PEDIATRIC EMERGENCIES 0889-8537/01 $15.00 + .

OO

NEONATAL SURGICAL
EMERGENCIES
Letty M. P. Liu, MD, and Leila Mei Pang, MD

Advances in perinatology and neonatology over the last 2 decades

have improved the survival of premature, especially extremely prema-
ture (< 1500 g) and critically ill, newborn babies. Most of the disorders
that were considered neonatal surgical emergencies in the past no longer
require immediate surgery because of new technology and new methods
of treating sick neonates. This article describes the more common neona-
tal disorders that require semi-elective or emergency surgery, and fo-
cuses on factors that affect the anesthetic management of patients with
these disorders.

PYLORIC STENOSIS

Pyloric stenosis is the most common gastrointestinal obstructive

anomaly in neonates. It occurs in approximately 1in 500 live births, and
is found more frequently in males. Symptoms are usually not apparent
until the second to sixth week of life, and are caused by hypertrophy of
the muscular layer of the pylorus. The affected area feels like an olive-
shaped mass. It is palpated most easily when the stomach is empty, and
is located slightly to the right of the midline in the epigastric area. A
barium swallow roentgenogram or ultrasonogram can confirm the le-
ion.^^
When an infant is suspected of having pyloric stenosis, oral feedings

From the Department of Anesthesiology, University of Medicine and Dentistry of New

Jersey-New Jersey Medical School, Newark, New Jersey; Department of Anesthesiol-
ogy and Pediatrics, Columbia University, College of Physicians and Surgeons of
Columbia University; and New York-Presbyterian Hospital, Columbia-Presbyterian
Medical Center, New York, New York

ANESTHESIOLOGY CLINICS OF NORTH AMERICA

VOLUME 19 NUMBER 2 * JUNE 2001 265

266 LIU&PANG

should be discontinued. Metabolic derangements associated with pyloric

stenosis result from persistent vomiting, with loss of water, hydrogen,
chloride, sodium, and potassium. As hydrogen ions are lost, the kidneys
initially secrete potassium in exchange for hydrogen to maintain a nor-
mal serum pH. When sodium depletion becomes more severe, the kid-
neys secrete potassium and hydrogen in exchange for sodium, which
results in a patient who is hypokalemic, hypochloremic, and alkalotic.'03
If electrolyte loss continues, the kidneys secrete an acid urine (i.e.,
paradoxic acidosis), and metabolic alkalosis becomes more profound.
Eventually, metabolic acidosis from dehydration and hypoperfusion
occurs.

Anesthetic Management

' Surgical correction of pyloric stenosis, pyloromyotomy, is not a

procedure that should be performed emergently if the patient is dehy-
drated or is depleted of electrolytes. Fluid, electrolyte, and acid-base
balance should be re-established before anesthetizing a neonate with
pyloric stenosis, so that perianesthetic complications can be avoided. For
example, if a patient is still alkalotic postoperatively, hypoxia can occur
as the baby attempts to correct the alkalotic state by hypoventilating.
Mild-to-moderate fluid and electrolyte problems (chloride > 90 mEq/L
and urine specific gravity < 1.020) can be corrected with 5% dextrose in
0.45% sodium chloride and 20 to 40 mEq/L of potassium, infused at a
rate of 10 mL/kg per hour. Severe dehydration and electrolyte imbalance
(chloride < 90 mEq/t, sodium < 120 mEq/L, and no urine output)
requires volume expansion with isotonic sodium chloride or colloid and
blood until urinary output is re-established. Potassium chloride then can
be added to the infusion. In severe cases of dehydration, restoring fluid,
acid-base, and electrolyte balance may take several days.
Immediately before the induction of anesthesia, standard monitor-
ing (e.g., blood pressure cuff, ECG leads, pulse oximeter probe, and
precordial stethoscope) should be started, and a large-bore orogastric
tube should be passed into the stomach several times until the amount
of fluid aspirated is minimal. Endotracheal intubation after preoxygen-
ation and a rapid-sequence induction is the method many anesthesiolo-
gists prefer for inducing anesthesia in these babies. Induction agents and
doses for these patients are found in Table 1. If difficulty with intubation
is anticipated, an endotracheal tube should be placed to secure the
airway before anesthesia is induced. An inhalation induction of anesthe-
sia should be avoided in these patients because they have an increased
risk for vomiting and aspirating gastric contents. Even after multiple
attempts to remove gastric contents by suction, a considerable amount
of residual material still may be present. Anesthesia may be maintained
with inhalation anesthetics or in combination with intravenous drugs.
Any volatile anesthetic drug may be used for anesthesia; however, the
same inspired concentration of volatile anesthetic generally causes a
 NEONATAL SURGICAL EMERGENCIES 267

Table 1. DRUGS AND INTRAVENOUS DOSE

Drugs Dose
Anticholinergic drugs
Atropine 0.01-0.02 mg/kg
Glycopyrrolate 0.005-0.01 mg/kg
Anesthetic induction drugs
Thiopental 6-7 mg/kg
Propofol 2.5-3 mg/kg
Etomidate 0.3 mg/kg
Muscle relaxants
Depolarizing
Succinylcholine 1-2 mg/kg
Nondepolarizing
Cisatracurium 0.2 mg/kg
Rocuronium O.&l mg/kg
Vecuronium 0.1 mg/kg
Pressors
Catecholamines
Dopamine 3-5 kg/kg/min

higher incidence of cardiovascular instability in infants, compared with

older patients. This effect can be attributed to several factors, including
faster equilibration, rapid myocardial and brain uptake, increased anes-
thetic requirements, and sensitivity of the immature myocardium to
volatile agents (especially halothane). These factors, the decrease in
myocardial contractile mass, and less magnitude and velocity of fiber
shortening produce the adverse cardiovascular effects. Volatile agents
also depress the baroreceptor reflexes in a concentration-dependent man-
ner in the younger compared with the older patient.76,77, Io8
Before terminating anesthesia, measures to minimize postoperative
pain should be instituted. These include rectal acetaminophen, (3040
mg/kg),15 caudal analgesia, and infiltration of the surgical incision with
a local anesthetic (e.g., 0.25% bupivacaine) before abdominal closure.
After the patient is awake and ventilating adequately, the endotracheal
tube can be removed.
Postoperatively, the child may exhibit signs of drowsiness or leth-
argy. Respiratory depression (e.g., apnea) also may be p r e ~ e n t If. ~total
body potassium is decreased (which may occur despite a normal serum
potassium), there may be altered electrical forces across cell membranes.
This causes an unpredictable response (i.e., potentiation) to nondepolar-
izing neuromuscular blocking agents and weakening of the muscles,
including the muscles of respiration, creating a need for postoperative
mechanical ventilation. If hypokalemia is severe, rhabdomyolysis and
impairment of smooth muscle function can occur. Bladder dysfunction,
ileus formation from lack of peristalsis, poor peripheral vascular tone
with orthostatic hypotension, and a poor pressor response to catechola-
mines are possible. Cardiac dysrhythmias also may be present because
268 LIU&PANG

hypokalemia enhances the intrinsic automaticity, sustains dysrhythmias,

and alters cond~ctivity.~~

CONGENITAL DIAPHRAGMATIC HERNIA

Approximately 1 in 2500 babies is born with a congenital diaphrag-

matic hernia.85The disorder is characterized by failure of the pleural and
peritoneal canal to close at approximately the eighth week of gestation,
resulting in herniation of the abdominal viscera into the thoracic cavity
and pulmonary hypoplasia from compression by the viscera on the
developing lungs. Hernias through the posterolateral portions of the
diaphragm (Foramen of Bochdalek) account for approximately 80% of
diaphragmatic hernias; herniation on the left side is five times more
common than on the right side. These Bochdalek hernias are the largest
diaphragmatic hernias, and pulmonary hypoplasia is more extensive in
these patients. Hernias through the anterior part of the diaphragm
(Foramen of Morgandi) account for only 2% of all diaphragmatic hernias;
esophageal hiatus hernias account for 18%.
The prevalence of posterolateral hernias may be related to the devel-
opment of the diaphragm. During the second month of gestation, a
pleuroperitoneal membrane is formed. This membrane eventually di-
vides the common pleuroperitoneal cavity into two compartments, the
pleural cavity and the peritoneal cavity. The last areas of the membrane
to develop are the posterolateral portions, with the right side closing
before the left. At approximately the ninth gestational week, the gut,
which was in the yoke sac, migrates to the peritoneal cavity. If this
migration occurs before the pleuroperitoneal membrane closes off the
pleural cavity, the stomach, spleen, liver, and bowel can migrate into the
pleural cavity and compress the developing lungs. Compression of the
lungs affects their growth. Although the lung on the side of the hernia
is more severely affected, the lung on the contralateral side is also
hypoplastic. Severe pulmonary hypoplasia with arrest of bronchial and
bronchiolar development occurs if a large volume of vicera is present in
the pleural cavity before the 12th week of gestation.66Compression of
the lungs, especially when airways are dividing and pulmonary arteries
are forming, causes a decrease in lung volume, number of bronchi and
alveoli, and cross-sectional area of pulmonary arterial branches.66The
pulmonary artery is smaller, arterial branching is diminished, and the
arteries are more muscular. Although significant lung growth at the
alveolar level and remodeling of the vasculature (i.e., in larger, less
muscular arteries) occurs after birth, the time this takes exceeds the
current limitation of supportive measures, such as extracorporeal mem-
brane oxygenation.1°Severe impairment of normal pulmonary develop-
ment often results in a critically ill neonate. Persistent pulmonary hyper-
tension with increased vascula; resistance frequently occurs; in many
cases, these conditions lead to hypoxemia, acidosis, and death.39,45, 66
The results of recent animal models have challenged current think-
 NEONATAL SURGICAL EMERGENCIES 269

ing regarding the cause of congenital diaphragmatic hernia. When the

drug nitrofen (2,2 dichloro-phenyl-p-nitrophenyl ether) was used, the left
lung failed to develop, leading to a left diaphragmatic hernia. Congenital
diaphragmatic hernia may be secondary to the failure of normal lung
development rather than being the cause of
With the increased use of prenatal ultrasonography, prenatal diagno-
sis of congenital diaphragmatic hernia has become more common.', 47, 56
Neonates with congenital diaphragmatic hernias usually have scaphoid
abdomens and barrel chests. Children who are in respiratory failure
within the first 6 hours of life have the highest mortality, whereas those
diagnosed later generally have a good prognosis.s6,111 Approximately
one third of the babies who are symptomatic in the first 6 hours of life
have an associated anomaly, with cardiac lesions pred~minating.~~, 40
The babies with associated anomalies, especially those with cardiac
anomalies, have significantly lower APGAR scores. Their best postductal
Poz before extracorporeal membrane oxygenation or surgery is lower
than that in those children with hernia with no other anomalies.40The
literature indicates that a sustained alveolar-to-arterial oxygen tension
gradient of greater than 600 mm Hg and an oxygenation index over 40
for 8 to 12 hours still are associated with mortality.99Some clinicians
suggest that the persistence of a postductal Pao2below 100 mm Hg and
of a Paco2 above 40 mm Hg despite optimal conventional therapy
indicates poor prognosis.
Data extrapolated from Boix-Ochoals and Bohn16,l7 indicate that
some babies who have a persistently low postductal Pao2 should have
their preductal Paoz measured because it is a directly measured physio-
logic value. An infant with congenital diaphragmatic hernia with a
persistent Paoz below or who cannot generate a preductal Pao2 of
at least 100 mm Hg at some point has pulmonary hypoplasia to a degree
incompatible with life despite extracorporeal life support (ECM0).99
Before Ladd and Gross's series in 1940 of long-term survivors,
operative repair of neonates with congenital diaphragmatic hernia was
considered impossible.68For years later, the survival rate of these pa-
tients remained at approximately 50%.50,75, 91 Despite improvements in
the clinical management of neonates, diagnostic tools, and surgical tech-
niques, the multicenter Congenital Diaphragmatic Hernia Study Group
reported, in 1998, a survival rate of only 63Y0.*~
In the past, babies with congenital diaphragmatic hernia were
rushed to the operating room immediately after birth, without taking
time for stabilization. The change in practice to delay surgery until the
respiratory problems are stabilized reflects the present belief that surgery
cannot correct respiratory insufficiency. The primary problem in these
babies is not respiratory failure from lung compression by the herniated
viscera but respiratory failure from severe pulmonary hypoplasia and
pulmonary hypertension.* Currently, neonates with congenital diaphrag-
matic hernia are managed medically until their respiratory and cardio-

*References 6, 13, 17, 19, 28, 54, 59, 72, 93, and 107.
270 LIU&PANG

vascular statuses are ~tabilized.4~. 51, 88, 96, lo4, lo9 Only after they are
optimized are they scheduled for semi-elective surgery.
Because pulmonary hypoplasia and associated anomalies are the
leading causes of death in neonates with congenital diaphragmatic her-
nia, treatments now are directed at correcting problems caused by these
conditions, with concurrent improvement in outcome. Lung rupture
from barotrauma easily can occur at any time during the perioperative
period. Ventilatory strategies that minimize airway pressure and reduce
barotrauma to the severely hypoplastic lungs improve survival. Gentle
ventilation that allows arterial CO, to be slightly higher than normal
(i.e., permissive hypercapnia) and marginal postductal oxygen saturation
are associated with a higher survival rate than conventional ventilation
with or without extracorporeal membrane 60, 74
41,

High-frequency ventilation has been advocated to reduce baro-

trauma and to produce alkalosis to improve pulmonary blood flow. This
artificial ventilation method can be used before, during, and after surgi-
cal repair.20,35, 74, 96 The major disadvantage of using high-frequency
ventilation during anesthesia is that end-tidal CO, is difficult to monitor.
Frequent monitoring of end-tidal CO, and O2 saturation and arterial
blood gas determinations are essential to ensure effective alveolar venti-
lation and oxygenation. If CO, tension is allowed to rise too high,
pulmonary vascular resistance, which is generally high, increases further.
A pneumothorax should be considered if sudden unexplained hypo-
tension or desaturation occurs. A pneumothorax that is not treated
promptly can lead to significant hypotension and hypoxia. A pneumo-
thorax on the contralateral side can affect ventilation more significantly
than one on the same side as the hernia, because the lung on the
contralateral side is generally the better developed.'15 In patients with
chest tubes, an increase in lung compliance and abdominal distension
may be caused by a pneumoperitoneum. If the air leak into the chest is
large and the chest tube cannot evacuate the gas from the thorax ade-
quately, gas could flow through the diaphragmatic defect into the perito-
neal cavity. Significant abdominal distension can result, causing dimin-
ished lung expansion and hypoxia. In this case, decompressing the
abdomen with a large-bore needle allows the gas to escape so the lungs
can expand.
New modes of therapy have led to some improvement in the sur-
vival rate of neonates with congenital diaphragmatic hernia. Extracor-
poreal membrane oxygenation is one treatment, that has improved
the survival rate of infants who originally had a predicted mortal-
ity of 80%.'01 Inhaled nitric oxide, a selective pulmonary vasodilator,
does not improve survival of neonates with congenital diaphragmatic
hernia.lo1The efficacy of surfactant replacement therapy in these pa-
tients is currently unclear.96 Further discussion of these treatment
modalities is beyond the scope of this article, but can be found in the
literature.21,23,43,69-71,82. 83
Surgical repair of congenital diaphragmatic hernia in neonates can
be performed through an abdominal incision, but some surgeons prefer
 NEONATAL SURGICAL EMERGENCIES 271

a thoracoabdominal approach. Small defects are closed by suturing

existing diaphragmatic tissue together. With large defects, an artificial
diaphragm is constructed. If the artificial diaphragm material is not
permeable to gases, abdominal distension from a pneumothorax is mini-
mized and the need for a peritoneal drain is reduced.37Occasionally,
abdominal closure may not be possible without compromising ventila-
tion. In these cases, an abdominal silo is created to house the viscera
until the abdominal wall stretches enough to allow abdominal closure
at a later stage.

Anesthetic Management

The anesthetic management of neonates with congenital diaphrag-

matic hernia involves a thorough assessment of the child. Management
of these patients depends on their physiologic condition and anatomic
pathology. A review of laboratory, radiographic, and physical findings
and optimization of any anomalies should be completed before anesthe-
sia is administered. Listening to breath sounds beforehand and knowing
whether the hernia is on the left or right side are important. For example,
a decrease in breath sounds over the right chest in a child with a right-
sided hernia may not be caused by the intubation of the left main stem
bronchus.
In the past, babies with congenital diaphragmatic hernia were oper-
ated on immediately after birth. Surgery now is delayed until the pa-
~ Consequently, many babies already
tients are medically stabilized.8,l l 112
are intubated, and have indwelling intravenous and arterial lines in
place by the time they arrive at the operating room. Children who are
not intubated and mechanically ventilated before arriving at the op-
erating room generally fare best if they are nursed in a semirecumbent
position with their affected side down. An orogastric or nasogastric tube
often is inserted to decrease and prevent distension of the stomach and
intestines. Neonates who are not intubated before arrival at the operating
room generally are intubated awake or after a rapid-sequence induction
(see Table 1). An anticholinergic drug, such as atropine, 0.02 mg/kg
administered intravenously, is often the only drug used for premedica-
tion. This drug is administered immediately before the induction of
anesthesia to prevent bradycardia and a drop in cardiac output during
induction. If the baby is stable and an awake intubation is planned,
analgesics may be administered to decrease the stress of airway instru-
mentation. Although an inhalation induction of anesthesia in a spontane-
ously breathing child is possible, assisted or controlled ventilation gener-
ally is required to ventilate the child adequately before a satisfactory
level of anesthesia is achieved for laryngoscopy and intubation. If the
stomach is ih the chest, mask ventilation with high inflation pressures
should be avoided to prevent gastric distension. High inflation pressures
also should be avoided because many of these children develop lower
272 LIU&PANG

esophageal sphincter incompetence and are at risk for reflux and aspira-
tion of gastric content.80
The choice of drugs for anesthesia should be determined by the
patient's general condition. In infants and children with small veins,
pentothal is preferred to propofol because of the high incidence of pain
when propofol is administered into small veins. High doses of narcotics
(e.g., fentanyl, 15-25 p,g/kg) can be used if hemodynamically tolerated.
Nitrous oxide should be avoided because it defuses into the bowel
lumen, causing further bowel expansion, and may compromise lung
expansion, especially if the intestines are in the chest. The risk and
benefits of the various inhalation anesthetics should be considered before
deciding on their use. Compared with other volatile anesthetics, halo-
thane produces a greater decrease in inotropy and chronotropy of the
heart at equipotent doses.
Vigilant monitoring is mandatory in neonates with congenital dia-
phragmatic hernias. Monitoring blood pressure, ECG, pulse oximetry,
capnography, temperature, and heart rate is essential during anesthesia
for all babies with congenital diaphragmatic hernias. Following preduc-
tal and postductal oxygen saturation with pulse oximeter and probes on
the right upper extremity and on a lower extremity allows early detec-
tion of right-to-left shunting. Although invasive monitoring is not neces-
sary in all patients, it may be indicated in those who are in respiratory
distress. Cannulation of the right radial artery will allow measurement
of preductal blood gases. In addition to providing continuous blood
pressure readings, preductal blood gases can be measured. Conditions
that can cause pulmonary vasoconstriction, such as hypoxia, acidosis,
and hypothermia, should be avoided. If possible, oxygen saturation
should be kept above 80 mm Hg, and CO, should be kept within normal
or only slightly above the normal range (i.e., permissive hypercapnia).
Metabolic acidosis should be treated immediately, and any significant
blood loss should be replaced.
Familiarity with the different therapeutic modalities that may be
needed during anesthesia is essential to provide optimal anesthetic care.
Understanding the principles of high-frequency ventilation and ECMO
is essential. Occasionally, the hernia is repaired while a child is still on
ECMO, despite hemodynamic and pulmonary in~tability.~~ For these
patients, an opioid and a nondepolarizing muscle relaxant may cause
less hemodynamic instability than an inhalation anesthetic. A volatile
anesthetic agent, such as isoflurane, can be used for these patients by
administering it through the EMCO circuit (Fig. l).7For children on
ECMO, drugs may be administered directly to them, or into the ECMO
circuit. Because patients on ECMO are heparinized, intraoperative bleed-
ing can be problematic because hemostasis is abnormal.1o6

ESOPHAGEAL ATRESIA AND

TRACHEOESOPHAGEAL FISTULA
Approximately 1 in 4000 babies are born with esophageal atresia,
and 90% of them have an associated tracheoesophageal fistula (i.e.,
h) Figure 1. lsoflurane during extracorporeal membrane oxygenation.
v
w
274 LIU&PANG

cardiac, gastrointestinal, genitourinary, musculoskeletal, or craniofacial

anomalies) are present in 30% to 50% of newborns with esophageal
atresia and tracheoesophageal fistulas.87,89, 92, Io2 One form of the associ-
ated anomalies is the VATER (i.e., vertebral and vascular anomalies,
imperforate unus, tracheoesophageal fistula, radial aplasia, and renal
abnormalities) or VACTERL (i.e., vertebral anomalies, imperforate unus,
cardiac anomalies, tracheoesophageal fistula, renal abnormalities, radial
limb aplasia) association.
The diagnosis of esophageal atresia is usually made when a tube
cannot be passed into the newborn’s stomach after delivery, after the
first feeding when coughing and choking occurs, or after recurrent
pneumonia associated with feedings. Prenatally, the diagnosis should be
considered if polyhydramnios is present. After birth, the diagnosis is
confirmed by the inability to pass an orogastric tube into the stomach.
Location of the atretic segment is confirmed by a radiograph showing
the tip of a radiopaque catheter that is passed into the esophagus until
it stops. Air in the stomach and intestines usually is seen in abdominal
radiographs of neonates with tracheoesophageal fistula. The absence of
air usually indicates esophageal atresia without a fistula. Occasionally,
the diagnosis of a tracheoesophageal fistula is not made until later in
the child’s life.
Although Thomas Gibson first described esophageal atresia in 1697,
no baby survived until Dr. Logan Levin performed the first successful
staged repair in 1939.79Four years later, the first primary repair was
performed. Because of subsequent advances in neonatal and periopera-
tive care, nearly all babies survive. The exceptions are those who are
very premature (< 1500 g) and those who have severe cardiac or chro-
mosomal anomalies.4,11, 12. 24.26. 92.98
The most common form of esophageal atresia and tracheoesopha-
geal fistula (80%-90% of patients) is an upper esophagus ending in a
blind pouch and the distal esophagus forming a tracheoesophageal
fistula.38,67 In most cases, anastomosis of the two ends of the esophagus
is possible. The challenge occurs when a long segment of atresia is
present. A staged repair with traction, myotomies, or multiple esopha-
gotomies on the segments is possible in some cases.34, In others, a
portion of the colon or jejunum or a tube made from the stomach was
interposed.2,3, 32, Gastric interposition is another procedure.s4Although
survival is improved with these interposition procedures, the mortality
rate in these patients is high.
Morbidity and mortality in neonates with esophageal atresia and
tracheoesophageal fistula generally are related to pulmonary complica-
33, ,90, 97 The goal of preoperative management should be to
t i o n ~ .26,~ ~
minimize these complications. Once the disorder is diagnosed, attempts
should be made to prevent aspiration pneumonitis. Oral feedings should
not be given, and the baby should be nursed in the semirecumbent
position to minimize the possibility of aspiration of gastric and nasopha-
ryngeal contents. A tube should be passed into the esophagus and
suctioned frequently to minimize the accumulation of nasopharyngeal
 NEONATAL SURGICAL EMERGENCIES 275

secretions in the blind pouch. For an extremely premature or critically

ill infant, a staged approach should be considered. After the diagnosis
is made, a gastrostomy is placed to prevent excess gastric distension
from impairing diaphragmatic excursion, and definitive repair is post-
poned until the baby is more stable.4,52

Anesthetic Management

Atropine generally is the only drug used for premedication of

infants with esophageal atresia and tracheoesophageal fistula. It is given
to decrease the incidence of bradycardia from vagal stimulation during
intubation and to maintain cardiac output during the induction of anes-
thesia. Monitors should include devices to measure blood pressure, ECG,
heart rate, temperature, pulse oximetry, and capnography. A precordial
stethoscope placed on the left chest under the axilla allows monitoring
of heart and breath sounds.
If a difficult intubation is anticipated, if gastric distension is present,
or if the baby is vomiting, endotracheal intubation should be performed
while the child is still awake. Otherwise, intubation usually is performed
after a rapid-sequence induction of anesthesia (see Table 1).If an inhala-
tion induction of anesthesia is chosen before the endotracheal tube is
placed distension of the stomach will be minimized if the child is
allowed to breathe spontaneously. To prevent gas from transiting
through the fistula and distending the stomach when ventilation is
assisted or controlled before the endotracheal tube is inserted airway
pressure should be minimized. Because a tracheoesophageal fistula gen-
erally is located on the posterior aspect of the trachea and just proximal
to the carina, placing the endotracheal tube into a bronchus and with-
drawing it until breath sounds are equal may prevent ventilation of the
fistula.94Rotating the endotracheal tube during intubation so the bevel
faces posteriorly may prevent intubation of the fistula. After the endotra-
cheal tube is placed, positive-pressure ventilation should be instituted,
and the size of the stomach should be assessed before administering a
muscle relaxant. If gastric distension becomes a problem, it may be
necessary to allow the child to breathe spontaneously until a gastrostomy
tube is placed or until the surgeon can obstruct the fistula manually.
After a gastrostomy, ventilating the patient adequately may be difficult
if the volume of gas flowing through the fistula is high. In these cases,
a balloon Fogarty catheter placed in the fistula through a bronchoscope
or insertion of this catheter under fluoroscopic guidance through the
gastrostomy into the fistula will occlude the fistula and allow for easier
ventilation of the 58
In most cases, the esophagus is anastomosed primarily, and the
fistula is ligated through a right thoracotomy. During surgery, airway
obstruction can occur if the trachea is compressed, secretions become
inspisated or a blood clot forms in the airway. The surgeon must be
276 LIU&PANG

informed of the problem so that he or she can correct the situation or

help change the endotracheal tube.
At the end of the surgical procedure, most infants are extubated if
they are awake and are not at risk for postoperative respiratory prob-
lems. Those who are at risk for respiratory problems (e.g., postanesthetic
apnea3',73, ]lo) should remain intubated until they demonstrate that they
can sustain adequate ventilation postoperatively. Premature extubation
also may be dangerous in these babies because the recently closed fistula
may be ruptured by the endotracheal tube if it needs to be reinserted.
Neonates with a tracheoesophageal fistula frequently have a reduc-
48, Io5 In babies with tra-
tion in tracheal cartilage (i.e., tra~heomalacia).3~,
cheomalacia, ventilation may be adequate immediately after extubation.
In some patients, ventilatory problems may not be apparent until later in
the postoperative period. Dynamic collapse of the trachea during inspira-
tion, map be magnified as the child's respiratory efforts increase. Tra-
cheopexy may be necessary, or a tracheotomy may be required.22,30, 65, m

OMPHALOCELE AND GASTROSCHISIS

Omphalocele and gastroschisis are abdominal wall defects that can

be diagnosed by fetal ultrasound in the first t r i m e ~ t e r .Salient
~ , ~ ~ features
of these conditions are shown in Table 2. Omphalocele occurs in 1 in
6000 births, and is caused by a failure of the gut to migrate from
the yolk sac to the abdomen during gestation.36,62 Neonates with an
omphalocele have a high incidence of other anomalies, and frequently
are premature at birth. Associated anomalies include-cardiac,gastrointes-
tinal (e.g., malrotation, Meckel's diverticulum, intestinal atresia), geni-
tourinary (e.g., extrophy of the bladder), metabolic (e.g., Beckwith-
Wiedemann syndrome: macroglossia, hypoglycemia, organomegaly,
gigantism), and chromosomal abnormalities. Cardiac and thoracic de-
fects are more frequent in neonates with epigastric omphaloceles,
whereas cloaca1 anomalies and extrophy of the bladder more frequently
are associated with hypogastric omphaloceles.
Gastroschisis results from occlusion of the omphalomesenteric ar-
tery during gestation, and involves herniation of the viscera through the
lateral abdominal wall defect.53Gastroschisis occurs in 1 in 15,000 births,
and is associated with a low incidence of other anomalies. Intestinal

Table 2. COMPARISON OF OMPHALOCELE WITH GASTROSCHISIS

Omphalocele Gastroschisis
Incidence 1:6000 1:15,000
Gestation Premature Term
Peritoneal covering Present Absent
Incidence of other anomalies High Low
 NEONATAL SURGICAL EMERGENCIES 277

atresia, however, is higher in neonates with gastroschisis, compared with

omphaloceles.

Anesthetic Management
Preoperative management of neonates with gastroschisis and om-
phalocele is similar, and is directed at preventing infection and minimiz-
ing fluid and heat loss. Covering the exposed viscera or membranous
sac with sterile saline-soaked dressings and wrapping these dressings
with plastic wrap decreases evaporative fluid and heat loss.
Although surgical correction of an omphalocele or gastroschisis is
urgent, surgery usually is delayed until the patient is properly prepared
for anesthesia. Preoperative preparation involves a thorough evaluation
because many of these babies have associated anomalies. Preoperative
management should be individualized. Fluid and electrolyte abnormali-
ties should be corrected preoperatively.
Anesthetic management involves volume resuscitation and the pre-
vention of hypothermia. Even when the abdomen is relaxed by muscle
relaxants and the viscera are not distended, primary closure may not
always be possible without markedly increasing intra-abdominal pres-
sure. Stretching the abdominal wall and pushing bowel contents toward
the stomach and rectum may help decrease intra-abdominal pressure.
Ventilatory compromise and decreased organ perfusion are the major
problems that result when intra-abdominal pressure is increased mark-
edly. Other problems include bowel edema, anuria, and hypotension. A
pulse oximeter probe on the lower extremity will detect a decrease in
oxygen saturation that could be caused by congestion of the lower
extremities due to obstruction of venous return. Measurement of intra-
gastric pressure, central venous pressure, or cardiac index can aid in
determining whether primary closure is appropriate.ll3r114 If primary
closure is not reasonable, a staged repair can be done. This involves
leaving the bowel extraperitoneally encased in a synthetic mesh (silon
chimney) or plastic pouch, that is reduced in stages over several days
until the abdominal cavity can accommodate the viscera without com-
promising organ perfusion or ventilation. Most neonates do not require
anesthesia for the daily reduction in size of the chimney, but they do
need anesthesia for the final stage of repair, which involves removing the
synthetic material and closing the abdomen. Postoperative management
depends on the type of repair and whether or not the child has associ-
ated anomalies. Fluid resuscitation should continue postoperatively be-
cause fluid loss through the viscera continues, especially in a staged
repair, where the viscera are left extraperitoneally.

NECROTIZING ENTEROCOLITIS
Necrotizing enterocolitis is a condition that results from hypoper-
fusion of the gut. It occurs predominantly in premature infants with a
278 LIU&I’ANG

gestational age of less than 32 weeks and with a weight of less than
1500 g. The pathogenesis of this condition is elusive. Immaturity of the
gastrointestinal mucosal barrier and the immune system and premature
oral feeding are conditions frequently implicated in causing this condi-
95 Early signs of necrotizing enterocolitis include feeding problems,
lethargy, hyperglycemia, bloody stools, and fever. The abdomen may be
distended and tender. Radiographic studies that may initially suggest
an ileus with edematous bowel show free air in the abdomen if intestinal
perforation occurs.
Necrotizing enterocolitis frequently is treated medically if the diag-
nosis is made early. Enteral feedings are discontinued, the abdomen is
decompressed with a nasogastric or orogastric tube that is attached to
intermittent low-pressure suction, and blood volume is replenished with
intravenous fluids. Broad-spectrum antibiotics usually are administered,
although no specific organism has been implicated in causing necrotizing
enterocolitis. Dopamine is given to improve renal and intestinal perfu-
sion and cardiac output.
Indications for surgery are usually refractory sepsis or intestinal
perforation. As a result, babies with necrotizing enterocolitis are usually
severely ill when they are scheduled for surgery. They may be hypoten-
sive, anemic, thrombocytopenic, and coagulopathic, and have prerenal
azotemia and a metabolic acidosis.

Anesthetic Management

Patients with necrotizing enterocolitis frequently arrive in the op-

erating room with an endotracheal tube in place. If they are not intu-
bated, a rapid-sequence or awake intubation is performed after the
standard monitors are placed. Drugs used for anesthesia depend on the
patient’s condition. A narcotic (e.g., fentanyl, 1-5 kg/kg) and a relaxant
(e.g., cisatracurium, 0.1-0.2 mg/kg) frequently are the only drugs used
for anesthesia, because potent inhalation anesthetics may decrease blood
pressure below acceptable levels. Nitrous oxide generally is avoided to
prevent further intestinal distension. Other drugs may be required to
treat cardiovascular instability or electrolyte abnormalities. Dopamine
may be indicated if renal perfusion and cardiac output are low; bicarbo-
nate may be indicated if the base deficit is significant.
Volume resuscitation is crucial when managing neonates with necro-
tizing enterocolitis. Third-space fluid loss is high, and multiple blood
volumes of lactated Ringer’s solution or albumin may be needed to
replenish intravascular volume. Blood, fresh frozen plasma, and platelets
also may be needed to improve oxygen-carrying capacity or to treat
factor deficiencies.
After surgery, babies are transported with their endotracheal tube
in place to the ICU, and are placed on a ventilator until extubation
criteria are met. Fluid and drug resuscitation may need to be continued
until vital signs are stable.
 NEONATAL SURGICAL EMERGENCIES 279

INTESTINAL OBSTRUCTION

Intestinal obstruction is a surgical emergency in the newborn that

requires swift intervention. Delays in diagnosis and treatment may lead
to intestinal perforation, bowel necrosis, and septicemia.

DUODENAL OBSTRUCTION

The incidence of duodenal obstruction in the neonate is 1 in 10,000

to 40,000 births. It frequently is associated with other congenital anoma-
lies, such as Down syndrome, cystic fibrosis, renal anomalies, intestinal
malrotation, and midline defects, such as esophageal atresia and imper-
forate anus.63,78Obstruction of the duodenum can be due to an intralumi-
nal diaphragm or membranous web. Infants with obstructions may or
may not pass meconium in the first day of life, depending on whether
the obstruction is partial or complete. The infant presents with vomiting
of bile or bile-stained gastric contents and minimal abdominal disten-
sion.

JEJUNOILEAL ATRESIA

Jejunoileal atresia causes complete intestinal obstruction. The inci-

dence is 1 in 5000 live births. This lesion, in contrast to duodenal
obstruction, rarely is associated with other congenital a n ~ m a l i e sFifty
.~~
percent of infants with this lesion are premature. Jejunoileal atresia is
believed to be secondary to an intrauterine vascular accident.

MECONIUM ILEUS

Meconium ileus is an intraluminal obstruction of the distal small

bowel caused by abnormal meconium. This entity is found exclusively
in patients with cystic fibrosis. Approximately 10% to 20% of the patients
with cystic fibrosis are born with meconium ileus. In some patients, the
obstruction is managed medically; in others, surgical intervention is
necessary. During surgery the obstruction is located, and the meconium
is massaged into the colon. Occasionally, the meconium may need to be
softened by agents such as acetylcysteine before it can be massaged into
the colon. If the meconium cannot be pushed into the colon, an enteros-
tomy is performed, and the meconium obstruction is evacuated from
the bowel.

MALROTATION AND VOLVULUS

The true incidence of malrotation and volvulus of the intestines is

not known because many patients are undiagnosed, and is believed to
280 LIU&PANG

occur because of abnormalities or arrests in the rotation of the bowel.

Areas of ischemia and atresia develop, resulting in bowel strangulation,
bloody stools, peritonitis, and hypovolemic shock. Malrotation fre-
quently is associated with major cardiac, esophageal, urinary, and anal
anomalies, congenital diaphragmatic hernia, and abdominal wall defects.
Malrotation is two times more common in male infants, and frequently
presents in the neonatal period. This problem also can present in adult-
hood.

IMPERFORATE ANUS

The incidence of anal atresia is 1 in 5000 live births. The presence

of this entity is usually obvious at birth, and can range from a mild
stknosis to a complex syndrome with other associated congenital anoma-
lies. In general, the higher the location of the anomaly, the greater the
incidence of associated anomalies. In male infants, an operative proce-
dure to relieve the obstruction may be required soon after birth. In
female infants, the usual presence of a rectovaginal fistula in association
with the imperforate anus prevents the development of distension; oper-
ative intervention may be postponed for several weeks.

Anesthetic Management

Anesthetic considerations for patients with intestinal obstruction

include airway management, fluid and electrolyte replacement, treat-
ment of sepsis, and postoperative pain management.
Complete intestinal obstruction increases the risk for aspiration of
gastric or intestinal contents. Diaphragmatic excursion is impeded by
abdominal distension and may cause respiratory distress. To prevent
further abdominal distension in a patient with a suspected difficult
airway, the patient should be intubated while awake; a rapid-sequence
induction with cricoid pressure might be considered in a patient with
no apparent airway problems.
Patients with intestinal obstruction may be volume depleted second-
ary to dyes used in diagnostic tests and to peritonitis, ileus, bowel
manipulation, and sepsis. Volume resuscitation with at least 10 mL/kg
per hour of normal saline or colloid may need to be given before
anesthesia can be induced. The quality of heart tones, urine output,
blood pressure, skin color, and turgor will aid in fluid management. In
some cases invasive monitoring with arterial and central venous pres-
sures are necessary.
The choice of anesthetic agents depends on the patient’s condition.
Nitrous oxide should be avoided to prevent further distension of the
bowel. Regardless of the anesthetic drugs used, an adequate circulating
blood volume must be maintained to ensure perfusion of vital organs.
 NEONATAL SURGICAL EMERGENCIES 281

SUMMARY

Improvements in the diagnosis and treatment of congenital disor-

ders have resulted in a change in surgical practice. Many conditions that
formerly required corrective surgery immediately after birth are no
longer surgical emergencies. Most babies with congenital anomalies that
can be corrected by surgery are now stabilized and optimized before the
procedure. This article focused on the more common conditions that
require semi-elective or urgent surgery in the neonatal period. Salient
features of each of these disorders were described. Factors unique to
each of these conditions that can affect the anesthetic course of these
children were discussed. Methods and techniques that may aid in the
anesthetic management of these children were delineated.

References

1. Adzick NS, Vacanti JP, Lillehei CW, et a1 Fetal diaphragmatic hernia: Ultrasound
diagnosis and clinical outcome in 38 cases. J Pediatr Surg 24654-658, 1989
2. Ahmad SA, Sylvester KG, Hebra A, et a 1 Esophageal replacement using the colon: Is
it a good choice? J Pediatr Surg 313026-1031,1996
3. Ahmed A, Spitz L: The outcome of colonic replacement of the esophagus in children.
Prog Pediatr Surg 19:37-54, 1986
4. Alexander F, Johanningman J, Martin LW Staged repair improves outcome of high-
risk premature infants with esophageal atresia and tracheoesophageal fistula. J Pediatr
Surg 28151-154, 1993
5. Andropoulos DB, Heard MB, Johnson KL, et al: Postanesthetic apnea in full-term
infants after pyloromyotomy. Anesthesiology 80:216-219, 1994
6. Areechon W, Reid L Hypoplasia of lung with congenital diaphragmatic hernia. Br
Med J 1:230-233, 1963
7. Atkinson JB, Hamid R, Steward DJ: General anesthesia with isoflurane for diaphrag-
matic hernia repair during ECMO. ASAIO J 40986989,1994
8. Azarow K, Messineo A, Pearl R, et al: Congenital diaphragmatic hernia: A tale of
two cities: The Toronto experience. J Pediatr Surg 32:395-400, 1997
9. Bair JH, Russ PD, Pretorius DH, et al: Fetal omphalocele and gastroschisis: A review
of 24 cases. AJR Am J Roentgenol 1471047-1051, 1986
10. Beals DA, Schloo BL, Vacanti JP, et al: Pulmonary growth and remodeling in infants
with high-risk congenital diaphragmatic hernia. J Pediatr Surg 27997-1002,1992
11. Beasley SW A practical approach to the investigation and management of long gap
oesophageal atresia. Indian J Pediatr 63:737-742, 1996
12. Beasley SW, Allen M, Myers N The effects of Down syndrome and other chromo-
somal abnormalities on survival and management in oesophageal atresia. Pediatr
Surg Int 12:550-551, 1997
13. Berdon WE, Baker DH, Amoury R The role of pulmonary hypoplasia in the prognosis
of newborn infants with diaphragmatic hernia and eventration. AJR Am J Roentgenol
103:413421, 1968
14. Bilbrey GL, Herbin L, Carter NW, et a1 Skeletal muscle resting membrane potential
and potassium deficiency. J Clin Invest 523011-3018,1973
15. Birmingham PK, Tobin MJ, Henthom TK, et al: Twenty-four-hour pharmacokinetics
of rectal acetaminophen in children: An old drug with new recommendations. Anes-
thesiology 87244-252, 1997
16. Bohn DJ, James I, Filler RM, et al: The relationship of Paco2and ventilation parameters
in predicting survival in congenital diaphragmatic hernia. J Pediatr Surg 19:666470,
1984
282 LIU&PANG

17. Bohn D, Tamura M, Perrin D, et a1 Ventilatory predictors of pulmonary hypoplasia

in congenital diaphragmatic hernia, confirmed by morphologic assessment. J Pediatr
111:423-431, 1987
18. Boix-Ochoa J, Peguero G, Seijo G, et al: Acid-base balance and blood gases in
prognosis and therapy of congenital diaphragmatic hernia. J Pediatr Surg 9:49-57,
1974
19. Bos AP, Tibboel D, Koot VCM, et al: Persistent pulmonary hypertension in high-risk
congenital diaphragmatic hernia patients: Incidence and vasodilator therapy. J Pediatr
Surg 28:1463-1465, 1993
20. Bouchut J-C, Dubois R, Moussa M, et al: High-frequency oscillatory ventilation during
repair of neonatal congenital diaphragmatic hernia. Paediatr Anaesth 10:377-379,2000
21. Brands W, Kachel W, Wirth H, et al: Indication for using extracorporeal membrane
oxygenation in congenital diaphragmatic hernias and pulmonary hypoplasia. Eur J
Pediatr Surg 2:81-86, 1992
22. Brawn WJ, Huddart SN: Tracheoaortopexy via midline sternotomy in tracheomalacia.
J Pediatr Surg 26:660462,1991
23. Butt W, Taylor B, Shann F: Mortality prediction in infants with congenital diaphrag-
. matic hernia: Potential criteria for ECMO. Anaesth Intensive Care 20439442, 1992
24. Chahine AA, Ricketts RR: Esophageal atresia in infants with very low birth weight.
Semin Pediatr Surg 9:73-78, 2000
25. Chetcuti P, Phelan PD, Greenwood R Lung function abnormalities in repaired
oesophageal atresia and tracheo-oesophageal fistula. Thorax 471030-1034, 1992
26. Choudhury SR, Ashcraft KW, Sharp RJ, et al: Survival of patients with esophageal
atresia: Influence of birth weight, cardiac anomaly, and late respiratory complications.
J Pediatr Surg 34:70-74, 1999
27. Clark RH, Hardin WD Jr, Hirschl RB, et al: Current surgical management of congenital
diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study
Group. J Pediatr Surg 33:1004-1009, 1998
28. Companale RP, Rowland RH: Hypoplasia of the lung associated with congenital
diaphragmatic hernia. Ann Surg 142:176-189, 1955
29. Connors RH, Tracy T Jr, Bailey PV, et al: Congenital diaphragmatic hernia repair on
ECMO. J Pediatr Surg 25:1043-1047, 1990
30. Corbally MT, Spitz L, Kiely E, et al: Aortopexy for tracheomalacia in oesophageal
anomalies. Eur J Pediatr Surg 3:264-266, 1993
31. Cot6 CJ, Zaslavsky A, Downes JJ, et al: Postoperative apnea in former preterm infants
after inguinal herniorrhaphy: A combined analysis. Anesthesiology 82:809-822, 1995
32. Cusick EL, Batchelor AAG, Spicer R D Development of a technique for jejunal interpo-
sition in long-gap esophageal atresia. J Pediatr Surg 28:990-994, 1993
33. Delius RE, Wheatley MJ, Coran AG: Etiology and management of respiratory compli-
cations after repair of esophageal atresia with tracheoesophageal fistula. Surgery
1121527-532, 1992
34. de Lorimier AA, Harrison MR Long-gap esophageal atresia: Primary anastomosis
after esophageal elongation by bougienage and esophagomyotomy. J Thorac Cardio-
vasc Surg 79:138-141, 1980
35. Desfrere L, Jarreau PH, Dommergues M, et a1 Impact of delayed repair and elective
high-frequency oscillatory ventilation on survival of antenatally diagnosed congenital
diaphragmatic hernia: First application of these strategies in the more 'severe' sub-
group of antenatally diagnosed newborns. Intensive Care Med 26:934-941,2000
36. deVries PA: The pathogenesis of gastroschisis and omphalocele. J Pediatr Surg 15:245-
251, 1980
37. Diaz J H Tension pneumoperitoneum-pneumothoraxduring repair of congenital dia-
phragmatic hernia. Anesth Analg 66577-580, 1987
38. Engum SA, Grosfeld JL, West KW, et al: Analysis of morbidity and mortality in 227
cases of esophageal atresia and/or tracheoesophageal fistula over two decades. Arch
Surg 130:502-508,1995
39. Falconer AR, Brown RA, Helms P, et al: Pulmonary sequelae in survivors of congenital
diaphragmatic hernia. Thorax 45:126-129, 1990
40. Fauza DO, Wilson JM: Congenital diaphragmatic hernia and associated anomalies:
 NEONATAL SURGICAL EMERGENCIES 283

Their incidence, identification, and impact on prognosis. J Pediatr Surg 29:1113-1117,

1994
41. Filston HC, Chitwood WR Jr., Schkolne B, et a1 The Fogarty balloon catheter as an
aid to management of the infant with esophageal atresia and tracheoesophageal
fistula complicated by severe RDS or pneumonia. J Pediatr Surg 17149-151, 1982
42. Fogata MLC, Collins HB 11, Wagner CW, et al: Prenatal diagnosis of complicated
abdominal wall defects. Curr Probl Diagn Radio1 28:106-128, 1999
43. Forestell CG, Lonngvist PA, Sonesson SE, et al: Near-fatal pulmonary hypertension
after surgical repair of congenital diaphragmatic hernia: Successful use of inhaled
nitric oxide. Anaesthesia 48:679483, 1993
44. Fujino Y, Takezawa J, Nishimura M, et a 1 High-frequency oscillation for persistent
fetal circulation after repair of congenital diaphragmatic hernia. Crit Care Med 17376-
377, 1989
45. Geggel RL, Murphy JD, Langleben D, et al: Congenital diaphragmatic hernia: Arterial
structural changes and persistent pulmonary hypertension after surgical repair. J
Pediatr 107457464, 1985
46. Germain JF, Farnoux C, Pinquier D, et al: Can blood gas values predict pulmonary
hypoplasia in antenatally diagnosed congenital diaphragmatic hernia? J Pediatr Surg
31:1634-1639, 1996
47. Guibaud L, Filiatrault D, Garel L, et al: Fetal congenital diaphragmatic hernia: Accu-
racy of sonography in the diagnosis and prediction of the outcome after birth. AJR
Am J Roentgen01 166:1195-1202, 1996
48. Guys JM, Triglia JM, Louis C, et al: Esophageal atresia, tracheomalacia and arterial
compression: Role of aortopexy. Eur J Pediatr Surg 1261-265, 1991
49. Haugen SE, Linker D, Eik-Nes S, et al: Congenital diaphragmatic hernia: Determina-
tion of the optimal time for operation by echocardiographic monitoring of the pulmo-
nary arterial pressure. J Pediatr Surg 26:560-562, 1991
50. Harrison MR, Adzick NS, Estes JM, et a1 A prospective study of the outcome for
fetuses with diaphragmatic hernia. JAMA 271:382-384, 1994
51. Hazebroek FWJ, Tibboel D, Bos AP, et a 1 Congenital diaphragmatic hernia: Impact
of preoperative stabilization. A prospective pilot study in-13 patients. J Pediatr Surg
231139-1146, 1988
52. Holder TN Jr., McDonald VGM, Wolley MM: The premature or critically ill infant
with esophageal atresia: Increased success with a staged approach. J Thorac Cardio-
vasc Surg 44344358,1962
53. Hoyme HE, Higginbottom MC, Jones KL The vascular pathogenesis of gastroschisis:
Intrauterine interruption of the omphalomesenteric artery. J Pediatr 98228-231, 1981
54. Iocono JA, Cilley RE, Mauger DT, et al: Postnatal pulmonary hypertension after repair
of congenital diaphragmatic hernia: Predicting risk and outcome. J Pediatr Surg
34349-353, 1999
55. Iritani I: Experimental study on embryogenesis of congenital diapahragmatic hernia.
Anat Embryo1 169:133-139, 1984
56. Jouppila P, Kirkinen P: Ultrasonic and clinical aspects in the diagnosis and prognosis
of congenital gastrointestinal anomalies. Ultrasound Med Biol 10:465472, 1984
57. Kamitsuka MD, Horton MK, Williams MA: The incidence of necrotizing enterocolitis
after introducing standardized feeding schedules for infants between 1250 and 2500
g and less than 35 weeks of gestation. Pediatrics 105:379-384, 2000
58. Karl Hw: Control of life-threatening air leak after gastrostomy in an infant with
respiratory distress syndrome and tracheoesophageal fistula. Anesthesiology 6267CL
672, 1985
59. Kawadia V, Greenough A, Laubscher B, et a 1 Perioperative assessment of respiratory
compliance and lung volume in infants with congenital diaphragmatic hernia: Predic-
tion of outcome. J Pediatr Surg 32:1665-1669,1997
60. Kays DW, Langham MR Jr., Ledbetter DJ, et al: Detrimental effects of standard
medical therapy in congenital diaphragmatic hernia. Ann Surg 230340-351, 1999
61. Khan AR, Stiff G, Mohammed AR, et al: Esophageal replacement with colon in
children. Pediatr Surg Int 13:79-83, 1998
62. Kim SH: Omphalocek. Surg Clin North Am 56:361-372, 1976
284 LIU&PANG

63. Kimble RM, Harding J, Kolbe A: Additional congenital anomalies in babies with gut
atresia or stenosis: When to investigate and which investigation. Pediatr Surg Int
12~565-570,1997
64. Kimura K, Soper RT Multistaged extrathoracic esophageal elongation for long-gap
esophageal atresia. J Pediatr Surg 29:566-568, 1994
65. Kimura K, Soper RT, Kao SCS, et al: Aortosternopexy for tracheomalacia following
repair of esophageal atresia: Evaluation by cine-CT and technical refinement. J Pediatr
Surg 25:769-772, 1990
66. Kitagawa M, Hislop A, Boyden EA, et al: Lung hypoplasia in congenital diaphrag-
matic hernia: A quantitative study of airway, artery, and alveolar development. Br J
Surg 58:342-346, 1971
67. Kluth D Atlas of esophageal atresia. J Pediatr Surg 11:901-919, 1976
68. Ladd WE, Gross RE: Congenital diaphragmatic hernia. N Eng J Med. 223:917-925,
1940
69. Lally KP, Breaux CW Jr: A second course of extracorporeal membrane oxygenation
in the neonate: Is there a benefit? Surgery 117:175-178, 1995
70. Lally KP, Clark R, Schwendeman C, et al: Extracorporeal membrane oxygenation in
the newborn. Mil Med 155:377-379, 1990
71. Lbvetpe C, Hemza J, Berg A, et al: Successful repair of a severe left congenital
diaphragmatic hernia during continuous inhalation of nitric oxide. Anesthesiology
80:1171, 1994
72. Levin DL: Morphologic analysis of the pulmonary vascular bed in congenital left-
sided diaphragmatic hernia. J Pediatr 92805-809, 1978
73. Liu LM, Cot6 CJ, Goudsouzian NG, et a1 Life-threatening apnea in infants recovering
from anesthesia. Anesthesiology 59:50&510, 1983
74. Miguet D, Claris 0, Lapillonne A, et al: Preoperative stabilization using high-fre-
quency oscillatory ventilation in the management of congenital diaphragmatic hernia.
Crit Care Med 22(suppl 9):77-82, 1994
75. Mishalany HG, Nakada K, Woolley MM: Congenital diaphragmatic hernias: Eleven
years experience. Arch Surg 114:1118-1123, 1979
76. Murat I, Lapeyre G, Saint-Maurice C: Isoflurane attenuates baroreflex control of heart
rate in human neonates. Anesthesiology 70:395400, 1989
77. Murat I, Levron JC, Berg A, et al: Effects of fentanyl on baroreceptor reflex control of
heart rate in newborn infants. Anesthesiology 68:717-722, 1988
78. Murshed R, Nicholls G, Spitz L: Intrinsic duodenal obstruction: Trends in manage-
ment and outcome over 45 years (1951-1995) with relevance to prenatal counseling.
Br T Obstet Gvnaecol 106:1197-1199. 1999
79. Myers NA: The history of esophegal surgery: Pediatric aspects. Pediatr Surg Int.
12:lOl-107, 1997
80. Nagaya M, Akatsukka H, Kato J: Gastroesophageal reflux occurring after repair of
congenital diaphragmatic hernia. J Pediatr Surg 29:1447-1451, 1994
81. Nakayama DK, Motoyama EK, Tagge EM: Effect of preoperative stabilization on
respiratory system compliance and outcome in newborn infants with congenital
diaphragmatic hernia. J Pediatr 118793-799, 1991
82. Newman KD, Anderson KD, Van Meurs K, et al: Extracorporeal membrane oxygen-
ation and congenital diaphragmatic hernia: Should any infant be excluded? J Pediatr
Surg 25:1048-1053, 1990
83. Payne NR, Kriesmer PJ, Mammel M, et al: Comparison of six ECMO selection criteria
and analysis of factors influencing their accuracy. Pediatr Pulmonol 11:223-232, 1991
84. Pedersen JC, Klein RL, Andrews DA: Gastric tube as the primary procedure for pure
esophageal atresia. J Pediatr Surg 31:1233-1235, 1996
85. Puri P, Wester T Historical aspects of congenital diaphragmatic hernia. Pediatr Surg
Int 12~95-100,1997
86. Raphaely RC, Downes JJ Jr: Congenital diaphragmatic hernia: Prediction of survival.
J Pediatr Surg 8:815-823, 1973
87. Rejjal A: Congenital anomalies associated with esophageal atresia: Saudi experience.
Am J Perinatol 16:239-244, 1999
88. Reyes C, Chang LK, Waffam F, et al: Delayed repair of congenital diaphragmatic
 NEONATAL SURGICAL EMERGENCIES 285

hernia with early high-frequency oscillatory ventilation during preoperative stabiliza-

tion. J Pediatr Surg 33:lOlO-1016,1998
89. Rittler M, Paz JE, Castilla EE: VATERL: An epidemiologic analysis of risk factors. Am
J Med Genet 73362-169, 1997
90. Robertson DF, Mobaireek K, Davis GM, et al: Late pulmonary function following
repair of tracheoesophageal fistula or esophageal atresia. Pediatr Pulmonol20:21-26,
1995
91. Ruff SJ, Campbell JR, Harrison M W , et a1 Pediatric diaphragmatic hernias: An 11
year experience. Am J Surg 139:641-645, 1980
92. Saing H, Mya GH, Cheng W The involvement of two or more systems and the
severity of associated anomalies significantly influence mortality in esophageal atre-
sia. J Pediatr Surg 33:1596-1598, 1998
93. Sakai H, Tamura M, Hosokawa Y, et al: Effect of surgical repair on respiratory
mechanics in congenital diaphragmatic hernia. J Pediatr 111:432438, 1987
94. Salem MR, Wong AY, Lin YH, et al: Prevention of gastric distension during anesthesia
for newborns with tracheoesophageal fistulas. Anesthesiology 38:82-83, 1973
95. Salzman NH, Polin RA, Harris MC, et al: Enteric defensin expression in necrotizing
enterocolitis. Pediatr Res 44:20-26, 1998
96. Somaschini M, Locatelli G, Salvoni L, et al: Impact of new treatments for respiratory
failure on outcome of infants with congenital diaphragmatic hernia. Eur J Pediatr
158:780-784, 1999
97. Somppi E, Tammela 0, Ruuska T, et al: Outcome of patients operated on for esopha-
geal atresia: 30 years experience. J Pediatr Surg 33:1341-1346,1998
98. Spitz L: Esophageal atresia: Past, present, and future. J Pediatr Surg 31:19-25, 1996
99. Stolar CJH, Price ME, Butler M W , et al: Management of infants with congenital
diaphragmatic hernia using ECMO. In Arensman R, Cornish D (eds): Extracorporeal
Life Support. Boston, Blackwell, 1993, p 252-261
100. Tazuke Y, Kawahara H, Yagi M, et a1 Use of a Palmaz stent for tracheomalacia: Case
report of an infant with esophageal atresia. J Pediatr Surg 341291-1293, 1999
101. The Congenital Diaphragmatic Hernia Study Group: Does extracorporeal membrane
oxygenation improve survival in neonates with congenital diaphragmatic hernia? J
Pediatr Surg 34:720-725, 1999
102. Torfs CP, Curry CJ, Bateson TF: Population-based study of tracheoesophageal fistula
and esophageal atresia. Teratology 52220-232, 1995
103. Touloukian RJ, Higgins E: The spectrum of serum electrolytes in hypertrophic pyloric
stenosis. J Pediatr Surg 18:394-397, 1983
104. Tracy TF Jr, Bailey PV, Sadiq F, et al: Predictive capab es of preoperative and
postoperative pulmonary function tests in delayed repair of congenital diaphragmatic
hernia. J Pediatr Surg 29:265-270, 1994
105. Triglia JM, Guys JM, Louis-Borrione C: Tracheomalacia caused by arterial compression
in esophageal atresia. Ann Otol Rhino1 Laryngol 103:516-521, 1994
106. Troug RD, Schena JA, Hershenson MB, et al: Repair of congenital diaphragmatic
hernia during extracorporeal membrane oxygenation. Anesthesiology 72750, 1990
107. Vanamo K, Rintala RJ, Lindahl H, et al: Long-term gastrointestinal morbidity in
patients with congenital diaphragmatic defects. J Pediatr Surg 31:551-554, 1996
108. Wear R, Robinson S, Gregory G: The effect of halothane on the baroresponse of adult
and baby rabbits. Anesthesiology 56:188-192, 1982
109. Weber TR, Kountzman 8, Dillon PA, et a1 Improved survival in congenital diaphrag-
matic hernia with evolving therapeutic strategies. Arch Surg 133498-502, 1998
110. Welbom LG, Greenspun JC: Anesthesia and apnea: Perioperative considerations in
the former preterm infant. Pediatr Clin North Am 41:181-198, 1994
111. Wilson JM, Lund DP, Lillehei CW, et al: Congenital diaphragmatic hernia: A tale of
two cities: The Boston experience. J Pediatr Surg 32:401-405,1997
112. Wung JT, Sahni R, Moffitt ST, et al: Congenital diaphragmatic hernia: Survival treated
with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg
30:406-409,1995
113. Yaster M, Buck JR, Dudgeon DL, et al: Hemodynamic effects of primary closure of
omphalocele/gastroschisis in human newborns. Anesthesiology 69:84-88, 1988
286 LIU&PANG

114. Yaster M, Scherer TLR, Stone MM, et al: Prediction of successful primary closure of
congenital abdominal wall defects using intraoperative measurements. J Pediatr Surg
24:1217-1220, 1989
115. Young DG: Contralateral pneumothorax with congenital diaphragmatic hernia. Br
Med J 4:433-434, 1968

Address reprint requests to

Letty M. P. Liu, MD
Department of Anesthesiology
University of Medicine and
Dentistry of New Jersey-
New Jersey Medical School
185 South Orange Avenue, MSB E-538
Newark, NJ 07103