skin produce a characteristic blueberry
muffin appearance.
Ch071-X3675.indd 2080
Figure 71-20 Neuroblastoma. A CT scan of the abdomen demon-
strating a large neuroblastoma surrounding the aorta (arrow)
and displacing the liver to the right. Punctate areas of calcium
can be seen dispersed throughout the tumor.
Numerous paraneoplastic syndromes can occur in
conjunction with NBL. Cerebellar ataxia, involuntary
movements, and nystagmus are the hallmark of the
“dancing eyes and feet” syndrome. Excess secretion of
vasoactive intestinal polypeptide may stimulate an intrac-
table watery diarrhea. Hypertension may be significant,
owing to excessive catecholamine production by the
tumor.
Preoperative Evaluation
Although histologic evaluation of tissue is necessary for
establishing the definitive diagnosis, a high level of sus-
picion may arise from the history and physical examina-
tion. Initial laboratory evaluation includes a complete
blood count, serum electrolytes, blood urea nitrogen,
creatinine, and liver function studies. A spot urine test is
done for the catecholamine metabolites homovanillic and
vanillylmandelic acid. In addition, several other biochem-
ical markers harbor prognostic significance. A serum
lactic dehydrogenase level greater than 1500 IU/mL,
serum ferritin level above 142 ng/mL, and neuron-spe-
cific enolase levels greater than 100 ng/mL correlate with
advanced disease and reduced survival.
CT and MRI are the preferred modalities for character-
izing the location and extent of the neuroblastoma. This
tumor frequently infiltrates vascular structures (Fig.
71-20). As such, many tumors that cross the midline are
generally not resectable. A CT scan of the chest is done
to exclude pulmonary metastasis and a bone scan to
identify potential bone metastasis. In addition, radiola-
beled metaiodobenzylguanidine (MIBG) is one of the
single best studies to document the presence of meta-
static disease. Finally, a bone marrow aspirate and biopsy
complete the staging evaluation. The international NBL
staging system is depicted in Table 71-5.
Although imaging at the time of presentation of most
tumors reveals unresectability, the definitive diagnosis
requires tissue. This can be obtained by an incisional or
needle biopsy of the tumor. Neuroblastoma identified
8/28/2007 11:37:58 AM
 Chapter 71 Pediatric Surgery 2081

Table 71-5 International Neuroblastoma Staging System Table 71-6 Schema of Clinical Factors Combined for Patient
Risk Group Assignment in Future Neuroblastoma Studies*
STAGE DEFINITION
RISK GROUP FACTORS
1 Localized tumor with complete gross excision,
with or without microscopic residual disease; Low Stage 1
representative ipsilateral lymph nodes negative Stage 2 <1 yr
for tumor microscopically (nodes attached to >1 yr, low N-myc
and removed with the primary tumor may be >1 yr, amplified N-myc;
positive) favorable histology
2A Localized tumor with incomplete gross excision; Stage 4S favorable biology
representative ipsilateral nonadherent lymph Intermediate Stage 3 <1 yr, low N-myc
nodes negative for tumor microscopically >1 yr, favorable biology
2B Localized tumor with or without complete gross Stage 4 <1 yr, low N-myc
excision, with ipsilateral nonadherent lymph Stage 4S Low N-myc
nodes positive for tumor; enlarged contralateral High Stage 2 >1 yr, all unfavorable biology
lymph nodes must be negative microscopically Stage 3 <1 yr, amplified N-myc
3 Unresectable unilateral tumor with contralateral >1 yr, any unfavorable biology
regional lymph node involvement; or midline Stage 4 <1 yr, amplified N-myc
tumor with bilateral extension by infiltration >1 yr
(unresectable) or by lymph node involvement Stage 4S Amplified N-myc

4 Any primary tumor with dissemination to distant
lymph nodes, bone, bone marrow, liver, skin,
and/or other organs (except as defined for
stage 4S)
4S Localized primary tumor (as defined for stage 1,
2A, or 2B), with dissemination limited to skin,
liver, and/or bone marrow (limited to infant
<1 yr of age)
within bone marrow aspirate or biopsy may also be
sufficient.
Prognostic Factors
Cytogenetic studies provide significant prognostic infor-
mation that may affect treatment. Amplification of the
N-myc oncogene is one of the classic factors associated
with rapid tumor progression and poor prognosis. In
addition, gain of genetic material from chromosome arm
17q is associated with deletion of chromosome 1p and
N-myc amplification and is highly predictive of poor
outcome.49 Diploid tumors have an unfavorable progno-
sis, whereas hyperdiploid tumors have a better prognosis.
Further, expression of the TRK proto-oncogene is inversely
associated with N-myc amplification and has a more
favorable prognosis. Finally, expression of the multidrug
resistance–associated protein (MRP) is associated with a
poor outcome. In addition to the cytogenetic studies,
prognosis may be derived from the pathologic classifica-
tion as proposed by Shimada and colleagues,50 taking
into account the degree of differentiation, the mitotic-
karyorrhexis index, and the presence or absence of
stroma.
*Favorable biology denotes low N-myc, favorable histology, and
hyperdiploidy (infants).
sional biopsy is the initial procedure, with re-evaluation
for resection following a course of adjuvant therapy. After
cytoreductive therapy, attempts at resection may be the
only option for long-term survival. Meticulous dissection
of major blood vessels, which often course through the
tumor, is required. These procedures are frequently pro-
longed and associated with significant blood loss.
Survival
Children of any age with localized neuroblastoma and
infants younger than 1 year with advanced disease and
favorable disease characteristics have a high likelihood
of long-term, disease-free survival. Older children with
advanced-stage disease, however, have a significantly
decreased chance for cure despite intensive therapy.
Prognosis resides in stratification of patients into low-,
intermediate-, and high-risk categories (Table 71-6).
These are associated with survival rates of greater than
90%, greater than 80%, and 10% to 20%, respectively.51
Wilms’ Tumor
Wilms’ tumor (WT) is an embryonal tumor of renal origin
and is the most common primary malignant kidney tumor
of childhood. Roughly 500 new cases of WT are diag-
nosed in the United States each year. This tumor is most
frequently seen in children between the ages of 1 and 5
years (∼80%), with a peak incidence between 3 and 4
years. Bilateral WT is present in up to 13% of cases and,
when present, is synchronous in 60%.

Treatment
Current therapy for NBL is multimodal, incorporating
surgery, chemotherapy, radiation, and occasionally immu-
notherapy. Surgical resection of the primary tumor and
adjacent lymph nodes is the goal and may be curative
for localized stage 1 and 2 disease. In most situations in
which the tumor is unresectable, exploration with inci-
Etiology
Despite the number of genes implicated in the genesis
of this neoplasm, hereditary WT is uncommon. Specific
germline mutations in one of these genes (Wilms’ tumor
gene-1, WT1) located on the short arm of chromosome
11, are not only associated with WT but also cause a
variety of genitourinary abnormalities such as cryptorchi-

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 2082 Section XIII Specialties in General Surgery

dism and hypospadias. A gene that causes aniridia is

located near the WT1 gene on chromosome 11p13, and
deletions encompassing the WT1 and aniridia genes may
explain the association between these two conditions.
There appears to be a second WT gene at or near the
Beckwith-Wiedemann gene locus, also on chromosome
11. Children with Beckwith-Wiedemann syndrome
(omphalocele, visceromegaly, macroglossia, and hypo-
glycemia) are at increased risk for WT. About one fifth
of patients with Beckwith-Wiedemann syndrome who
develop WT present with bilateral disease at the time of
diagnosis.

Clinical Presentation
Most patients (60%) with WT present clinically with a
palpable abdominal mass (Fig. 71-21). Often, the patient
has no symptoms, and the parents discover the mass
during bathing, or the pediatrician discovers it during a
A
routine physical examination. Hypertension is present in
about 25% of patients and hematuria in 15%. Because
WT is associated with several syndromes, including the
Denys-Drash syndrome (WT, intersex disorder, and pro-
gressive nephropathy), WAGR syndrome (WT, aniridia,
genitourinary anomalies, mental retardation), and
Beckwith-Wiedemann syndrome, patients with these
phenotypes need to be screened closely into adulthood
for the potential development of WT.

Preoperative Evaluation
The initial evaluation of the child with an abdominal
mass and suspected WT is by ultrasonography. This is
useful in confirming not only that the mass originates
from the kidney, but also whether the mass is cystic or
solid, and in the detection of potential tumor thrombus
within the renal vein and inferior vena cava (IVC). Fre-
quently, it is difficult to distinguish WT from NBL. Both
CT and MRI are frequently useful in this regard (Fig.
71-22) because WT originates in the kidney and NBL
develops in the adrenal or sympathetic ganglia. In cases
in which the origin of the mass is difficult to determine,
urinary catecholamine measurements can distinguish WT
from NBL; they are elevated in most cases of NBL, but
not in WT. CT and MRI are also indicated preoperatively
to identify bilateral WT, characterize potential invasion
into surrounding structures, document liver or lung
metastasis, and detect tumor thrombus within the IVC. A
preoperative plain chest radiograph is also necessary for
staging purposes.
Surgical Management
After the preoperative evaluation is completed as outlined
earlier, exploratory laparotomy is crucial for both staging
and treatment of WT. Preoperative chemotherapy may be
indicated in cases in which WT is present in a solitary or
horseshoe kidney or in both kidneys, with the presence
of respiratory distress from extensive metastatic tumor, or
when IVC tumor thrombus has extended above the level
of the hepatic veins. In these situations, chemotherapy-
induced tumor shrinkage may allow for a more complete
resection with less morbidity and with the potential to
salvage maximal functional renal parenchyma.
B
Figure 71-21 Wilms’ tumor. The large left-sided flank mass is
obvious on visual inspection (A). Upon entering the peritoneal
cavity (B), the large Wilms’ tumor within the left kidney (out-
lined by arrows) can be seen behind the descending colon, dis-
placing it anterior and medially.
The goals of operative therapy for WT are to confirm
the diagnosis, assess the opposite kidney and other
abdominal organs for metastatic spread, and completely
resect the primary tumor, ureter, and adjacent lymph
nodes. These are achieved through a generous transverse
or midline transperitoneal incision. At some point during
the exploration, Gerota’s fascia of the opposite kidney
must be opened to more definitively exclude bilateral
tumor. The anterior and posterior surfaces of the opposite
kidney must be carefully inspected and palpated. Despite
the large size of the tumor, complete resection by radical
nephroureterectomy can be safely performed. Care must
be taken to avoid tumor rupture because this increases
the stage of the tumor and mandates additional postop-
erative adjuvant therapy. Frequently, the ipsilateral
adrenal gland is removed en bloc with the kidney. Inva-

Ch071-X3675.indd 2082 8/28/2007 11:37:58 AM


Figure 71-22 CT scan of a Wilms’ tumor involving the right
kidney. Remnants of the remaining functional kidney (arrows)
are noted at the periphery of the tumor.
sion into surrounding organs such as the pancreas,
spleen, or liver may direct their removal as well.
Surgical exploration, coupled with the preoperative
imaging studies and histology, permits accurate staging of
WT, which correlates with prognosis and guides postop-
erative adjuvant therapy. The pathologic evaluation of WT
involves inspection of the three elements of normal renal
development (blastemal, epithelial, and stromal) and iden-
tification of the absence or presence of anaplasia, which
distinguishes the classification of either favorable (FH) or
unfavorable (UH) histology, respectively. The current
staging scheme as proposed by the National Wilms’ Tumor
Study (NWTS) group is depicted in Table 71-7.
Survival
Treatment of WT represents one of the greatest triumphs
in the field of pediatric oncology. In contrast with what
used to be a lethal malignancy, the current overall sur-
vival rate exceeds 85%. The successful treatment of this
tumor is a direct result of collaboration between multiple
disciplines to form two major associations (NWTS and
the International Society of Pediatric Oncology) in which
there has been a systematic organization of multicenter
trials designed to address focused, highly relevant ques-
tions. The recommended treatment of WT based on stage
is shown in Table 71-8. The survival rates of patients with
stage I or II FH or stage I UH are both about 95%. For
all stages, the overall survival rate of patients with FH is
90%. For patients with UH, stages II, III, and IV are asso-
ciated with 70%, 56%, and 17% 4-year survival rates,
respectively.52
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is a soft tissue malignant
tumor of skeletal muscle origin and accounts for about
3.5% of the cases of cancer among children younger than
14 years. It is a curable disease in most children, with
more than 60% surviving 5 years after diagnosis. The
most common primary sites for RMS are the head and
Ch071-X3675.indd 2083
Chapter 71 Pediatric Surgery 2083
Table 71-7Staging System Used by the National
Wilms’ Tumor Study Group
STAGE DEFINITION
I Tumor limited to the kidney and completely
excised without rupture or biopsy. Surface of
the renal capsule is intact.
II Tumor extends through the renal capsule but is
completely removed with no microscopic
involvement of the margins. Vessels outside
the kidney contain tumor. Also placed in stage
II are cases in which the kidney has
undergone biopsy before removal or where
there is “local” spillage of tumor (during
resection) limited to the tumor bed.
III Residual tumor is confined to the abdomen and
of nonhematogenous spread. Also included in
stage III are cases with tumor involvement of
the abdominal lymph nodes, “diffuse”
peritoneal contamination by rupture of the
tumor extending beyond the tumor bed,
peritoneal implants, and microscopic or grossly
positive resection margins.
IV Hematogenous metastases at any site.
V Bilateral renal involvement.
Table 71-8 NWTS-5 Treatment Recommendations
for Wilms’ Tumor*
Stage I (FH): Surgery, no radiotherapy,
dactinomycin + vincristine for 18 wk
Stage I focal anaplasia: Surgery, no radiotherapy,
dactinomycin + vincristine for 18 wk
Stage II (FH): Surgery, no radiotherapy,
dactinomycin + vincristine for 18 wk
Stage II focal anaplasia: Surgery, 1080 cGy to tumor bed,
dactinomycin + vincristine + doxorubicin for 24 wk
Stage III (FH): Surgery, 1080 cGy to tumor bed,
dactinomycin + vincristine + doxorubicin for 24 wk
Stage III focal anaplasia: Surgery, 1080 cGy to tumor
bed, dactinomycin + vincristine + doxorubicin for 24 wk
Stage IV (FH) focal anaplasia: Surgery, 1080 cGy to
tumor bed according to local tumor stage, 1200 cGy to
lung and/or other metastatic sites,
dactinomycin + vincristine + doxorubicin for 24 wk
Stage II-IV diffuse anaplasia: Surgery, radiotherapy
(whole lung; abdominal 1080 cGy),
cyclophosphamide +
etoposide + vincristine + doxorubicin + mesna for 24 wk
Stage I-IV (clear cell sarcoma): Surgery, radiotherapy
(abdominal 1080 cGy; whole lung, stage IV only),
cyclophosphamide + etoposide + vincristine +
doxorubicin + mesna for 24 wk
Stage I-IV (rhabdoid tumor): Surgery, radiotherapy,
carboplatin + etoposide + cyclophosphamide + mesna for
24 wk
*Infants <11 mo are given half the recommended dose of all drugs. Full
doses lead to prohibitive hematologic toxicity in this age group. Full
doses of chemotherapeutic agents should be administered to those
>12 mo.
NWTS, National Wilms’ Tumor Study.
8/28/2007 11:38:00 AM
 2084 Section XIII Specialties in General Surgery
neck (parameningeal, orbit, pharyngeal), the genitouri-
nary tract, and the extremities. Other, less common
primary sites include the trunk, gastrointestinal (including
liver and biliary) tract, and intrathoracic or perineal
region. Most cases of RMS occur sporadically with no
recognized predisposing factors, although a small pro-
portion are associated with other genetic conditions.
These include the Li-Fraumeni cancer susceptibility syn-
drome (with germline p53 mutations), neurofibromatosis
type I, and Beckwith-Wiedemann syndrome.
The prognosis for a child or adolescent with RMS is
related to patient age, site of origin, extent of tumor at
time of diagnosis or after surgical resection, and tumor
histology.53 Age less than 10 years is considered a more
favorable prognosis. With regard to tumor site, a more
favorable prognosis is afforded when tumors are located
in the orbit and nonparameningeal head and neck, geni-
tourinary system (excluding bladder and prostate), and
biliary tract.
Patients with smaller tumors (<5 cm) have improved
survival when compared with that of children with larger
tumors, whereas children with metastatic disease at diag-
nosis have the worst prognosis. The prognostic signifi-
cance of metastatic disease is further modulated by tumor
histology, patient age, and primary site. Patients younger
than 10 years with metastatic disease and with embryonal
histology have 5-year survival rates greater than 50%,
whereas those older than 10 years or with alveolar histol-
ogy have a much poorer outcome. The presence of
regional lymph node involvement is also associated with
a worse prognosis. The ability to resect the tumor com-
pletely is associated with a better outcome when com-
pared with gross residual disease after initial surgery.
From a histologic standpoint, the botryoid and spindle
cell subtypes are associated with a more favorable
outcome. Embryonal and pleomorphic subtypes are
intermediate, and alveolar or undifferentiated subtypes
are generally associated with a worse prognosis. Favor-
able prognostic groups have been identified by previous
Intergroup Rhabdomyosarcoma Studies, and treatment
plans have been designed based on assignment of patients
to different groups according to prognosis (Table 71-9).
The diagnostic workup generally involves CT or MRI.
Because there are no useful markers at present, an accu-
rate diagnosis depends on incisional biopsy of the tumor.
In the extremity, the direction of the incision must allow
it to be incorporated into the wound created by a sub-
sequent wide local excision.
All children with RMS require multimodality therapy.
This entails surgical resection, if possible, followed by
chemotherapy, followed by second-look surgery for some
patients with initially unresectable tumors, and depend-
ing on original histologic type, extent of disease, and
extent of resection, radiation therapy.
The basic surgical principles for the treatment of RMS
are complete resection of the primary tumor with a sur-
rounding margin of normal tissue, coupled with sampling
of the adjacent lymph nodes. This may not be feasible
in patients with obvious metastatic disease but is done if
possible. Because RMS can arise from so many primary
muscle sites, surgical care must be tailored to the unique
Ch071-X3675.indd 2084
Table 71-9 Staging for Rhabdomyosarcoma
Group I: Localized disease that is completely resected
with no regional node involvement (13%)
Group II: (∼20%)
IIA: Localized, grossly resected tumor with microscopic
residual disease but no regional nodal involvement.
IIB: Locoregional disease with tumor-involved lymph
nodes with complete resection and no residual
disease.
IIC: Locoregional disease with involved nodes, grossly
resected, but with evidence of microscopic residual
tumor at the primary site and/or histologic
involvement of the most distal regional node (from
the primary site).
Group III: Localized, gross residual disease including
incomplete resection, or biopsy only of the primary
site (∼48%).
Group IV: Distant metastatic disease present at the time
of diagnosis (∼18%).
aspects of each site. Surgical management of the more
common primary sites is described next.
Head and Neck
For those tumors that are superficial and nonorbital, wide
excision of the primary tumor with ipsilateral neck lymph
node sampling of clinically involved nodes is appropri-
ate. Because of cosmetic and functional concerns, margins
smaller than 1 mm are acceptable. For patients with
tumors that are considered unresectable, chemotherapy
and radiation therapy become the primary management.
Rhabdomyosarcomas of the orbit require a biopsy to
establish diagnosis and then chemotherapy and radiation
therapy. Orbital exenteration is reserved for the small
number of patients with local, persistent, or recurrent
disease.
Extremity
The definitive surgical procedure involves wide local exci-
sion with en bloc of normal tissue. If it is anatomically fea-
sible, a re-excision procedure is associated with better
outcome in patients whose initial surgical procedure left
microscopic residual disease on pathologic examination.
Amputation is reserved for selected patients with lesions
involving major neurovascular structures in addition to the
muscle of origin. Because of the significant incidence of
nodal spread for extremity primary tumors (often without
clinical evidence of involvement), and because of the
prognostic and therapeutic implications of nodal involve-
ment, surgical assessment for regional nodal involvement
is important.54 For clinically negative nodes, axillary or
femoral node sampling is done for upper or lower extrem-
ity tumors, respectively. If clinically positive nodes are
present, biopsy of more proximal nodes is recommended
before sampling of the involved nodal region.
Trunk
As with RMS in other locations, wide local excision and
an attempt to achieve negative microscopic margins is
8/28/2007 11:38:00 AM

the goal. Reconstruction may require use of prosthetic
materials. Extremely large masses undergo biopsy ini-
tially, followed by a course of chemotherapy with or
without radiation. This may shrink the tumor enough to
permit a subsequent margin-negative resection with suc-
cessful reconstruction.
Genitourinary
The initial surgical procedure in most patients consists of
a biopsy, which often can be performed using a cysto-
scope, transanally, or under direct vision. Bladder salvage
is an important goal of therapy for patients with tumors
arising in the prostate and bladder. Occasionally, when
the tumor is confined to the dome of the bladder, it can
be completely resected. Otherwise, pre-resection chemo-
therapy and radiation therapy allow preservation of a
functional bladder in most patients. For patients with a
biopsy-proven residual malignant tumor after chemo-
therapy and radiation therapy, appropriate surgical man-
agement may include partial cystectomy, prostatectomy,
or anterior exenteration.
Testis or spermatic cord RMS are removed by radical
orchiectomy and resection of the entire spermatic cord.
Resection of hemiscrotal skin may be necessary when
there is tumor fixation or invasion, or if a previous trans-
scrotal biopsy has been performed. Because paratesticu-
lar tumors are associated with a relatively high incidence
of lymphatic spread, all patients with paratesticular
primary tumors have thin-cut abdominal and pelvic CT
scans with contrast to evaluate nodal involvement. Ret-
roperitoneal lymph node sampling is needed for patients
with suggestive or positive CT scans who are younger
than 10 years. In contrast, an ipsilateral retroperitoneal
lymph node dissection is required for all children older
than 10 years with paratesticular RMS for staging.
Liver Tumors
Liver cancer is rare in childhood and essentially is either
hepatoblastoma (HBL) or hepatocellular carcinoma
(HCC). Several important differences exist between these
two subtypes. HBLs usually occur before 3 years of age,
whereas HCC may be found in children and adults of all
ages. Hepatoblastoma is most often unifocal, whereas
HCC is often extensively invasive or multicentric at the
time of diagnosis. Complete resection is therefore more
often possible in patients with HBL. Childhood HBL fre-
quently involves associated mutations in the β-catenin
gene, the function of which is closely related to the
development of familial adenomatous polyposis. In addi-
tion, HBL is associated with hemihypertrophy, very low
birthweight, and Beckwith-Wiedemann syndrome. In
contrast, HCC is associated with a history of perinatally
acquired hepatitis B and C infection, mutations in the
hepatocyte growth factor receptor gene (c-met), and tyro-
sinemia, biliary cirrhosis, and α1-antitrypsin deficiency.
The serum tumor marker α-fetoprotein levels parallel
disease activity for both HBL and HCC. The overall sur-
vival rate for children with HBL is 70%, as compared with
25% for HCC. A general staging scheme for hepatic
tumors in children is depicted in Table 71-10. Children
Ch071-X3675.indd 2085
Chapter 71 Pediatric Surgery 2085
Table 71-10 Liver Tumor Staging
STAGE DEFINITION
I No metastases, tumor completely resected.
II No metastases, tumor grossly resected with
microscopic residual disease (i.e., positive
margins); or tumor rupture, or tumor spill
at the time of surgery.
III No distant metastases, tumor unresectable or
resected with gross residual tumor, or
positive lymph nodes.
IV Distant metastases regardless of the extent of
liver involvement.
diagnosed with stage I and II HBL have a cure rate of
greater than 90%, whereas stage III is associated with a
60% survival rate. Children with stage IV disease have a
survival rate of roughly 20%. Children diagnosed with
stage I HCC generally have a good outcome. Stage II is
too rarely seen to predict outcome, and stages III and IV
are usually fatal.
Complete resection of the primary tumor with negative
surgical margins is one of the most critical factors in
prognosis. Preoperative chemotherapy can convert an
unresectable tumor into one that is resectable and may
lessen the incidence of postoperative morbidity.55 Preop-
erative chemotherapy is more effective in the treatment
of HBL than HCC. Surgical resection of distant disease
has also contributed to the cure of children with hepa-
toblastoma. Resection of pulmonary metastases is recom-
mended when the number of metastases is limited. Liver
transplantation may be useful therapy in patients with
unresectable hepatic tumors.56 Five-year survival rates
approximating 83% for children with HBL and 63% for
children with HCC have been reported. Because of the
worse prognosis in patients with HCC, liver transplanta-
tion is considered early in the course for disorders such
as tyrosinemia and familial intrahepatic cholestasis before
the development of liver failure and malignancy. The
fibrolamellar variant of HCC may have a better prognosis
with liver transplantation than other types.
Teratoma
Teratomas are tumors that contain elements derived from
more than one of the three embryonic germ layers. In
addition, teratomas must contain tissue that is foreign to
the anatomic site in which they occur. Teratomas can
occur anywhere in the body and present as cystic, solid,
or mixed lesions. When they occur during infancy and
early childhood, they are most commonly extragonadal.
In contrast, in older children, teratomas most frequently
involve the gonads.
Teratomas occur most frequently in the neonatal
period, and the sacrococcygeal region is the most common
site. Sacrococcygeal teratoma (SCT) is four times more
common in females and is most often an obvious external
8/28/2007 11:38:00 AM
 2086 Section XIII Specialties in General Surgery
Figure 71-23 Sacrococcygeal teratoma. Despite its large size, this
tumor is benign in most cases.
presacral mass (Fig. 71-23). Although most of the tumor
is usually external, with a minimal intrapelvic presacral
component, there is a spectrum of tumor distribution, to
the extent of being entirely presacral, with no visible
external component. As such, a digital rectal examination
of a neonate with care to feel the normal presacral space
may be an important screening technique. Occasionally,
SCTs are identified during routine prenatal ultrasonogra-
phy. It is important that these lesions be carefully fol-
lowed with serial sonography until delivery because the
blood supply to the tumor may grow to the point of
stealing a significant proportion of placental blood flow
to the fetus. The development of hydrops or placento-
megaly is associated with a poor prognosis. In these
situations, in utero resection of the tumor may be
lifesaving.57
Most neonatal SCTs are benign. The incidence of
malignancy is related to age at time of diagnosis and is
most frequently represented as endodermal sinus tumors
(yolk sac tumors) or embryonal carcinomas. The pres-
ence of trophoblasts is associated with choriocarcinoma.
The likelihood of malignancy is slightly increased in
males.
Ch071-X3675.indd 2086
Treatment of SCT is complete surgical excision through
a chevron-shaped buttock incision. Most tumors can be
completely removed using a sacral approach. If preop-
erative imaging demonstrates significant intra-abdominal
extension of the tumor, a combined abdominal-sacral
approach may be needed. Resection of the coccyx is
critical because failure to remove this structure results in
significantly higher local recurrence rates. Care must be
taken to individually ligate the vessels supplying the
tumor, including the middle sacral artery and branches
of the hypogastric arteries. After the tumor is excised, the
levator muscle complex is secured to the presacral fascia,
and the remaining wound is closed in layers. Careful
follow-up is necessary because recurrence may be sig-
nificant, even for benign tumors.
FETAL SURGERY
The motivation for fetal surgical intervention has been
the realization that with certain congenital anomalies,
irreversible changes have already occurred by the time
of birth. In utero intervention therefore could theoreti-
cally prevent continued progression of the anomaly,
reverse the pathophysiology, and prevent fetal demise.
At present, there are a few indications for open fetal
surgery, which needs to be performed only at centers
with multidisciplinary expertise in this area. As with open
surgery in general, the indications for minimally invasive
fetal surgical intervention are expanding.
Fetal Imaging
Ultrasonography remains the mainstay of imaging modal-
ities for the fetus. It is rapid, noninvasive, and applied in
most pregnancies. Although three-dimensional ultrasound
has added little to what can be seen by conventional
ultrasound, four-dimensional ultrasound may provide
tomographic assessment of the fetus in three dimensions,
which may significantly enhance diagnostic capabilities.
Ultrafast MRI is complementary to ultrasound imaging. In
particular, MRI has proved superior in the assessment of
central nervous system lesions.
Open Fetal Surgery
One of the major conditions that have been systemati-
cally evaluated with regard to open fetal surgery has been
congenital diaphragmatic hernia. Initial attempts at in
utero repair demonstrated some success, but outcomes
were no different for low-risk patients when compared
with contemporary postnatal management. In high-risk
patients (liver up in the chest, a right lung-to-head cir-
cumference ratio [LHR] < 1.4), open fetal surgery was
associated with poor survival, primarily because of
kinking of the hepatic veins and IVC when the liver was
moved into the abdomen. At present, open fetal surgery
for congenital diaphragmatic hernia has been abandoned.
Occlusion of the fetal trachea by either external clips or
intraluminal balloons has been shown to induce lung
growth; however, outcomes have not been significantly
improved.39
8/28/2007 11:38:00 AM

Although congenital cystic adenomatoid malformation
often presents as a benign pulmonary mass in infants and
children, a few fetuses have extremely large lesions asso-
ciated with the development of hydrops fetalis. Without
intervention, these infants die in utero. Careful ultrasound
surveillance of large lesions is therefore necessary to
detect the first signs of hydrops. Fetal pulmonary resec-
tion has been successful in preventing this complication.
For lesions consisting of a single large cyst, thoracoam-
niotic shunting may be useful.
Another condition in which open fetal surgical inter-
vention has been valuable is sacrococcygeal teratoma. In
large teratomas, increased blood flow to the tumor is
associated with high-output heart failure in the fetus
(hydrops) as well as placentomegaly. The development
of these two findings is associated with dismal survival.
Open fetal resection of the teratoma has been reported
in a few cases to reverse the hydrops and to salvage the
fetus.
Although it has been initially assumed that the spinal
cord in infants with myelomeningocele is intrinsically
abnormal, recent work has suggested that much of the
neurologic damage may be due to in utero exposure and
trauma to the spinal cord. At present, a randomized pro-
spective multicenter trial is underway to determine the
potential benefit of in utero repair on need for postnatal
ventricular decompressive shunting and neurocognitive
development.
Fetal Endoscopic Surgery
With the explosion of minimally invasive technology, the
applications to fetal surgical intervention are also expand-
ing. Fetal urethral obstruction is most commonly due to
posterior urethral valves in males and ureteral atresia in
females. Urethral obstruction results in oligohydramnios,
pulmonary hypoplasia, and renal dysplasia. In fetuses
with a good prognosis (fetal urine Na+ < 100 mEq/L,
Cl− < 90 mEq/L, and osmolarity <210 mOsm/L), percuta-
neous vesicoamniotic shunt or fetocystoscopic ablation
of urethral valves may restore amniotic fluid volume and
prevent death from pulmonary hypoplasia. Whether
these approaches can preserve renal function is less
clear.
Fetoscopic intervention appears to have had the great-
est impact on complications associated with monochori-
onic (single placenta) twins. In cases in which there is
spontaneous or imminent demise of one twin with lethal
anomalies, death of the normal twin may rapidly follow.
In this scenario, fetoscopic ligation of the cord to the
anomalous twin prevents deterioration of the normal
twin. Twin reversed arterial perfusion (TRAP) sequence
is a rare situation in which one twin is acardiac and
acephalic. Without intrinsic cardiac function, blood flow
thorough this fetus is entirely drained from the normal
heart of the so-called pump twin, resulting in cardiac
failure and death. Diathermic occlusion or radiofrequency
ablation of the umbilical cord going to the acardiac and
acephalic twin will therefore halt the progression of
cardiac failure in the opposite twin. Finally, the single
most commonly performed fetal surgical procedure is
Ch071-X3675.indd 2087
Chapter 71 Pediatric Surgery 2087
fetoscopic photocoagulation of communicating placental
blood vessels in twin-twin transfusion syndrome. This
intervention will arrest the development of a steal phe-
nomenon of blood flow from one twin to the other. A
comparison of this method with the standard treatment
of serial amnioreduction for polyhydramnios in hope of
prolonging the pregnancy has just been completed.
Ex Utero Intrapartum Treatment
Lesions that compromise the fetal airway constitute an
immediate threat at the time of delivery for hypoxia, isch-
emic brain injury, and death. These include large cervical
teratomas, vascular malformations, and laryngeal atresia.
As such, a strategy known as the EXIT procedure has
converted these potentially catastrophic events into a
more controlled and safe procedure for establishment of
an airway. The basic premise of the EXIT procedure is
controlled uterine hypotonia to facilitate uteroplacental
circulation. Thus, appropriate time can be taken to secure
a safe airway just before clamping the umbilical cord and
delivery of the fetus. In contrast with a typical cesarean
birth in which uterine tone is maximized to prevent post-
partum hemorrhage and minimal inhalation agents are
used for fear of neonatal depression, the EXIT procedure
employs deep inhalation anesthesia and tocolytic agents
to maximize uterine relaxation, uterine volume is pre-
served to prevent placental abruption, and a surgical level
of anesthesia is achieved in the fetus. During this time,
endotracheal intubation can be achieved. For large lesions,
division of the strap muscles of the neck, subtotal tumor
resection, or tracheotomy may be needed before cord
clamping. The congenital high airway obstruction syn-
drome (CHAOS) is associated with hydrops in which
nearly complete or complete intrinsic obstruction of the
fetal airway prevents egress of lung fluid from the tracheo-
bronchial tree. These children may benefit from an EXIT
strategy. Further, the EXIT procedure may be applied
during the transition to ECLS in cases of known severe
cardiac anomalies as a bridge to postnatal intervention.
Selected References
Ashcraft KW, Holcomb GW III, Murphy JP (eds): Pediatric
Surgery (4th ed). Philadelphia, Elsevier/Saunders, 2005.
This is an excellent reference source for most pediatric surgical condi-
tions. Although most topics are not covered as in-depth as in the Gros-
feld text mentioned next, this book is easy to read and serves as an
outstanding practical resource.
Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran AG (eds): Pedi-
atric Surgery (6th ed). Philadelphia, Mosby Elsevier, 2006.
This two-volume monograph provides a very comprehensive review of
the field of pediatric surgery. This book is considered to be the most
authoritative and traditional textbook for pediatric surgeons.
Mattei P (ed): Surgical Directives: Pediatric Surgery. Philadel-
phia, Lippincott Williams & Wilkins, 2003.
This is an outstanding reference text for senior surgery residents,
fellows, and general surgeons. The chapters are short, to the point, and
written to provide a reasonable approach to most of the common pedi-
atric surgery conditions.
8/28/2007 11:38:02 AM
 2088 Section XIII Specialties in General Surgery
Oldham KT, Colombani PM, Foglia RP, Skinner MA (eds): Prin-
ciples and Practice of Pediatric Surgery. Philadelphia, Lippincott
Williams & Wilkins, 2005.
This is a superb reference book that tends to integrate more patho-
physiology and basic science discussions of pediatric surgical
conditions.
Ziegler MM, Azizkhan RG, Weber TR (eds): Operative Pediatric
Surgery. New York, McGraw-Hill, 2003.
This is a beautifully illustrated text that emphasizes various pediatric
surgical techniques. This is especially valuable for review of the more
commonly done pediatric surgery procedures.
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