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29

General Abdominal and Urologic


Surgery

Tom G. Hansen, Steen W. Henneberg, Jerrold Lerman

General Principles of Abdominal Surgery

“The Full Stomach”: The Risk for Pulmonary Aspiration of


Gastric Contents

Rapid-Sequence Induction

Indications for Preoperative Nasogastric Tube Placement

Fluid Balance

Potential for Strangulated or Ischemic Bowel and Sepsis

Presence of Abdominal Compartment Syndrome

Preoperative Laboratory Testing and Investigations

Monitoring Requirements

Choice of Anesthetic

General Principles of Urologic Surgery

Reduced Renal Function

Systemic Arterial Hypertension

Corticosteroid Medications

2955
Infection or Sepsis

Monitoring Requirements

Laparoscopic Surgery

Respiratory Effects

Cardiovascular Effects

Central Nervous System Effects

Renal Effects and Fluid Requirements

Pain Management

Robot-Assisted Surgery

Specific General Surgical and Urologic Conditions

Nissen Fundoplication

Pectus Excavatum

Circumcision

Hypospadias and Chordee

Cryptorchidism and Hernias: Inguinal and Umbilical

Torsion of the Testis

Posterior Urethral Valves

Prune-Belly Syndrome

Ureteral Reimplantation

Pyeloplasty

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Nephrectomy

Neuroblastoma

Wilms Tumor

Bladder and Cloacal Exstrophy

Bariatric Surgery in Children

Multidisciplinary Approach to Bariatric Surgery in Adolescents

Anesthetic Implications of Obesity in Adolescents

ABDOMINAL SURGERY AND UROLOGIC interventions make up a


large fraction of anesthetic practice for the pediatric anesthesiologist. The
field is rapidly evolving, with increased use of laparoscopic surgery,
including robot-assisted procedures.1 This chapter focuses on the specific
issues related to abdominal and urologic surgery, particularly in young
children. The management of infants for pyloromyotomy and other
neonatal abdominal procedures is discussed in Chapter 37.

General Principles of Abdominal Surgery


“The Full Stomach”: The Risk for
Pulmonary Aspiration of Gastric Contents
Many abdominal surgeries are emergent procedures that require a rapid
induction of anesthesia and protection of the airway to prevent
regurgitation and pulmonary aspiration. Adhering to fasting guidelines
for elective surgery does not ensure that the stomachs of children with
acute abdomens are empty of liquids and solids. The only metric that has
been associated with gastric emptying after an acute emergency in
children is the time interval between the last food ingested and the
occurrence of the pathologic event or trauma.2 However, there is no firm
fasting interval after a trauma that predicts a zero risk of regurgitation
and aspiration. The presence or absence of bowel sounds is also not
predictive of gastric emptying or of the risk of regurgitation.

2957
Preoperatively, some children with acute abdomens are administered
oral contrast agent before abdominal ultrasonography and/or computed
tomography (CT) to visualize the stomach contents and to estimate their
volumes. However, these radiologic tools may not provide reliable
estimates of the volume of the gastric contents.3,4 Indeed, the absence of
gastric contents in these scans does not eliminate the risk of vomiting and
regurgitation. Consequently, there is no evidence that delaying the
surgical procedure for the express purpose of emptying the stomach will
reduce the risk of regurgitation; postponing may actually increase the
risk of complications by delaying the urgently needed surgical attention
to the acute abdomen.

Rapid-Sequence Induction
Rapid-sequence induction (RSI) is recommended for children with a full
stomach to quickly secure the airway. This approach is intended to
minimize the risk of aspiration, although it is not evidence-based. The
strategy is to predetermine the drug doses for the child and have all the
required airway equipment (age- and size-appropriate) ready to use. The
predetermined drug doses are administered in a rapid sequence and
when muscle relaxation has been achieved, the trachea is intubated and
the cuff (if used) inflated. Many clinicians apply cricoid pressure to
occlude the esophageal lumen during RSI, although not a single
randomized trial has compared the frequency of regurgitation and
aspiration with cricoid pressure during RSI with that with an inhalational
or slow IV induction in either children or adults as summarized in a
recent Cochrane review.5 Recently, the presence of microaspirates in the
tracheas of 95 adults at risk for regurgitation (obese, those with diabetes,
and those with gastroesophageal reflux) was compared with and without
cricoid pressure during induction of elective surgery. There was no
difference in the frequency of microaspirates in the two groups.6 This
lack of evidence, combined with both theoretical and actual
complications associated with RSI and cricoid pressure in children, has
led to great skepticism regarding their roles in preventing pulmonary
aspiration in children who are at risk, although large population-based
studies are lacking.7 Only 74% of anesthesiologists in Northern Ireland
perform RSI for children scheduled for appendectomy, 78% in the United
States use it for pyloromyotomy, and 83% in England use it for forearm

2958
fractures within 2 hours of eating and after recent opioid
administration.8–10 In two surveys, 16% and 28% of anesthesiologists in
the United States and United Kingdom, respectively, reported that a
number of children with full stomachs had experienced gastric
regurgitation despite using RSI with cricoid pressure; several of them
progressed to serious harm and even death.9,11
Despite the lack of evidence supporting the effectiveness of RSI in
children with full stomachs, we continue to recommend this approach for
the majority of children at risk. Whether cricoid pressure during RSI
contributes substantively to preventing regurgitation performed remains
unclear. Based on evidence from non-randomized controlled studies
(RCTs), a recent Cochrane review concluded that cricoid pressure may
not be necessary to safely achieve RSI.5 The authors assert that well-
designed and properly conducted RCTs should be encouraged to assess
the safety and effectiveness of cricoid pressure in infants and children;
however, because the frequency of pulmonary aspiration is so small, it
would take many thousands of cases to establish a substantive outcome
and likely would not be readily approved by an institutional review
board.5
Complications of RSI, for the most part, relate to improperly
performed RSI (e.g., excessive cricoid pressure distorting the anatomy of
the airway,12 causing difficulty in securing the airway) or poor selection
of patients (those with a known difficult airway, where a more measured
approach must take precedence over concerns for possible aspiration).
RSI in infants and children requires more planning than in older
children and adults for several reasons. First, the induction drugs should
be flushed into the child's veins using a separate flush syringe to ensure a
rapid bolus administration of the drugs. Succinylcholine remains useful
for rapid-sequence tracheal intubation for brief procedures. The
introduction of intermediate-acting neuromuscular blocking drugs
(NMBDs) with rapid onset, coupled with concerns about the risk of
hyperkalemia after succinylcholine in children with undiagnosed
neuromuscular diseases (especially males <8 years of age), has
dramatically reduced the use of succinylcholine in elective surgery. With
the shift from succinylcholine to nondepolarizing NMBDs for rapid
paralysis in young children, inability to intubate and prolonged paralysis
may present serious, possible life-threatening problems. However, with

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the availability of sugammadex, the use of rocuronium (1.2 mg/kg)
represents a safe alternative to succinylcholine.13–15 Second,
preoxygenation is often difficult in infants and children because they
commonly resist the tight application of the face mask needed to fully
denitrogenate the lungs. Failure to ventilate the lungs after induction and
before tracheal intubation may result in desaturation more rapidly in
young infants and children than older children, and in those with upper
respiratory tract infections or other causes of a limited oxygen
reserve.16,17 During laryngoscopy and intubation, mask ventilation with
100% oxygen should begin when the saturation reaches 95%, to attenuate
the nadir in oxygen saturation that follows.16 Third, the force needed to
occlude the esophagus when applying cricoid pressure to infants and
children is poorly understood and poorly applied, can distort the view of
the larynx during laryngoscopy, may not occlude the lumen of the
esophagus, and may actually deform the lumen of the trachea if excessive
force is applied.7,12,18 For example, as little as 10-N force will distort the
shape of the cricoid ring and reduce the lumen by 50% in children
younger than 5 years of age.12 Effective cricoid pressure that occludes the
esophagus in children and permits bag-and-mask ventilation with up to
40 cm H2O peak inspiratory pressure without gastric insufflation19 is
known as a modified RSI. Thus, if the first attempt at tracheal intubation
fails or the child desaturates during laryngoscopy, properly maintained
cricoid pressure allows bag-and-mask ventilation to restore oxygenation,
without increasing the risk of regurgitation. A third technique that uses
low insufflation pressures, known as the controlled RSI technique, has
also been proposed.20,21 In the United Kingdom, a recent survey of adult
anesthetists that documented a large variability in how the RSI was
performed22 has prompted calls for evidence-based guidelines for RSI in
infants and children.

Indications for Preoperative Nasogastric


Tube Placement
Although there are no published guidelines for placing nasogastric tubes
preoperatively, it is reasonable to insert a tube preoperatively to allow
drainage of gastrointestinal fluids in cases of documented bowel
obstruction (e.g., ileus, strangulated bowel, pyloric obstruction) or in

2960
other situations in which the risk of aspiration is judged to be substantial.
The child may experience discomfort when a nasogastric tube is inserted
preoperatively, but this must be balanced against the need to decompress
the stomach and reduce the risk of regurgitation during induction of
anesthesia. For every other indication, the nasogastric tube may be
placed after tracheal intubation. It should be noted that the presence of a
nasogastric tube may decrease lower esophageal sphincter tone, increase
the risk for reflux, and reduce the ability to clear refluxed gastric contents
from the distal esophagus.23,24 Thus the anesthesiologist is faced with the
dilemma of whether or not to remove the nasogastric tube that was
placed before induction of anesthesia. It may be reasonable to apply
suction to the nasogastric tube, evacuate all of the gastric contents, and
then remove the nasogastric tube before inducing anesthesia because it is
unclear whether cricoid pressure, even if properly applied, prevents
wicking of gastric contents along the path created by the nasogastric
tube. It should be further noted that even with a well-placed nasogastric
tube, one can never guarantee that the stomach has been completely
emptied.

Fluid Balance
Many acute abdominal emergencies are associated with pronounced and
significant shifts in fluids, mainly in the form of dehydration, electrolyte
losses, third-space fluid shifts, and hypovolemia. In most instances,
correction of these derangements is mandatory before proceeding with
anesthesia and surgery. However, when a large fraction of the bowel
becomes strangulated and ischemic, large volumes of fluid may be
sequestered in the bowel. In these cases, hypovolemia should be
suspected and resuscitation initiated as anesthesia is rapidly induced. In
some elective cases (e.g., bowel resection because of inflammatory bowel
disease), fluid and electrolyte resuscitation should routinely be a focus of
special interest, because the child may not be fully compensated at the
time of surgery.
To date, published studies have suggested that resuscitation with
crystalloid fluids and colloids have equipoise.25 In children, initial
resuscitation is usually undertaken with balanced salt solutions. A recent
Cochrane review concluded that the use of isotonic intravenous (IV)
fluids with sodium concentrations similar to that of plasma reduces the

2961
risk of hyponatremia.26 Although colloids may result in less tissue edema
and less volume infused, the expense may not justify their routine use. In
fact, some have even questioned the use of colloids in patients with
sepsis.27

Potential for Strangulated or Ischemic Bowel


and Sepsis
The need for anesthesia and surgery becomes more urgent when the
bowel is potentially ischemic and/or necrotic. For example, if a volvulus
is suspected, immediate action is necessary; otherwise the child is at risk
for massive bowel necrosis necessitating resection of dead bowel with
subsequent short bowel syndrome, a condition associated with serious
lifelong medical problems or even death. Even if the child is far from
optimally resuscitated, anesthesia must be induced and maintained,
preferably with anesthetics that maintain circulatory homeostasis, while
simultaneously correcting the dehydration (or hypovolemia) and
electrolyte imbalance. The situation is somewhat less critical in the child
with an incarcerated inguinal hernia, although delay of even this surgery
should be minimized.
Ischemic bowel may release a host of mediators that can cause severe
hemodynamic instability. Children with acute intraabdominal disease
should always be regarded as being at risk for bacterial translocation and
possible septicemia. Those with overt sepsis are usually easy to identify
and may have already been admitted to the pediatric intensive care unit.
However, children with incipient or early sepsis may not exhibit overt
signs. Accordingly, the signs of sepsis should be actively sought. If
septicemia is present or suspected, appropriate IV antibiotics should be
administered without delay, preferably before anesthesia and surgery.
Children with sepsis or presepsis can be extremely unstable and may
require inotropic and/or vasoactive drugs. Immediately after induction of
anesthesia vascular sympathetic tone may be attenuated, leading to
sudden hemodynamic instability. Thus, when a substantial segment of
the bowel becomes ischemic, the anesthesiologist must maintain
anesthesia without depressing the circulation excessively, acutely
resuscitate the child with appropriate fluids, correct electrolyte
imbalances, particularly potential hyperkalemia, and consider the use of

2962
inotropic and/or vasoactive medications as needed. It should be noted
that hemodynamic instability might acutely worsen when ischemic
bowel is suddenly reperfused or immediately after the abdominal cavity
is opened. In such cases, close communication with the surgeon is
paramount. Furthermore, the presence of sepsis-induced acute lung
injury may reduce pulmonary compliance. Thus the anesthesiologist
needs to prepare for invasive monitoring (arterial and central venous
pressures) and an intraoperative ventilator that is capable of delivering
high positive end-expiratory pressure (PEEP).

Presence of Abdominal Compartment


Syndrome
Acute intraabdominal disease processes may lead to a critically increased
intraabdominal pressure (IAP).28 If the IAP increases above the capillary
perfusion pressure of the intraabdominal organs, an abdominal
compartment syndrome can develop. Organ perfusion will become
compromised and ischemia and/or necrosis may develop. The most
commonly affected organs in this situation are the bowels, kidneys, and
liver. Abdominal compartment syndrome occurs less frequently in
children than in adults.29 Causes of abdominal compartment syndrome
include burns, extracorporeal membrane oxygenation,30 closure of
gastroschisis or omphalocele (see Chapter 37),31 abdominal trauma,32,33
abdominal surgery,34 and a host of other intraabdominal pathologies,
including necrotizing enterocolitis, Hirschsprung enterocolitis,
perforated bowel, diaphragmatic hernia, and Wilms tumor.30,35,36
Insufficient perfusion of the bowel may cause an ileus, translocation of
bacteria, lactate accumulation, and production of mediators that cause
hemodynamic instability. Increased IAP can reduce liver blood flow,
which will reduce hepatic function,37 mainly manifested as an inability to
metabolize lactate, impaired drug metabolism,37,38 and, in severe cases,
impaired synthesis of coagulation factors. Because the pressure is also
transmitted to the retroperitoneal space, renal function may become
impaired, resulting in oliguria or anuria and reduced excretion of
drugs.35 In addition, cranial displacement of the abdominal contents and
splinting of the diaphragm may seriously compromise ventilation.39
If acute intraabdominal compartment syndrome is suspected, then the

2963
IAP should be monitored to prevent the pressures from exceeding the
critical threshold of 20 to 25 mm Hg. IAP can be measured indirectly by
transducing a nasogastric tube or bladder catheter.40 Some define
compartment syndrome when the vesicular (bladder) pressure exceeds
10 to 12 mm Hg.41,42 The diagnosis of intraabdominal compartment
syndrome should be suspected when the triad of (1) massive abdominal
distention, (2) increased bladder pressures and increased peak
inspiratory airway pressures, and (3) evidence of hepatic, renal, and/or
cardiac dysfunction are present.36,43,44
Children with acute intraabdominal compartment syndrome are often
hemodynamically unstable. Although decompression of the abdomen by
a laparotomy will immediately normalize the IAP, reperfusion of the
ischemic tissues almost always releases a host of biologically active
substances that cause profound hypotension. These substances may also
precipitate acute renal failure and lead to a disseminated intravascular
coagulopathy. As in the case of sepsis, the anesthesiologist must be fully
prepared to address these challenges by ensuring that blood products are
present in the operating room and vasopressors are drawn up and
available before induction of anesthesia. Some children will require a
patch abdominoplasty as a temporizing measure to protect abdominal
organs that require delayed primary closure of the anterior abdominal
wall.36,43

Preoperative Laboratory Testing and


Investigations
Most minor elective cases (e.g., umbilical or inguinal hernia repair) do
not require any preoperative workup beyond a basic history and physical
examination. Many centers require a preoperative urine (or hemoglobin)
screen for pregnancy in females who have reached menarche (see
Chapter 4).45 More complex elective cases may warrant additional
laboratory testing, including basic hematology screening and electrolyte
profile.
Preoperative laboratory testing is strongly advised in more critically ill
children. Liver and renal function tests, coagulation profile, and serum
albumin concentration should be assessed and blood typed and
crossmatched. In children with sepsis or who have an acute

2964
intraabdominal compartment syndrome, a preoperative chest radiograph
may indicate the severity of pulmonary involvement. An
echocardiogram may be needed to assess myocardial contractility and
volume status if cardiac dysfunction is suspected.

Monitoring Requirements
Routine elective cases rarely require more than standard monitoring
equipment. In children undergoing major intraabdominal procedures,
invasive arterial and central venous blood pressure monitoring may be
indicated. A multiple-lumen central venous line inserted at the beginning
of the procedure will facilitate administration of inotropic and/or
vasoactive drugs, in addition to measuring central venous pressure;
ultrasound-guided insertion is strongly recommended.46 These lines are
of great value in the immediate postoperative period for blood sampling,
drug administration, ongoing assessment of intravascular volume status,
and parenteral nutrition. Transesophageal echocardiographic,
transesophageal Doppler, or continuous noninvasive cardiac output
(CO)47 evaluation may provide valuable intraoperative and postoperative
information regarding the child's volume status, as well as cardiac
contractility (see also Chapter 52).48–58
A urinary catheter with a pediatric urometer (i.e., a graduated
collection receptacle), which provides an accurate measure of urine
output, is a useful monitor for most intraabdominal procedures.
Maintaining a stable hourly urine output may safeguard against the
development of hypovolemia and possibly prerenal azotemia (see the
section “Laparoscopic Surgery” for discussion of changes in urine output
with increased IAP).
Monitoring IAP is important during laparoscopic surgery, although it
is of minimal value in omphalocele and gastroschisis surgeries, as long as
the abdomen remains open. Once the abdomen is closed, however, IAP
provides useful prognostic information regarding intraabdominal organ
(e.g., renal) blood flow, circulatory stability, and respiratory
embarrassment (see Chapter 37).39

Choice of Anesthetic

2965
The anesthesiologist may use his or her personal preference of anesthetic
technique for the management of both elective and emergency
intraabdominal surgery in children. However, airway management
associated with intraabdominal surgery requires careful consideration.
Even when the child is not at increased risk for regurgitation and
aspiration, the risk of regurgitation can be increased if the surgeon
positions the child in the Trendelenburg position and/or insufflates the
peritoneal cavity with carbon dioxide (CO2) during laparoscopic surgery.
A particular concern arises when the surgeon decides to decompress
distended bowel by creating an enterotomy and directly draining the
fluid, or by “milking” or “stripping” the bowel in a retrograde direction
until the contents can be vented with a nasogastric tube. The latter
method can cause massive intestinal regurgitation that exceeds the
capacity of the nasogastric tube causing pulmonary aspiration.59 It is for
this reason and others that a laryngeal mask airway (LMA) should not be
used during intraabdominal surgery; we strongly recommend that the
trachea should be intubated with a cuffed tube as standard practice in
these cases.
Regional anesthetic techniques may be useful adjuncts in children
undergoing both minor and major abdominal surgery. Those who have
had open abdominal procedures will require IV opioids or the use of a
continuous epidural infusion of local anesthetics with or without opioids
for perioperative pain management (see Chapter 44). Analgesia is
commonly supplemented with parenteral nonsteroidal antiinflammatory
drugs and/or acetaminophen. In most children who have had
laparoscopic surgery, adequate postoperative analgesia may be achieved
by infiltrating local anesthetics at the port insertion sites. However,
referred shoulder pain, the result of accumulated air under the
diaphragm, may require IV opioids.60 In critically unstable children or
those with sepsis, the use of neuroaxial anesthesia is not recommended
because sympathetic blockade may further exacerbate the hemodynamic
instability and the catheter could provide a nidus for infection.

General Principles of Urologic Surgery


With the exception of acute drainage of urinary obstruction (i.e.,
ultrasound-guided nephrostomy or cystostomy procedures) and torsion

2966
of the testis, most pediatric urologic surgeries are elective. In the vast
majority of cases, these children are otherwise healthy or have stable
medical conditions that do not require more than a careful history,
physical examination, and review of the child's medical record. Children
who undergo urologic procedures may be suffering from emotional
disturbance because of repeated interventions and sensitivity of the
surgical site. This mandates special psychological attention before and
after anesthesia.
Worldwide, the prevalence of latex allergy has recently been estimated
to be 9.7%, 7.2%, and 4.3% among health care workers, susceptible
patients, and the general population, respectively.61 In the 1990s, the
prevalence of latex allergy in children with spinal dysraphism exceeded
70%, several years later, it had decreased to less than 17%, and most
recently evidence suggests the prevalence may be as small as 3% in those
with dysraphism in a nonlatex environment.62 Indeed, latex anaphylaxis
is of particular concern in children with chronic urologic disorders.63–66 In
the past, children with spina bifida were prone to developing latex
allergy much more often than those without spina bifida67–70 because the
former were repeatedly exposed to latex urinary catheters beginning
early after birth. All children with congenital malformations of the
urinary tract who were repeatedly exposed to latex via their mucous
membranes beginning in the neonatal period were at significant risk for
developing latex hypersensitivity until recently.71,72 However, a
widespread shift in practice to avoid exposure to all latex products in
these children has been extremely (but not 100%) effective in attenuating
the prevalence of this allergy.62 The exceptions usually occur when
someone unfamiliar with the child's latex allergy introduces a latex
product and sensitizes the child or triggers a reaction. Thus latex-free
management is highly recommended in this population.62,73–75

Reduced Renal Function


Children with chronic renal disease have impaired renal function, which
may affect drug dosing and disposition, as well as cause secondary
effects on the cardiovascular system. In the most severe cases, the child
may require dialysis to balance fluids and electrolytes. In children with
renal disease, it is essential to determine the degree of renal impairment
by consulting the child's nephrologist and reviewing the serum

2967
creatinine, blood urea nitrogen, sodium, and potassium concentrations
(see also Chapter 28). Because renal impairment may also affect clotting,
a coagulation profile, including platelet count, should be reviewed
preoperatively if substantial blood loss is anticipated. These children are
prone to fluid overload, particularly those who are anuric and dialysis
dependent. Apart from clinical signs associated with fluid overload,
measuring the child's weight and comparing it with their normal weight
is a simple means to assess the child's current volume status. If cardiac
function or volume status remains in doubt, an echocardiogram should
be obtained. Children with chronic renal insufficiency often have
impaired left ventricular function even before they require dialysis, so a
preoperative echocardiogram may be indicated76–80; pericardial effusion
is also a concern.77,81,82
For children undergoing dialysis, the most recent date of dialysis
should be documented. Overhydration and/or hypervolemia and
hyperkalemia should be corrected preoperatively with dialysis. Although
dialysis corrects these abnormalities, and may transiently improve
platelet function (peritoneal dialysis yielding more consistent
improvement than hemodialysis), it is best to avoid dialysis within 12
hours of anesthesia to preclude a relative hypovolemia and to allow
sufficient time for body fluids to reequilibrate (see Chapter 28).83,84
Postdialysis laboratory indexes of serum electrolytes (particularly
potassium), hemoglobin or hematocrit, renal function (creatinine and
blood urea nitrogen), and the child's weight loss should be assessed. For
children who undergo hemodialysis, IV access and blood pressure
measurements should not be obtained in the extremity ipsilateral to the
arteriovenous fistula.

Systemic Arterial Hypertension


Systemic hypertension is common in renal insufficiency in adults but is
far less common in children. Nonetheless, some children with urologic
disorders develop systemic hypertension associated with disturbances in
the renin-angiotensin system.85–88 As in adults, it is important to control
systemic hypertension before induction to avoid wide swings in blood
pressure. In contrast to adults, however, hypervolemia is an important
cause of hypertension in children with renal insufficiency that should be
considered and treated preoperatively. Children and adults are often

2968
treated with similar antihypertensive medications (see Chapter 28).87,88
All medications should be continued up to and including the morning of
surgery to maintain intraoperative and postoperative hemodynamic
stability with the exception of angiotensin-converting enzyme (ACE)
inhibitors. The latter drugs should be stopped 1 day before surgery to
avoid intraoperative hypotension89–91; however, withholding this
medication may also lead to rebound hypertension postoperatively. If
these medications are not withheld, vasopressors may be required during
anesthesia to stabilize the blood pressure.92 Because therapy-resistant
renal hypertension is an indication for nephrectomy, one should
anticipate and be prepared to treat wide fluctuations in blood pressure,
including severe hypertension during the first stage of the operation and
profound hypotension when the responsible kidney is removed.
Therefore, long-acting antihypertensive agents are best avoided during
the early stages of a nephrectomy in a child.

Corticosteroid Medications
Children with renal disease may be chronically treated with
corticosteroids as part of their medical management (e.g., children with
proteinuria or who have undergone previous renal transplant surgery).
In such cases, a stress dose of parenteral corticosteroids during surgery is
indicated, with continued supplementation until the child resumes his or
her normal corticosteroid medication by the enteral route. In more
complex situations, consultation with a pediatric nephrologist or
endocrinologist is warranted to optimize corticosteroid supplementation,
although in more straightforward cases, a dose of 2.5 mg/kg of IV
hydrocortisone two to three times each day is usually adequate (see
Chapter 27).

Infection or Sepsis
Obstructive urinary tract disease or chronic renal insufficiency increases
the risk for urinary tract infections. If treated properly, the infection
should not interfere with anesthesia. However, in children with overt
signs of systemic illness or septicemia, the anesthetic and postoperative
courses may be difficult.

2969
Monitoring Requirements
Standard noninvasive monitoring is adequate for the vast majority of
urologic procedures, as they are of minor or moderate magnitude (e.g.,
circumcision, orchiopexy, pyeloplasty, and ureter neoimplantation). The
anesthetic care required for infants and children undergoing penile
surgery is routine, including a forced-air heating mattress to prevent
hypothermia for surgeries lasting longer than 1 hour. However,
recognizing the minimal amount of skin that is exposed during this type
of surgery, the child's temperature often increases, necessitating close
monitoring of the child's temperature to avoid overheating.
Invasive monitors (i.e., arterial and central venous access for pressure
monitoring and administration of vasoactive drugs) may be indicated in
major surgical interventions, and in cases with concomitant problems
(e.g., decreased renal function, significant hypertension, associated
cardiac dysfunction, sepsis). If central venous access is deemed
necessary, the coagulation status of the child should be evaluated
because platelet function may be substantially compromised. Ultrasound
guidance improves safety while securing central venous access in cases
in which a coagulopathy is present or suspected on clinical grounds (see
Chapter 49). In the unstable child, even more complex monitoring (e.g.,
esophageal Doppler monitoring, transesophageal echocardiography, or
continuous noninvasive CO)47 should be considered (see Chapter 52).
It is important to monitor urine output, although the volume of urine
may not reflect renal function. During surgery that involves the bladder
or in children who are anuric, urine output will not be available for part
or all of the surgery. Hence, other indexes of fluid status and perfusion
must be used. Heart rate and systolic blood pressure are reliable indexes
of volume status and perfusion in most children, although as stated
earlier central venous pressure and invasive arterial pressure
measurement may be indicated in special cases. Surgeons may be
heartened to observe urine flowing into the bladder after reimplantation
of ureters and kidney transplantation in place of measuring it. Preloading
with a balanced salt solution may be useful in such circumstances;
occasionally a loop diuretic is requested.

Laparoscopic Surgery
2970
Background
Although laparoscopic surgery was introduced more than 80 years ago,
its role in pediatric surgery has become notable only in the past 10 to 15
years. To a large extent, this has been due to improved technology in
optics and miniaturization of the instruments. An increasing number of
general and urologic surgical procedures (including appendectomy,
cholecystectomy, and splenectomy, as well as those to treat inguinal
hernia and undescended testicles) are performed either laparoscopically
or using robotic techniques in children of all ages (Figs. 29.1 to 29.4).93–101
More sophisticated laparoscopic techniques have enabled the
performance of complex surgical procedures, including Nissen
fundoplication, colectomy, pyeloplasty, bowel pull-through, and removal
of large organs, including the kidney and spleen.99,102–105 Technological
advances now permit laparoscopic surgery in neonates and small infants,
including those with hypoplastic left heart syndrome after stage 1 or 2
repair.101,106–108 It must be noted, however, that laparoscopic surgery in
children with cyanotic heart disease carries with it a substantial risk that
exceeds most other populations having this form of surgery. Although
several reports suggest that infants and children of all ages, at all stages
of palliative repair of their cyanotic heart disease, tolerate laparoscopic
surgery,108–110 the risk for those with Fontan physiology undergoing
laparoscopic surgery is substantial. It is essential to understand that in
Fontan physiology, pulmonary blood flow is passive, and decreases in
venous return (whether from increases in intrathoracic pressure or head-
up positioning) or increases in pulmonary vascular resistance (because of
increased CO2 partial pressures or decreased minute ventilation) could
severely reduce CO.111 Creating a pneumoperitoneum for laparoscopic
surgery and extreme positions increase IAP and arterial CO2 tensions
(increasing pulmonary artery pressure) reduces venous return and
increases the risk of reducing the CO in children with Fontan physiology
(see later discussion). Successful management of these children during
laparoscopic surgery requires a multidisciplinary team that functions in
concert to enhance the child's outcome (1) by optimizing the child's
condition preoperatively and identifying any cardiac issues, (2) by
recruiting surgeons who can complete the surgery quickly and
efficiently, (3) by maintaining the children in the supine position
throughout the surgery, (4) by maintaining normocapnia, (5) by

2971
monitoring the child with a transesophageal echo probe as needed, as
well as with an arterial invasive pressure monitor for blood gas analysis,
and (6) by insufflating the abdomen to the minimal (<8 mm Hg) IAP that
is surgically feasible. Larger studies are required before children with
cyanotic heart disease, particularly those with Fontan physiology, can
routinely undergo laparoscopic surgery in any center except those with
specialized teams to manage these children.

FIGURE 29.1 A child undergoing laparoscopic


appendectomy, presented as a paradigm for the surgical
setup for any multiple-trocar laparoscopic surgery. Three
incisions were made, two in the anterior abdominal wall
and the third in the umbilicus. The first two trocars carry
instruments to manipulate the appendix, while the third
holds the camera for viewing by all personnel in the
operating room. All cables swing widely off the surgical
field.

FIGURE 29.2 Wide-angle view of the operating room with

2972
surgeon and scrub nurse viewing the appendix being
held by the grasper, shown on the overhead monitor.

FIGURE 29.3 Inside the abdominal cavity with a view of


the inflamed appendix that has been mobilized. The
peritoneal attachments must be peeled off the appendix
before it is ligated and removed.

FIGURE 29.4 The appendix is ligated, inserted into a


plastic container, and withdrawn, usually through the
trocar or incision site without contaminating adjacent
tissues. The appendix is shown in the surgeon's hand
after removal.

Laparoscopic surgery offers a number of advantages over open


surgery, including more rapid emergence from anesthesia, faster
ambulation, earlier discharge from the hospital, and reduced
perioperative complications.112–115
Because laparoscopic surgery involves the insufflation of gas into the
abdominal cavity to visualize the intraabdominal organs, the stomach

2973
should be decompressed using a nasogastric or orogastric tube for upper
abdominal surgery, whereas the bladder should be emptied using a
urinary catheter for lower abdominal surgery. Surgical access to the
peritoneal cavity is achieved with trocars introduced through one to
three small (3 to 10 mm in diameter) incisions; the laparoscopic
instruments and a camera are then passed through the trocars. Laparo-
endoscopic single-site (LESS), single-port, single-incision laparoscopic
surgery (SILS), single-incision multiport laparoscopy (SIMPL), or “belly
button” surgery has been developed, in which all instruments pass
through a single incision and a single large trocar (often in the umbilicus)
(E-Figs. 29.1-29.3).116–120

E-FIGURE 29.1 The “poor man's” single incision multiport


laparoscopy (SIMPL) device. Through a flap lifted in the
umbilicus, a collapsible sheath is passed into the
peritoneal cavity that opens once inside the abdomen to
stabilize the sheath. On the skin surface, a ring stabilizes
the sheath, over which a surgical glove is affixed. A
fingertip on the glove is nicked and the gas insufflator
(carbon dioxide feed) is inserted. The peritoneal cavity is
then inflated to the desired pressure. Through adjacent
finger tips, the camera and operating instruments are
passed to perform the surgery. (Courtesy Dr. K. Vali, Pediatric
Surgery, Women and Children's Hospital of Buffalo, Buffalo, NY.)

2974
E-FIGURE 29.2 A commercially available single incision
multiport laparoscopy (SIMPL) device for a child in which
a single sheath is inserted through a flap lifted in the
umbilicus. The ports and gas insufflator are mounted on
the outside. For more information, see Chandler NM,
Danielson PD. Single-incision laparoscopic
cholecystectomy in children: a retrospective comparison
with traditional laparoscopic cholecystectomy. J Pediatric
Surg. 2011;46(9):1695–1699. (Courtesy Dr. K. Vali, Pediatric Surgery,
Women and Children's Hospital of Buffalo, Buffalo, NY.)

E-FIGURE 29.3 A commercially available single incision


multiport laparoscopy (SIMPL) device in a child through
which the camera and two laparoscopic instruments
have access to the abdominal cavity. This is achieved
through the umbilicus with one surgical incision. For
more information, see Chandler NM, Danielson PD.
Single-incision laparoscopic cholecystectomy in children:

2975
a retrospective comparison with traditional laparoscopic
cholecystectomy. J Pediatric Surg. 2011;46(9):1695–
1699. (Courtesy Dr. K. Vali, Pediatric Surgery, Women and Children's Hospital of
Buffalo, Buffalo, NY.)

Pneumoperitoneal pressure is a major concern for all laparoscopic


approaches. Experimental evidence in adult and newborn pigs
demonstrated that the risks of cardiorespiratory consequences and
potentially fatal emboli were directly related to the peak
pneumoperitoneal pressure.121,122 In infants and children, the optimal
pneumoperitoneal pressure is the least pressure that enables adequate
surgical access. CO2 should be insufflated through one of the trocars until
the IAP reaches 6 to 15 mm Hg; most surgeons currently limit the IAP in
neonates and young infants to 6 to 8 mm Hg and in children to 10 to 12
mm Hg.123 The IAP is maintained throughout the surgery by
intermittently insufflating additional CO2. Although CO2 is most
commonly used, a number of gases have been investigated (see later
discussion).124 CO2 is the preferred gas because it does not support
combustion, is rapidly cleared from the peritoneal cavity at the end of
surgery, and does not expand into bubbles or spaces.103,125,126

Adverse Effects of Pneumoperitoneum

The increased IAP during laparoscopic surgery may cause a number of


physiochemical side effects, including cardiorespiratory depression,
hypothermia as the result of dry gas leakage, pneumothorax or
subcutaneous emphysema, endobronchial intubation resulting from the
upward shift of tracheal bifurcation, or injury resulting from
paracentesis.

Carbon Dioxide

The major disadvantage of using CO2 to insufflate the peritoneum arises


from its rapid absorption from the peritoneum, with the absorption and
washout of CO2 more rapid and the peak end-tidal partial pressure of
CO2 (PETCO2) greater in infants than in older children.127,128 The inverse
relationship between age and the rate of CO2 absorption from the
abdomen has been attributed to the thinner peritoneum and the reduced

2976
peritoneal fat deposits in the abdomen of infants compared with older
children.128,129 Because CO2 is so readily absorbed, particularly in infants,
both the PETCO2 and PaCO2 may increase 20% to 50% above
baseline.104,123,125,127,130 The resultant hypercapnia, which occurs more
commonly in surgery lasting longer than 1 hour, particularly in
neonates,131 necessitates an increase in minute ventilation (from 50% to
100%) to maintain a physiologic pH.103,132 The difference between the
partial pressures of arterial and end-tidal CO2 (PaCO2−PETCO2) often
increases during insufflation of CO2.130,132 In one report, the
PaCO2−PETCO2 gradient before and after pneumoperitoneum increased
from a mean of 5.7 mm Hg to 13.4 mm Hg.133 It should be noted that in
neonates and in children with cyanotic congenital heart disease, the
PETCO2 may not reliably track the PaCO2 during CO2 insufflation, leading
some to recommend arterial blood gas monitoring to validate the PETCO2
measurements.130,133,134
Increased PaCO2 may also trigger spontaneous respiratory efforts that
could interfere with surgery. In addition, it may initiate a sympathetic
response, including increases in the heart rate, blood pressure, and
cerebral blood flow, as well as precipitate ventricular arrhythmias,
although this occurs rarely with sevoflurane. A sudden increase in
PETCO2 may also suggest a diagnosis of malignant hyperthermia if
accompanied with sudden onset of tachycardia.135–137 If this diagnosis
proves to be difficult to confirm or reject (in malignant hyperthermia
expect venous PvO2 <40 mm Hg; see Chapter 41), it may be necessary to
desufflate the abdomen and determine if the clinical and laboratory
findings suggestive of malignant hyperthermia resolve.129,138

Gas Emboli

Gas emboli have been reported in several laparoscopic studies, most of


which were interesting curiosities of no clinical consequence, although in
several, the result was profound cardiovascular collapse.139–141 Most
consider the emboli to be intravascular CO2 bubbles, although there is
evidence to suggest that some emboli contain nitrogen or air.142
Intravascular embolization of CO2 may occur when the insufflation
pressure exceeds the venous pressure, forcing CO2 bubbles into the
venous circulation, resulting in sudden cardiovascular collapse.140

2977
Continuous precordial Doppler or expired CO2 partial pressure are
effective in detecting a gas embolus, although the Doppler may be overly
sensitive, with numerous false-positive results. It has been suggested that
many episodes of subclinical gas embolism are unrecognized because the
symptoms are mild and nonspecific.143 However, children with right-to-
left shunts or potential right-to-left shunts, such as a patent foramen
ovale, are vulnerable to the systemic effects of these emboli. Although
CO2 is soluble in blood and rapidly buffered, CO2 emboli dissolve slowly
in blood, taking 2 to 3 minutes to disappear.144 Hence, large emboli may
block blood flow in the heart for several minutes or more before they
dissolve. The minimum rate of infusion of CO2 into blood that triggers
cardiovascular collapse in pigs is 1.2 mL/kg per minute144; comparable
data in humans are lacking. Anesthesiologists should be aware of the
high-risk conditions that predispose to CO2 emboli (e.g., increased
abdominal insufflation pressure, hypovolemia and reduced venous
pressure, spontaneous respirations, and resection of vessel-rich
parenchymatous organs) and communicate closely with the surgeons at
all times.
Some investigators suggest that clinically important gas emboli are
actually nitrogen, not CO2 gas emboli.140 Whereas transient emboli are
thought to be composed of CO2, emboli that persist may be nitrogen
gas.140 Nitrogen is insoluble in blood (blood/gas partition coefficient of
0.014), which explains its persistence as an embolus in blood. These
emboli may arise from air, either present in or entrained by the trocar
during insufflation of the peritoneum, that is forced into a transected
blood vessel while the carboperitoneum is pressurized.140 This
mechanism is a rare source for gas in the circulation, which may explain,
in part, why the emboli that occur during laparoscopy very rarely result
in cardiovascular instability and arrest.
In contrast to CO2, insufflation with oxygen, air, and nitrous oxide for
laparoscopic surgery has been eschewed because they all support
combustion. However, repeat desufflations of the pneumoperitoneal gas
during laparoscopy in pigs whose lungs were ventilated with 66%
nitrous oxide in oxygen prevents the concentration of nitrous oxide from
exceeding 10% in the pneumoperitoneal cavity.145 Nitrous oxide is still
avoided for both insufflation and as an adjunctive anesthetic gas during
laparoscopy, because it also expands into gas-filled cavities should gas

2978
emboli appear in the circulation.125 Its use during laparoscopic surgery
may distend the bowel in bowel obstruction and obscure the surgeon's
view, as well as expand any CO2 emboli that develop.
The inert gases argon and helium were also candidate gases for
insufflation to create a pneumoperitoneum as they cannot be oxidized
(and therefore ignited), although they are much more expensive than
CO2. When argon was used to create a pneumoperitoneum in pigs,
embolization occurred more frequently than with CO2.146 In theory, both
argon and helium can cause serious sequelae if embolized into the
vascular system because they are insoluble in blood (blood/gas solubility
of helium is 0.007 and argon is 0.029) and therefore likely to
persist.103,125,126,146

Respiratory Effects
As the pressure within the peritoneal cavity increases, the frequency of
adverse respiratory effects also increases, particularly at IAP greater than
15 cm H2O. The respiratory manifestations of increased IAP include
cephalad displacement of the diaphragm, decreased excursion of the
diaphragm, and decreases in pulmonary and thoracic compliance, vital
capacity, functional residual capacity, and closing volume.147 Cephalad
displacement of the diaphragm shifts ventilation to the nondependent
parts of the lungs, creating a ventilation-perfusion mismatch. With a
small functional residual capacity in children, cephalad displacement of
the diaphragm further compresses the lungs, causing collapse of the
small airways, ventilation-perfusion mismatch, and possibly hypoxemia.
Other adverse pulmonary mechanics after insufflation include increased
peak inspiratory pressures up to 27% and decreased compliance as much
as 39%.148 These physiologic changes are compounded by the extreme
body tilting (i.e., extreme head-up or head-down positions) often
requested by surgeons.125,149 Positioning the child head down (i.e.,
Trendelenburg position) first decreases compliance by 17%, and second,
adding a pneumoperitoneum decreases compliance by an additional
27%, requiring increases in peak inflation pressures of 19% and 32%,
respectively.150 In a recent study in which the pneumoperitoneum was
created before Trendelenburg positioning in children whose lungs were
ventilated using pressure control and 5 cm H2O PEEP, a

2979
pneumoperitoneum of 12 cm H2O decreased both dynamic compliance
and tidal volume by 42%.151 The addition of 20 degrees of Trendelenburg
positioning only decreased these values by an additional 10%. All of
these changes in pulmonary function can be offset by increasing minute
ventilation (rate and peak inflation pressure) by as much as 50% to 100%.
Pulmonary function appears to be restored more readily after
laparoscopic than open surgery.103
Another respiratory concern is that the pneumoperitoneum and the
extreme Trendelenburg position can shift the tracheal tube in a rostral
direction, as far as 1.2 to 2.7 cm, possibly having an impact on the carina
or passing into a bronchus.152 With these changes, inspired oxygen
concentrations in excess of 30% may be required, along with PEEP to
restore adequate oxygenation. A persistent 5% decrease in oxygen
saturation has been associated with partial or intermittent endobronchial
intubation.153
Securing the airway for laparoscopic surgery requires particular
attention. Cuffed tracheal tubes are preferred over uncuffed tubes to
ensure effective alveolar ventilation despite the conditions described
above,134 although LMAs and ProSeal supraglottic airways (Teleflex
Medical Inc.; Research Triangle Park, NC) have been used for brief
procedures during laparoscopic surgery without complications.126,154 If
the child has a mature tracheotomy, the air leak around the tracheotomy
must be assessed before surgery and if the leak is excessive, the
tracheotomy should be replaced with a cuffed tracheotomy, a cuffed
tube, or an armored (cuffed) tracheal tube. If the air leak around the
tracheotomy is insignificant, then ventilation should be adequate even in
the presence of increased IAP during laparoscopic surgery.
Excessive IAP may cause gas to track across the diaphragm, causing a
pneumomediastinum or pneumothorax.126 This is more common in
hiatus hernia surgery and Nissen fundoplication, during which
dissection of the esophagus may create passages for gas to traverse the
diaphragm. Pneumomediastinum should be suspected if subcutaneous
emphysema appears. If surgery creates a transdiaphragmatic passage for
CO2 to accumulate in the pleural space, the resulting pneumothorax may
produce cardiorespiratory manifestations. A chest radiograph should be
obtained if subcutaneous emphysema appears during or after surgery, or
if there is a high index of suspicion that a pneumothorax has formed.

2980
Both pressure- and volume-controlled ventilation have been used during
laparoscopic abdominal surgery in infants and children. In a single
randomized study, both ventilation strategies with 5 mm Hg PEEP
maintained effective ventilation and gas exchange.155
To avoid the cardiorespiratory compromise of a pneumoperitoneum, a
gasless laparoscopic approach has been described. This requires lifting
the anterior abdominal wall to create an intraabdominal tent.126,156
Implementation of the gasless approach in pediatric medicine has been
rather slow, presumably because of technical difficulties and the scarcity
of instruments for infants and children.

Cardiovascular Effects
Three major factors may contribute to adverse cardiovascular responses
during pneumoperitoneum: (1) IAP, (2) position (i.e., steep head-up or
reverse Trendelenburg), and (3) release of neurohumoral vasoactive
substances.126,147,156 Increased IAP exerts a biphasic effect on venous
return and CO. In neonatal pigs, the cardiac index (CI) decreased 55%
when IAP exceeded 20 mm Hg.126 In the animal model, the magnitude of
the increase in IAP determined the degree to which the circulation was
depressed.134,157 For example, at IAP 15 mm Hg or less, blood is
compressed out of the splanchnic circulation, increasing venous return,
which either increases or results in no change in CO. In contrast, at IAP
greater than 15 mm Hg, the inferior vena cava is compressed, reducing
venous return and therefore CO. Studies in children yielded similar
results; when the IAP exceeded 12 mm Hg, myocardial contractility158
and venous return125 decreased. In both infants and children, CO2
insufflation to IAP 10 to 13 mm Hg decreased CI approximately
13%.159–161 In studies in infants and children in which the CI decreased
during increased IAP (10-12 mm Hg), the CI returned to preinsufflation
values when the abdomen was desufflated.158–160 Left ventricular systolic
function was diminished, and septal wall motion abnormalities have
been reported with an IAP of 10 to 12 mm Hg in children with a CO2
pneumoperitoneum.158,159 No significant changes in echocardiographic
indexes of left ventricular work, preload or afterload, have been noted if
the IAP is 10 mm Hg or lower during the pneumoperitoneum.123 When
standard indexes of hemodynamics were measured in infants and young

2981
children during laparoscopic Nissen fundoplication, IAP of 10 mm Hg or
less yielded no significant changes in heart rate and blood pressure but a
slight increase in CI.162,163 If IAP is maintained at 10 mm Hg or less, then
the impact on hemodynamics (particularly CO) should be clinically
insignificant, because venous return may be enhanced as a result of
displacement of blood from the splanchnic bed, and afterload is not
increased.125,134,162 Most importantly, if the IAP is less than 5 mm Hg, CI
is maintained in infants during a CO2 pneumoperitoneum162; these low
insufflation pressures are those often used to examine the contralateral
side of a hernia defect during surgery.
Body position during laparoscopic surgery may exaggerate
cardiovascular changes. For Nissen fundoplication, a steep head-up
position (>20-degree incline) has been used, which reduces venous
return.149 In adult pigs, laparoscopic surgery for Nissen fundoplication
increased pleural and mediastinal pressures that, in turn, reduced CO
episodically at an IAP of 15 mm Hg.164 These decreases in CO were
manifested by episodes of hypotension and hypoxia. When children
were positioned in the steep head-up position, they developed transient
hypotension and bradycardia that were reversed immediately with fluid
loading and atropine.165
It is imperative to continuously monitor IAP to minimize the
cardiorespiratory effects of laparoscopic surgery and to avoid excessive
insufflation pressures. In adults, induction of anesthesia, insufflating the
IAP to 14 mm Hg and a 10-degree head-up tilt decrease the CI more than
50%.166 Some clinicians recommend a maximum IAP during laparoscopy
in children of 6 to 8 mm Hg to limit the cardiorespiratory effects,167
although most studies favor pressures of 10 to 12 mm Hg.151,157,159,168 In
neonates and infants, the maximum IAP should not exceed 6 to 8 mm Hg
to minimize cardiorespiratory effects. Based on the current literature, the
net cardiovascular effects of insufflating the abdomen to pressures of 12
mm Hg or less, combined with the head-up position, are likely to be well
tolerated if adequate hydration is maintained and bradycardia is
avoided.165

Central Nervous System Effects


Laparoscopic surgery must be carefully evaluated if planned for a child

2982
with increased intracranial pressure (ICP) or in the presence of a
ventriculoperitoneal shunt. The combination of increased IAP, increased
systemic vascular resistance, increased PaCO2 tension, and
Trendelenburg position (as in lower abdominal surgery) may
dramatically increase ICP. In adults during extreme head-down position
(40 degrees) for prolonged periods, such as in robot-assisted surgery in
the pelvis, cerebral tissue oxygen saturation is well maintained, as
evidenced by near-infrared spectroscopy.169 Under similar surgical
conditions, intraocular pressure increased 100%, and scleral edema and
blurred vision may develop.170 In anesthetized children during
laparoscopic surgery, intraocular pressure increased 30% after
insufflation (<15 mm Hg IAP) compared with laparotomy surgery.171
Patency of a VP shunt should be evaluated before the procedure to
prevent sudden increases in ICP during the procedure. Children with
reduced brain ventricular system compliance may sustain dramatic
increases in ICP if the IAP is sufficient to attenuate the drainage of
cerebrospinal fluid into the abdominal cavity. In these children,
laparoscopic surgery may be relatively contraindicated.149 The risks
should be thoroughly explored and discussed with the neurosurgeon,
general surgeon, anesthesiologist, and the family. Children with VP
shunts have shown a range of responses to laparoscopy from dramatic
increases in ICP to no change at all.147,172,173 Accordingly, a variety of
approaches have been suggested. Some advocate externalizing the shunt
and clamping the distal (intraabdominal) end of the shunt before surgery
to prevent CO2 from passing retrograde up the shunt or from the
laparoscopic pressure disrupting the shunt valve, although these valves
are stable with an IAP up to 80 mm Hg.174 Another approach has been to
temporarily isolate the tip of the VP shunt in an Endopouch bag (Ethicon,
Somerville, NJ) while insufflating pressures to 12 mm Hg and then
removing the bag at the end of the procedure.175 Others discourage
externalizing the shunt as sequelae have been reported, and instead
recommend monitoring the ICP to prevent and detect increases in ICP
and further recommend retraction of abdominal tissue during the
laparoscopic surgery.174 For the surgeon, using a SILS approach to
laparoscopic surgery in these children reduces the risk of both
traumatizing and infecting the VP shunt.176 One retrospective review
reported that laparoscopy in children with VP shunts was not associated
with an increased risk of shunt infection compared with open

2983
procedures.174 No single strategy can provide the optimal management
for every child with a VP shunt undergoing laparoscopic surgery.

Renal Effects and Fluid Requirements


Increased IAP decreases renal blood flow, renal function (creatinine
clearance and glomerular filtration rate), and urine output.134,147,177 One
study examined renal oxygenation with near-infrared spectroscopy and
found no evidence of renal hypoxemia when age-appropriate IAPs were
used (<6 mm Hg in neonates, <8 mm Hg in 2- to 12-month old infants,
<10 mm Hg 1 to 2 years of age, <12 mm Hg 2–8 years of age). At the same
time, they reported increasing cerebral oxygen saturation (likely from
increased cerebral blood flow from increased arterial CO2), heart rate,
and mean arterial pressure.178 The overall effects of IAP on renal function
and renal filtration are poorly understood. Decreases in urine output
during laparoscopic surgery in children vary, in part, with the age of the
child: oliguria occurs in older children and anuria in infants younger
than 1 year of age.147,149,179 The etiology of the renal dysfunction and
oliguria is multifactorial but includes direct and indirect effects of IAP on
renal perfusion, antidiuretic hormone (ADH), endothelin, and nitric
oxide (NO).147,180 ADH concentrations increase as a result of reduced
renal blood flow, resorbing water, and decreasing urine output. IAP
increases renal endothelin (endothelin-1), resulting in renal
venoconstriction, which reduces renal blood flow and urine output.147,180
Inhibiting endogenous NO exacerbates the renal dysfunction during
pneumoperitoneum through several mechanisms, including reduced
renal perfusion and increased salt and water resorption (e.g., oliguria).180
In theory, pretreating patients with a NO donor (such as L-arginine or
nondepressor doses of nitroglycerin) may attenuate the detrimental
effects of a pneumoperitoneum on renal function.180 Renal tubular injury
does not contribute to the renal dysfunction associated with increased
IAP.181 In adult donor nephrectomy patients, an overnight infusion of
fluids followed by a colloid bolus immediately before the
pneumoperitoneum attenuated the adverse hemodynamic effects and
reduced the magnitude of changes in creatinine clearance associated with
increased IAP.182 Comparable data in children have not been
forthcoming.

2984
Fluid administration during laparoscopic surgery should be carefully
monitored. Open abdominal surgery may require 10 to 15 mL/kg per
hour of balanced salt solution to offset third-space fluid losses from
extensive bowel manipulation. Although the existence of and
manipulation of a real “third space” are debated, the conceptual fluid
shift is real. During laparoscopic surgery, however, these fluid
requirements are reduced because little fluid is lost and the bowels are
minimally manipulated. In fact, care must be taken to avoid fluid
overload. Urine output is often used as an index of preload in children
undergoing abdominal surgery, but 88% of infants and 33% of children
develop anuria or oliguria during laparoscopic surgery. Because these
issues completely resolve within several hours of desufflation, fluid
challenge is not necessary in these children as fluid overload is a real
possibility.179 Transient oliguria in children after laparoscopic surgery
should not be viewed as an early indicator of impending renal
dysfunction.

Pain Management
Postoperative pain after open general and urologic surgery primarily
results from the skin and muscle incisions. With the small incisions used
during laparoscopic surgery, perioperative pain is less than with open
surgery.183–185 A systematic review of pain after laparoscopic antireflux
surgery in children reported mild to moderate pain in ~20% of children
and severe pain in 4%.186 Intraperitoneal administration of local
anesthetic has also been evaluated as a strategy to attenuate pain after
laparoscopic surgery. A recent systematic review of this strategy in
children concluded that there is some benefit from this approach
although the dose of local anesthetic is limited by the child's weight.
Additional studies are needed to fully assess the effectiveness of this
approach.187 To date, there have been a dearth of studies investigating
pain after laparoscopic surgery in children.
Pain after laparoscopic surgery arises from several sources, including
the incision sites, residual gas in the abdomen, referred pain from the
diaphragm, and stretch on nerves from peculiar patient positions. A
long-acting local anesthetic should be infiltrated around the incision sites
at the end of laparoscopic surgery to prevent postoperative incisional
pain. Some children develop pain after laparoscopic surgery, including

2985
back and shoulder tip pain. In these instances, multimodal pain therapy
including acetaminophen, nonsteroidal antiinflammatory agents, and
(less commonly) opioids are effective.104,105,126 Recent evidence suggests
that SIMPL for appendectomy causes less pain than surgery with a
multiport system.188

Robot-Assisted Surgery
Robot-assisted surgery is relatively new to pediatric surgery and urology,
with only limited published experience, although it has been used
extensively in adults since the late 1990s to facilitate minimally invasive
endoscopic surgery. Enhanced with three-dimensional magnifying views
and feedback-controlled enhanced motions of human hands, robot-
assisted surgery enables very fine manipulation of surgical instruments
while eliminating natural human tremors. It has great potential as the
future direction for pediatric surgery and urology.1,169,189–194
Robotic surgery was initially introduced as a tool for remote battlefield
surgery. It is now available to assist pediatric surgeons in performing
complex surgery, with less tissue and organ damage on extremely small
surgical targets. Most of the information currently available is limited to
one product (da Vinci Surgical System, Intuitive Surgical, Sunnyvale,
CA) and to adult urologic procedures. However, rapid innovation and
miniaturization of equipment has resulted in several pediatric centers
undertaking robot-assisted laparoscopic surgery, with excellent
outcomes (Videos 29.1 and 29.2 ). Concerns from the anesthesiologist's
perspective regarding this relatively new approach are summarized in
Table 29.1. Most of the perceived problems relate to the extended
duration of procedures and the steep Trendelenburg position used,
although numerous surgeries have been performed without this
position.169,189 Published evidence with robot-assisted surgery has
yielded outcomes comparable to laparoscopic surgery in children,
prompting many to question whether this new and expensive surgical
venture is justified.195–197 However, rapidly advancing technology offers
enormous opportunities for robot-assisted surgery in children in the very
near future, for very fine and technical dissection in small and
constrained spaces.198
TABLE 29.1

2986
Issues Regarding Current Robotic-Assisted Procedures From the Anesthetist's Perspective

Dependence on Extreme Body Position, Such as Extreme Trendelenburg Position (Because of


Absence of Adequate Surgical Assistance for Surgical Field Exposure), May Lead to:

Increased intracranial pressure, ocular pressure, and impairment of cerebral perfusion

Optic nerve or retinal morbidity

Cerebrofacial congestion, airway edema, vocal cord palsy, delayed awakening

Possible overstretching of nerves passing through the axilla and nerve plexus damage

Extended lithotomy leading to compartment syndrome in the lower legs

Tendency to apply greater intraabdominal pressure

Circulatory depression and respiratory depression

Carbon dioxide insufflation related complications

Absence of Touch, Traction, and Compression Sensations of Holding Instruments or Tissues:

All the manipulations are dependent exclusively on the visual sense

Inability to know the events occurring in an invisible place, overt tissue damage

Possibility of overlooking overt bleeding or tissue damage

Absence of Robot Legs and Easy Movability:

Once fixed, it is hard to change the position of the robot or the child's position

Difficulty in checking IV access sites and the airway

Unlimited and unexpected surgical approaches

Exploration of new surgical approaches and complex tasks at awkward angles

2987
Greater surgical and setup times, hypothermia, and pressure sores

Unpredictable movement of robot and camera arms

Hitting or compressing the child's face

Organization of all tubing and cables, including intravenous tubing and breathing hoses.

Need for Emergency Undocking of the Robot in Case of Mechanical Failure or Critical Events

Specific General Surgical and Urologic


Conditions
Nissen Fundoplication
This surgery is indicated for children with documented gastric fluid
reflux for whom medical management failed. It involves mobilizing the
muscles around the esophagus and suturing them tightly around the
esophagus at the level of the lower esophageal sphincter. This surgery
requires general anesthesia and tracheal intubation and is usually
performed laparoscopically, with increasing application of robot-assisted
technology.199
Children who require a Nissen fundoplication often have a neurologic
injury (i.e., cerebral palsy) that causes esophageal dysmotility. This
dysmotility heralds esophageal reflux that, if severe, may result in
recurrent aspiration pneumonia. If medical and gastric tube therapies
fail, a Nissen fundoplication may be considered. The anesthetic
considerations are few because surgery is not associated with
postoperative pain, large fluid shifts, or large blood loss. Positioning a
bougie within the esophagus during surgery allows the surgeons to
gauge how tight to tie the muscles around the esophagus; without a
bougie, the muscles around the esophagus may be overtightened,
causing an esophageal obstruction. Care must be taken to avoid
dislodging the tracheal tube while manipulating the bougie. At the end
of the procedure, it is common for the surgeon to request that 50 to 60 mL

2988
of air be insufflated into the stomach via the gastric tube to ensure there
are no anastomotic leaks. In general, surgery is completed in less than 1
hour in experienced hands, with a complication rate of about 10% and an
average hospital stay of about 1.6 days. Postoperative pain is usually
easily managed; however, open procedures usually require continuous
analgesia for 2 to 3 days.200,201

Pectus Excavatum
Although this is a deformity of the chest wall, pediatric surgeons most
often carry out the corrective procedure. The classic approach to
correcting a pectus excavatum involves an open procedure with fracture
of the sternum, removal of multiple costal cartilages, and elevating the
sternum with fixation, using one or two stainless steel bars. The Nuss
procedure is a less invasive technique,202,203 whereby a U-shaped bar is
blindly passed through the thorax hugging the undersurface of the
sternum. Once across the chest, the bar is flipped, through which process
the sternum is pushed anteriorly without fracturing it, thus avoiding the
creation of a flail chest by the removal of the costal cartilages. This
procedure has since been modified, whereby the bar is passed through
the thorax under direct vision, using thoracoscopy to reduce possible
perforation of major structures (e.g., the heart or lungs).204–206 Although
the risk of this complication is reduced when the Nuss procedure is
performed under direct vision, it may still occur.207–209 Both blind and
thoracoscopic approaches cause significant postoperative pain, which
may be treated with patient-controlled analgesia, a thoracic epidural
catheter, or a lumbar epidural catheter and epidural morphine (see
Chapters 42-45).210–212 Because these procedures are generally performed
in teenagers, the thoracic epidural is preferably placed with the teenager
awake but sedated. Compliance with inserting the epidural catheter
while awake may be difficult in less mature teenagers. It is unclear
whether the thoracic epidural provides improved analgesia compared
with standard patient-controlled analgesia for this procedure.213 It should
be noted that these children will return for removal of the pectus bar
after several years. Occasionally, the bar has become adherent to the
pericardium or lung, resulting in a severe, sudden, and catastrophic
rupture of a major vessel or chamber in the heart when the pectus bar is
removed.214 It would be prudent to establish ample IV access to provide

2989
the means to rapidly transfuse fluids and blood should a catastrophic
blood loss occur.

Circumcision
Globally, the prevalence of male circumcision is 40%, with half of these
surgeries performed for religious or cultural reasons.215 Circumcision is
performed in neonates, infants, children, and adults with local, regional,
or general anesthesia. The indications for circumcision include phimosis,
recurrent balanitis, religious beliefs, and parental preference. Inhalational
anesthesia supplemented by a regional block is preferred. Classic
circumcision involves cutting the foreskin and cauterizing and suturing
the skin edges. The duration of surgery is usually less than 1 hour. The
type of anesthetic and airway management do not significantly affect
perioperative outcomes. The most common complication arising from
circumcision is bleeding.
In infants and children, circumcision is performed with the patient
under general anesthesia. Multimodal pain therapy includes
acetaminophen (e.g., 10–15 mg/kg orally or 30–40 mg/kg rectally before
starting surgery) or 10 to 15 mg/kg IV, parenteral opioids (i.e., morphine
0.05–0.1 mg/kg), and/or local anesthetic without epinephrine (dorsal
penile block, caudal block, subcutaneous ring block, and topical
lidocaine-prilocaine [eutectic mixture of local anesthetics (EMLA)]) (see
Chapter 42).216 In a comparative study, suprapubic penile block provided
better analgesia than subcutaneous ring block of the penis.217 When a
caudal block was compared with penile blocks and parenteral analgesics,
a Cochrane review concluded that both rescue analgesia and nausea and
vomiting were comparable with all three techniques, although the
analysis was limited because of small numbers and poor
methodology.218,219

Hypospadias and Chordee


This congenital malformation occurs in 1 of 250 liveborn males. It often
occurs in isolation, without other congenital anomalies. Hypospadias
refers to a malposition of the meatus of the urethra: rather than opening
at the distal tip of the penis, the urethra opens along the undersurface of

2990
the penis anywhere from just proximal to the glans to the scrotum (Fig.
29.5). The majority of hypospadias defects are distal, occurring near or at
the glans of the penis. Between 15% and 50% instances of hypospadias
have an associated chordee, whereas 8% have an undescended testis. A
small number of children with hypospadias have urethral openings
remote from the glans of the penis, including the scrotum (Fig. 29.6; E-
Figs. 29.4 and 29.5).

FIGURE 29.5 Classic hypospadias. Saline solution is


injected to perform an erection test before surgical
correction. (Courtesy Dr. P. Williot, Pediatric Urology, Women and Children's
Hospital of Buffalo, Buffalo, NY.)

2991
FIGURE 29.6 Scrotal hypospadias with the urethra
opening in the midline of the scrotum. (Courtesy Dr. P. Williot,
Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo, NY.)

E-FIGURE 29.4 A penoscrotal hypospadias with the


urethra opening at what is the fusion of the penis and the
scrotum. (Courtesy Dr. P. Williot, Pediatric Urology, Women and Children's
Hospital of Buffalo, Buffalo, NY.)

2992
E-FIGURE 29.5 Urethral hypoplasia and hypospadias.
The ultrathin tissue of the urethra presents a surgical
problem for providing sufficient tissue to correct the
hypospadias. (Courtesy Dr. P. Williot, Pediatric Urology, Women and
Children's Hospital of Buffalo, Buffalo, NY.)

Surgery is undertaken with an expected duration of between 1 and 4


hours depending on the severity of the hypospadias. It is important to
establish an understanding with the urologist of the type of regional
block that will suit the extent of surgery: those requiring a minor
hypospadias repair (single-stage procedure—that is, meatal
advancement and glanuloplasty technique [MAGPI]) or Mathieu repair
may be managed with a face mask, LMA, or tracheal tube. The anesthetic
prescription is at the discretion of the anesthesiologist; the children are
outpatients and receive either a penile block or a single-shot caudal
block. Those with more extensive hypospadias who require longer
surgery will require either an LMA or a tracheal tube. They may be
admitted to a hospital for one to two nights and need a strategy for
continuous postoperative analgesia. For the latter, either a caudal or a
lumbar epidural catheter may be inserted after induction to reduce the
anesthetic requirements and provide postoperative analgesia. If opioids
are avoided, a caudal-epidural block consisting of only local anesthetic
(e.g., bupivacaine 0.125–0.175% plain) does not delay micturition after
the urinary catheter has been removed.220

2993
Cryptorchidism and Hernias: Inguinal and
Umbilical
These surgeries, together with hydrocele repair, are common outpatient
procedures. Orchiopexy refers to mobilizing the undescended testis that is
either in the inguinal canal or, less commonly, within the abdominal
cavity (Fig. 29.7), and securing it firmly in the scrotum. Approximately
33% of preterm infant males are born with one undescended testis,
whereas only 3% of full-term males are similarly affected. Although the
incidence of undescended testis decreases to 1% by 3 months of age, the
incidence remains at 1% thereafter. Cryptorchidism usually occurs in
isolation, although it is associated with a number of conditions, including
Prader-Willi syndrome, Noonan syndrome, and cloacal exstrophy.

FIGURE 29.7 Intraabdominal testis in the hernia ring,


discovered during laparoscopy for an undescended
testicle. (Courtesy Dr. P. Williot, Pediatric Urology, Women and Children's
Hospital of Buffalo, Buffalo, NY.)

Undescended testes are categorized, based on physical examination, to


include testes that are truly undescended, those that are ectopic, and
those that are retracted. The retracted ones are not true undescended
testes because they can be massaged into the scrotum and require no
further treatment. In the case of true undescended testes, the testes must
be located, mobilized, and then fixed within the scrotal sac to ensure
viability. Failure to mobilize the testes out of the inguinal canal or
abdomen may result in atrophy, torsion, testicular cancer, or hernias.

2994
An inguinal hernia in a child is a congenital failure of the processus
vaginalis to obliterate. In this case, a loop of bowel protrudes beyond the
internal ring, causing a bulge in the inguinal region or scrotum. These
protuberances may appear periodically, with complete resolution in the
interim. On occasion, a small sac of fluid is present in the scrotum,
known as a hydrocele, and is confused for a loop of bowel in the scrotum
(E-Fig. 29.6). Hydroceles are removed electively with the same approach
as for an inguinal hernia. On occasion, the loop of bowel does not reduce
spontaneously from the hernia and remains trapped in the canal,
necessitating a visit to the emergency department. A surgeon is often
required to manually reduce the trapped bowel. In these cases, the hernia
repair is then scheduled as an urgent or elective surgery, depending on
whether there is suspicion of persistent or potential recurrent ischemia to
the bowel. In some cases, the entrapped bowel cannot be reduced and an
incarcerated hernia or obstructed bowel is diagnosed. Incarcerated
hernias and bowel obstructions are surgical emergencies that require
general anesthesia and muscle relaxation to reduce the strangulated
bowel. The emergency nature of the surgery requires careful questioning
regarding the time interval between the last meal and the onset of
abdominal pain and strangulated bowel. It is usually assumed that these
children have a full stomach. At the time of open reduction, if the bowel
does not appear to have adequate perfusion, a segment of the ischemic or
necrotic bowel may have to be resected.

2995
E-FIGURE 29.6 Right hydrocele in the scrotum,
evidenced by the swollen right sac. (Courtesy Dr. P. Williot,
Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo, NY.)

Management of cryptorchidism and inguinal hernia requires general


anesthesia (face mask, LMA, or tracheal tube) and a pain management
strategy. When the surgeon pulls on the foreskin, hernia sac, or testis
during surgery, laryngospasm may occur if the depth of anesthesia is
inadequate. Anesthesia can be deepened most rapidly by an IV bolus of
propofol; increasing the inspired concentration of inhalational anesthetic
may also deepen the anesthetic provided gas can be exchanged.
Multimodal pain therapy, as described earlier, may be used together
with a regional block. Regional blocks (ilioinguinal, iliohypogastric,
scrotal block; caudal-epidural block; or transversus abdominis plane
block) are used for both orchiopexy and inguinal hernia surgery, using
either a landmark-based or ultrasound-guided method (see Chapters 42
and 43).221
An umbilical hernia is a 1- to 5-cm defect in the anterior abdominal wall
(usually halfway between the umbilicus and the xiphisternal junction),
with intermittent protuberance of bowel through the defect. This defect
occurs in 15% of children, more commonly in children of African rather
than European descent, and equally in both sexes. It also occurs
frequently in preterm and low–birth-weight infants. Many resolve
spontaneously in the first year of life, but those that persist require
surgical closure. If the defect is small, then an LMA may be sufficient,
provided a deep level of anesthesia is maintained when the suture
needles pass through each side of the rectus muscle. If the defect is large,
tracheal intubation and, depending on the surgeon, muscle relaxation
may be required. A deep level of anesthesia with an inhalational
anesthetic or an IV bolus of propofol (1–2 mg/kg) while the defect is
closed usually provides sufficient relaxation to reduce the defect in most
cases.

Torsion of the Testis


Presentation of a male with sudden onset of acute scrotal pain in the
absence of trauma requires immediate investigation and possible surgery
to preserve a potentially viable testis. The differential diagnosis of acute

2996
onset of torsion of the testis (Fig. 29.8) includes torsion of the testicular
appendix, torsion of the spermatic cord, epididymitis, and incarcerated
hernia. The majority of testes can be saved if surgery is performed within
6 hours of the onset of pain if the diagnosis is confirmed by Doppler
ultrasonography or suspected on clinical grounds.222 The salvage rate for
the testis decreases to 50% if surgery is undertaken 6 to 12 hours after the
onset of pain. Children with suspected acute testicular torsion should be
considered “an acute abdomen” and assumed to have a full stomach and
require RSI and tracheal intubation. Although pain at the time of
induction of anesthesia may be intense, when the torsion is relieved, the
pain abates. Hence, at the conclusion of surgery many of these children
no longer have substantive pain that warrants aggressive treatment.

FIGURE 29.8 Torsion of the newborn testis. (Courtesy Dr.


Daniel P. Doody, MD, Pediatric Surgery, MassGeneral Hospital for Children,
Boston.)

Posterior Urethral Valves


Posterior urethral valves (PUV) is a spectrum of urethral obstruction that
varies from mild to severe. The diagnosis is often made antenatally
(preferably by 24 weeks gestation) by ultrasound identification of
bladder distention, megaureters, and hydronephrosis. When PUV is
diagnosed antenatally, the severity of the disease tends to be greater.223
Decompression of the urogenital system may be achieved by a
vesicoamniotic shunt in utero. Although some recommend that an
intervention should be undertaken as quickly as possible to minimize the

2997
impact on renal function, evidence suggests that early intervention does
not affect the outcome, because renal damage may have already occurred
in utero.224,225
Postnatally, a lack of or decrease in urine output, urinary retention, or
a poor urine stream may be the only indications of the presence of these
valves.226 Affected children can have associated renal insufficiency
resulting from congenital renal dysplasia and urethral valve obstruction.
Because the renal concentrating mechanism is often impaired, these
infants commonly present with greater than normal urine output.
Consequently, careful monitoring of urine output and balanced salt
solution infusion rate is necessary. Primary valve ablation is required to
decompress the urogenital system.
Several indexes have been postulated as predictors of poor long-term
renal function in infants with PUV, including a creatinine value of 0.8
mg/dL or greater at birth, antenatal diagnosis, proteinuria, moderate or
severe hydronephrosis, and renal dysplasia.223,227 One study indicated
that a nadir creatinine greater than 1 mg/dL and bladder dysfunction
were the only independent predictors of long-term renal dysfunction.228
Children with PUV are scheduled for elective surgery. A general
anesthetic is required, with the specific management left to the discretion
of the anesthesiologist; there are few special considerations needed.

Prune-Belly Syndrome
Prune-belly syndrome is a disorder that occurs predominantly (97% of
the time) in males, with an incidence of 1 in 40,000 births. Affected
infants present with a range of findings, from stillborn to a full-term
neonate, with a host of possible organ and chromosomal
abnormalities.227,229 Affected organs may involve orthopedic in 50%
(congenital hip dislocation and scoliosis), gastrointestinal in 30%
(malrotation and volvulus), congenital heart disease in 10% (tetralogy of
Fallot and ventricular septal defect), and chromosomal defects (trisomy
18 and trisomy 21).227
In utero, the child's abdomen often swells with fluid (in the presence of
oligohydramnios) that is resorbed by birth, leaving the characteristic
wrinkled redundant abdominal wall (Fig. 29.9 and E-Fig. 29.7). The
pathophysiology of this syndrome is unclear, but it has been suggested

2998
that a urethral obstruction in utero leads to dilatation of the urethra
(megaurethra is a common finding), which, combined with bladder
distention and ascites, causes distention of the abdomen in utero. This
ultimately leads to vesicoureteral reflux and ureteral dilatation in 80% of
affected children.

FIGURE 29.9 Prune-belly syndrome. View of the


distended, weak muscled abdomen; the muscle wall is
so thin that the abdominal surface is bulging in front of
the bowels. (Courtesy Dr. P. Williot, Pediatric Urology, Women and Children's
Hospital of Buffalo, Buffalo, NY.)

E-FIGURE 29.7 View of the distended, weak-muscled


abdomen in a child with prune-belly syndrome. The
muscle wall is so thin that bowel peristalsis may be
visualized on the abdominal surface. (Courtesy Dr. P. Williot,

2999
Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo, NY.)

Abdominal overdistention in utero causes weak rectus abdominis


muscles that undermine the child's ability to exhale forcefully and to
generate a strong cough to clear secretions. As a result, chronic aspiration
pneumonia may contribute to an early demise. Some have suggested that
aggressive intervention to correct the weak rectus muscles by plication
and muscle transfer may improve respiratory function,230,231 reduce back
strain and pain, decrease bladder volume, and arrest scoliosis.232
However, this view is not shared by others who prefer to observe the
child for signs of regurgitation and aspiration before intervening.233
Controlling the type of feeds, preventing gastrointestinal reflux disease
and constipation, and using antibiotics to treat pneumonia permit the
child to grow. Pneumonia must be aggressively treated and completely
resolved before entertaining surgery. Percutaneous endoscopic
gastroscopy tubes are generally eschewed in these children when feeding
is a problem, as abdominal wall surgery is difficult. These children often
require urologic surgery to correct vesicoureteral reflux and orchiopexy.
Urethral obstruction may be due to the angulation of the urethra within
the prostate.
Trisomy 18, the second most common autosomal trisomy, occurs in 1
in approximately 7000 live births and is associated with prune-belly
syndrome. In contrast to the simple prune-belly syndrome, 60% to 80% of
these infants are female. Trisomy 18 is characterized by severe neurologic
developmental problems (including microcephaly), micrognathia and/or
retrognathia, microstomia, auricular abnormalities, and others. In fact,
95% die in utero, with only 5% to 10% surviving 1 year and only 1%
reaching 10 years of age. Mortality results from cardiac anomalies (90%
of affected children have a ventricular septal defect, valvular heart defect,
atrial septal defect, hypoplastic left heart syndrome, tetralogy of Fallot, or
other cardiac defect), renal anomalies, failure to thrive, and apnea.234,235
Additional findings include pulmonary hypoplasia and gastrointestinal
anomalies (including omphalocele, ileal atresia, and esophageal atresia).
General anesthesia with tracheal intubation is required for most
surgeries in children with prune-belly syndrome. Controlled ventilation
is recommended because of the variability in the strength of the
abdominal muscles. It is prudent to suction the lungs once the trachea
has been intubated to assess the severity of secretions. As little muscle

3000
relaxant as possible should be used during the surgery, with preferably
no neuromuscular blocking drugs administered during the last hour, to
ensure the child's muscles have had time to recover to maintain an
adequate tidal volume after extubation. Opioids should be used
cautiously to limit respiratory depression; regional anesthesia may be
preferred in view of their difficulty with clearing secretions from the
tracheobronchial tree.236

Ureteral Reimplantation
Vesicoureteral reflux, in which urine passes retrograde up the ureter, is a
congenital disorder affecting 0.5% to 2% of children.237 It occurs 10 times
more commonly in Caucasian than African American children, is more
common in male neonates but 5 to 6 times more common in females
younger than 1 year of age, and more commonly occurs in red-haired
children.237 There is a genetic component to this disorder with 34% of
siblings affected, although the pattern of inheritance is unknown.238
Recurrent episodes of pyelonephritis may occur, leading to renal scarring
and reduced renal function, depending on the severity of the reflux. The
pathology is thought to be an anatomically abnormal insertion of the
ureter into the bladder that fails to close tight when the bladder fills and
contracts. A voiding cystourethrogram is the definitive test to diagnose
reflux and assess its severity. Mild forms of vesicoureteral reflux are
managed with daily antibiotics until the child outgrows the reflux.
However, more severe forms or those who develop kidney infections
despite antibiotic therapy require surgical correction. The classic surgical
approach for vesicoureteral reflux is an open procedure in which the
affected ureters are reimplanted into the bladder wall, re-creating a
normal muscle flap valve.239 This involves a lower abdominal incision
and 2 to 4 hours of surgery. Postoperatively, the pain is often intense,
necessitating 2 to 3 days of continuous infusion of local anesthetic via an
indwelling caudal or epidural catheter.
More recently, laparoscopic techniques have been developed, with and
without robotic control, for reimplantation of the ureters.239 Preliminary
evidence suggests an excellent success rate (>90%) for this approach,240
although the time for the surgery is more than twice than with the open
technique and more complications were identified in children with small
bladders.

3001
In the past 20 years, the search for alternatives to surgery has spawned
a number of compounds to inject into the terminal submucosal tract of
the ureter to prevent reflux. Initially, polytetrafluoroethylene (Teflon;
Chemours, Wilmington, DE) was used but more recently a safer, more
durable polymer comprised of dextranomer and hyaluronic acid (Deflux;
Valeant Pharmaceuticals International, Bridgewater, NJ) supplanted
Teflon. In this way, surgeons create a swelling just inferior to the opening
of the ureter into the bladder that prevents urine from refluxing (Fig.
29.10, before and after injection of polymer). The technique is successful
in 80% to 100% of cases of grade 1-2 reflux after one injection and in 85%
of cases of grade 3-4 reflux after two injections (where the grade of reflux
was related to the ureterorenal involvement).241 Although this procedure
still requires general anesthesia, its advantages far outweigh its
disadvantages in that the duration of the procedure is very brief (usually
15–30 minutes), avoids an abdominal incision, causes no postoperative
pain, and it may be performed as ambulatory surgery. However, the
results of this injection technique continue to be monitored because long-
term outcomes and sequelae have not been determined.

FIGURE 29.10 A child with ureteral reflux. Preinjection (at


left) with the bladder orifice wide open with no valve to
prevent the backwash of urine up the ureter. Polymer
was injected into the submucosa of the bladder, raising a
mound to seal off the orifice of that ureter. Postinjection
(at right) with the orifice now appearing as a dimple on
the peak of the mound (arrow). (Courtesy Dr. P. Williot, Pediatric
Urology, Women and Children's Hospital of Buffalo, Buffalo, NY.)

Pyeloplasty
Ureteropelvic junction obstruction occurs in 0.1% to 0.2% of neonates,

3002
approximately twice as frequently in males than females. Pyeloplasty is
performed to decompress the renal pelvis either because of intrinsic
(congenital) or extrinsic (major vessel) compression of the ureter. A
distended renal pelvis is often diagnosed antenatally and, in some
instances, decompressed in utero by percutaneous nephrostomy. The
ureteric narrowing often occurs at the ureteropelvic junction, where the
ureter exits the renal pelvis. Surgery involves disconnecting the ureter at
the renal pelvis, reshaping it, and then reinserting it into the kidney. Such
surgery is often performed with the patient in the lateral or prone
position, with the table jackknifed. The duration of this surgery is
approximately 2 hours and has a success rate of approximately 95%.
Dissection is often retroperitoneal, but the incision is placed immediately
below the rib cage. This procedure has been performed laparoscopically
and with the use of robotics.115,242
Anesthetic considerations include the prone or lateral decubitus
position (complicated by placing the table in the jackknife position),
postoperative pain, and adequate fluid resuscitation. IV access should be
established in an upper extremity to maintain adequate atrial filling
pressures. If the table is placed in the jackknife position, it is very
important to measure the child's blood pressure before and after the table
is jackknifed, because venous return may become severely compromised,
necessitating reducing the extent of the jackknife and resuscitating the
child with fluid. For postoperative pain management, an indwelling high
lumbar or low thoracic epidural catheter is placed. We administer
lidocaine 2% with 1:200.00 epinephrine immediately after inserting the
catheter to set the block before the table is jackknifed. Thereafter a
bupivacaine or ropivacaine epidural infusion may be used.
The laparoscopic approach to pyeloplasty in children is relatively new.
In renal surgery, CO2 is insufflated in the retroperitoneal space to
provide surgical access.243 Because of the anatomic differences between
the retroperitoneal and intraperitoneal spaces, greater pressures may be
required to provide adequate surgical visibility. A mean retroperitoneal
pressure of 12 mm Hg increases PETCO2 and peak inspiratory pressures,
and decreases blood pressure.244 Early evidence indicates that
laparoscopic surgery yields outcomes similar to the open approach,
although the duration of surgery is approximately one-third greater.245,246
In part, this has been attributed to the learning curve of this technique.

3003
Evidence suggests that the laparoscopic approach decreases the length of
hospital stay and may decrease postoperative pain115,246 (see previous
discussion of pain management after laparoscopic surgery).

Nephrectomy
Indications for nephrectomy and partial nephrectomy in children include
nonfunctioning kidney, dysplastic kidney, urolithiasis, Wilms or other
tumor, end-stage renal failure (pretransplantation or to control
hypertension), hemolytic uremic syndrome, and polycystic disease.
Children who need a nephrectomy require a thorough preoperative
assessment in terms of history, physical examination, and laboratory
testing, depending on their underlying pathology. These children are
often anemic because of chronic disease and possibly decreased
erythropoietin concentrations. If the child is being staged for kidney
transplant, the nephrologists may prefer to avoid blood transfusions at
this time. If time is available and the patient is anemic, oral ferrous
sulfate and vitamin C should be commenced 3 to 6 weeks before surgery
(vitamin C increases gut absorption of ferrous sulfate) to increase the
hemoglobin concentration, particularly if erythropoietin
247,248
supplementation is planned. These children are often small in size
and this should be considered when preparing the anesthetic equipment.
Nephrectomies are commonly performed using a retroperitoneal
approach with the child in the lateral decubitus position and the table
jackknifed. The incision is usually large and located just subcostal to the
twelfth rib. If an open approach is planned, an epidural catheter may be
placed (see “Pyeloplasty” earlier). If, however, a laparoscopic or robotic-
assisted approach is planned,99 then a neuraxial block is not necessary
but the same surgical approach occurs. Outcomes after laparoscopic
nephrectomy in children are similar to those after an open procedure,
according to the experience from one center,249 although recent evidence
points to less pain and earlier discharge from the hospital.99

Neuroblastoma
The adrenal glands are located in the retroperitoneal space, adjacent to
the superior pole of the kidneys. Masses that arise in the adrenal gland

3004
may be tumors, hemorrhage, infections, or cysts. The tumors in the
adrenal glands may arise from the cortex (as in adenoma) or medulla
(including the sympathetic chain, as in neuroblastoma or
ganglioneuroma) and may be either hyperfunctioning or nonfunctioning.
Neuroblastoma is the most common extracranial solid tumor that
presents in childhood, representing 10% of all tumors and 15% of all
deaths from tumors.250 It is the second most common abdominal tumor
after Wilms tumor. Its incidence is approximately 1 in 100,000 children in
the United States.251,252 Neuroblastomas arise in the abdomen in 75% of
cases and along the sympathetic chain anywhere from the neck to the
pelvis in 25%. Only one-third of those that arise in the abdomen arise in
the adrenal glands. The median age at presentation is 2 years, with up to
90% occurring in children younger than 5 years with equal sex
prevalence. In some series, up to 50% of cases appear in the first month of
life, with some diagnosed antenatally; reports of familial neuroblastoma
are rare.250 However, these tumors may be associated with other
disorders, including Beckwith-Wiedemann syndrome,
neurofibromatosis, Hirschsprung disease, and central hypoventilation
syndrome.250
Most neuroblastomas are first detected as palpable masses in the
abdomen. Symptomatic presentation may present as local pressure on
adjacent organs or structures (liver, kidney, or spine), as metastases
(lymph nodes, bone marrow, liver, and skin), or with manifestations of
excess neurohumoral production (that is, from catecholamines [e.g.,
systemic hypertension] or vasoactive intestine polypeptides [e.g.,
diarrhea]).250 Urinary catecholamines are increased in greater than 90% of
children (>1 year of age) with neuroblastoma. In a minority of instances,
the diagnosis is made through incidental examination, either by
radiography or ultrasonography.
Staging of these tumors follows two protocols.251 The International
Neuroblastoma Staging System depends on tumor resectability, lymph
node involvement, and metastases. However, if patients are not surgical
candidates, the staging score holds little relevance. To further stage these
tumors, a second scoring system was developed, the International
Neuroblastoma Risk Group classification system, which is based on the
preoperative radiologic findings only. Survival likelihood depends on a
low score, extraabdominal (as opposed to intraabdominal) primary

3005
tumor, and younger age. Therapeutic intervention is tailored to the
staging of the tumor at presentation and the size of the tumor. Those
with favorable tumor biology, no distant metastases, and age younger
than 18 months of age are often curable with surgical resection alone.
Those with less favorable tumor biology, metastases, and a large tumor
size that may present a challenge surgically are ideally treated with a
combination of chemotherapy, radiation, and/or bone marrow
transplantation to reduce the tumor size first and then with surgical
resection. Patients with less favorable biology present a challenge for
surgical resection because of local infiltration, large tumor size, and
vascular extension. Debulking the tumor has been met with mixed
reviews, with great success in low-risk patients but with less success
when one of several other therapies in high-risk patients.253 The most
promising treatments for neuroblastomas with poor prognoses include
molecular profiling of the tumor.251 The long-term survival after treating
these tumors is 88% for infants and children younger than 18 months,
49% for children 18 months to 12 years, and 10% for those 12 years and
older.254 Preoperative assessment requires a general systems review, with
particular focus on the organ systems involved with the tumor. These
tumors may present as a large intraabdominal mass that compromises
respiration, most commonly manifested as tachypnea. Those with
catecholamine-secreting tumors may also present with chronic
hypertension. This is mitigated to a large extent if the tumor is shrunk
preoperatively, but intraoperative hypertensive episodes still occur in
25% of the children (with urinary catecholamines) during surgery.
Children with catecholamine-secreting neuroblastomas should have both
α-adrenergic and β-adrenergic blockades established preoperatively (see
Chapter 27) to avoid swings in blood pressure during manipulation of
the tumor.255 Less frequently, chronic diarrhea from vasoactive intestine
polypeptides may cause chronic dehydration and electrolyte
derangements that require correction.
The anesthetic plan depends on the nature and extent of the surgery. A
general anesthetic with tracheal intubation and controlled ventilation, as
well as standard anesthetic monitors, is required. These should be
supplemented with invasive monitoring, including arterial and central
venous access for catecholamine secreting tumors, large tumors, and
those that are expected to bleed excessively. Anticipation of massive
blood loss will necessitate using large-bore upper extremity IV access,

3006
blood warmers, and possibly a rapid transfusion device. No specific
anesthetic regimen has been recommended for these surgeries.
Although the blood pressure may be controlled with established α-
adrenergic blockade, manipulation and squeezing of the tumor during its
removal may cause a surge in catecholamine release, necessitating the
use of antihypertensive medications intraoperatively (see Chapter 27).255
Labetalol may be effective in children with hypertension during resection
of neuroblastomas255; however, it may cause paroxysmal hypertension
and heart failure in children who have only β-adrenergic blockade
established because of the predominant β-adrenergic blocking action of
labetalol.

Wilms Tumor
Renal tumors represent 2.5% to 7% of tumors in children. Wilms tumor is
the most common abdominal tumor, with an incidence of 1 in 100,000
children younger than 15 years of age, and the most common solid renal
tumor beyond the first year of life.252,256 These tumors arise from
persistent immature parenchymal renal tissue (referred to as Wilms
tumorlet cells), often in the periphery of the kidney (as opposed to the
collecting ducts), enclosed by a pseudocapsule. They may achieve a large
size before detection, often compressing adjacent renal parenchyma (Fig.
29.11). Histologically, the tumor often includes up to three distinct tissue
cell lines: epithelial, blastemal, and stromal cells.256 The presence of
anaplastic cells (in 4% of Wilms tumors) and, more specifically, whether
the cells are focal or diffuse in the tumor, and older age at the time of
presentation suggest a less favorable response to chemotherapy and less
favorable long-term prognosis.256 With tailored multimodal therapy, the
survival from Wilms tumor in the past several decades has increased
dramatically, from 30% to ~90%.257

3007
FIGURE 29.11 A Wilms tumor was mobilized and
exteriorized from the abdomen. Note that it is
encapsulated and contiguous with the left kidney. (Courtesy
Daniel P. Doody, MD, Pediatric Surgery, MassGeneral Hospital for Children,
Boston.)

Eighty percent of the children with Wilms tumor present between 1


and 5 years of age (peaking at 3–4 years of age) with no gender or racial
predominance.252,256 Presentation of Wilms tumor is similar to that of
other intraabdominal tumors in the form of an incidental mass on
physical examination; approximately 6% are bilateral. Congenital
anomalies coexist with Wilms tumors in 12% of children, notably
genitourinary anomalies (5%), hemihypertrophy (2.5%), and aniridia
(1%).256 A number of genetic syndromes are associated with Wilms
tumors (e.g., Beckwith-Wiedemann, Fanconi syndrome, and Trisomy
18).252 Wilms tumors occur twice as frequently in children with horseshoe
kidneys than in those with normal kidneys and are also more frequent in
those with multicystic dysplastic kidneys.
Preoperatively, most children with Wilms tumors appear well,
presenting with constitutional findings including weight loss, loss of
appetite, and malaise; a palpable abdominal mass is present in 75% to
90%. Laboratory investigations before nephrectomy include routine
complete blood cell count, electrolytes, renal function, as well as
coagulation indexes. Polycythemia may be present from tumor-induced
excess erythropoietin production; acquired von Willebrand disease is
present in less than 10%.252 Microscopic hematuria occurs in 25%,
whereas overt hematuria is rare,256 suggesting tumor invasion of the
collecting ducts. Systemic hypertension is present in 25%, presumably as
a result of hyperreninemia. Preoperative investigations should include

3008
radiography and ultrasonography, the latter highlighting
hyperechogenic structures within the kidney. Tumor extension into the
ipsilateral renal vein and inferior vena cava must be examined before
embarking on surgery, as chemotherapy may lead to involution of these
tumors (Fig. 27.12).252 Magnetic resonance imaging and CT are valuable
tools for delimiting the tumor borders within the kidney, as well as
metastases in the lungs or other organs.252 Most recent summaries of the
radiologic investigations may determine the need for additional
interventions, including echocardiogram and lung scan, to determine the
presence of tumor in the heart and lungs, respectively. An
echocardiogram may be specifically required to evaluate myocardial
function if doxorubicin and other anthracycline chemotherapeutic agents
have been administered.252

FIGURE 29.12 Coronal view of the CT scan of a Wilms


tumor. Note the massive tumor enveloping the inferior
vena cava, as the vena cava is only visible where it
enters the liver in the upper midportion of the radiograph
(arrow). (Radiograph courtesy Daniel P. Doody, MD, Pediatric Surgery,
MassGeneral Hospital for Children, Boston.)

Anesthetic management of children with Wilms tumor is similar to


that with neuroblastoma. No specific anesthetic regimen is preferred. The
potential for massive and rapid blood loss must be anticipated and

3009
appropriate blood products should be immediately available. Invasive
monitoring and large-bore IV access (upper extremities should the tumor
extend into or compress the inferior vena cava) with adequate blood
warming capability is mandatory; a rapid infusion device should be close
at hand. Hypertension (precipitated by tumor handling), coagulopathy
(acquired von Willebrand disease), extension of the tumor into the
proximal inferior vena cava or right atrium, pulmonary tumor emboli,
acute right heart failure, and considerations concerning preoperative or
previous treatment with chemotherapeutic drugs are anticipated
potential complications during anesthesia.252 These drugs may impair
hepatic or hematopoietic function (actinomycin D), cause inappropriate
antidiuretic hormone release (vincristine), or myocardial damage
(anthracyclines) (see also Chapter 11).252 Postoperative pain may be
controlled using either IV opioids or regional anesthesia, though the risk
of a coagulopathy must be ruled out before placing an epidural block.

Bladder and Cloacal Exstrophy


Bladder exstrophy is a rare congenital anomaly of the genitourinary tract
occurring in 1 in 50,000 births with a 2 : 1 male/female ratio, more
common in Caucasians than non-Caucasians.258,259 It occurs as a failure of
the abdominal wall to close during fetal development and results in
defects of the anterior wall of the bladder and overlying midline
abdominal wall. Bladder exstrophy may present with a spectrum of
anomalies, including widening of the symphysis pubis, and genital
anomalies, such as epispadias, bifid clitoris, and undescended testes (Fig.
29.13). Some regard bladder and cloacal exstrophies as distinct entities,
whereas others consider them both extremes in a continuum of antenatal
defects.260 Cloacal exstrophy includes features of bladder exstrophy, plus
an omphalocele and spinal defects, and always includes imperforate
anus. Because the antenatal diagnosis of bladder exstrophy using
ultrasound may be technically difficult, and thus dependent on indirect
signs (absence of bladder filling, a low-set umbilicus, widening of the
pubic ramus, small external genitalia, or a lower abdominal mass), the
diagnosis is usually confirmed at birth when the anterior abdominal wall
defect with the exposed bladder mucosa are evident (Fig. 29.13).

3010
FIGURE 29.13 Bladder exstrophy in a neonate. Note the
open anterior abdominal wall, protuberant bladder,
distorted genitalia, and splayed hips.

Therapy is aimed at the surgical reconstruction of the bladder with


preservation of renal function while achieving urinary continence and
satisfactory appearance of the external genitalia.261,262 In a select group of
infants, surgical management may be carried out as a single-stage
procedure263; however the majority require a staged surgical repair
directed at closure of the bladder, posterior urethra, and abdominal
wall.264 In addition, a bilateral iliac osteotomy may be performed to
facilitate surgical closure, decrease the stress on the midline soft tissues,
and reduce the risk of postoperative wound dehiscence.
Wound dehiscence, bladder prolapse, and multiple attempts at bladder
closure are among the risk factors for decreased bladder growth and the
inability for the later development of continence.265 In addition to the
immediate postoperative complications, children who have undergone
surgical repair of bladder exstrophy carry an increased risk for the
development of renal, bladder, and colon adenocarcinoma.266 Children
who require frequent bladder catheterizations or repeat surgeries are at
risk for developing latex allergy if latex products are used66; fortunately
most bladder catheters exclude latex.
Surgical management of the epispadias is usually performed at 6 to 12
months of age, and reconstruction of the bladder neck by 5 years, the
latter to allow for bladder training.264 After the initial surgical repair,
children are immobilized for 4 to 6 weeks,267 with a successful outcome
likely when a modified Buck traction with an external fixator or a
modified Bryant traction268 and adequate postoperative analgesia are
used.269

3011
The preoperative assessment should evaluate the presence and
severity of all congenital anomalies, particularly cardiac abnormalities.
Signs or symptoms of renal insufficiency and electrolyte imbalance may
be present. If the child has renal insufficiency, the dose and frequency of
administration of potentially nephrotoxic drugs, as well as the commonly
used anesthetic drugs, must be carefully evaluated, particularly water-
soluble drugs. Typically, surgery is performed with the child in the
supine and lithotomy positions. The duration of surgery is often
prolonged, requiring alternating urologic and orthopedic teams for their
respective segment of the surgery, and repositioning the child depending
on the stage of the surgery, thus requiring judicious management of
fluids, blood loss, and temperature control. It is essential to communicate
with the surgical teams to ensure an optimal perioperative strategy for
balanced salt solutions, colloids, and blood products; a preoperative plan
for multimodal perioperative pain management and anticipation of
possible postoperative ventilatory support is essential.264 After applying
the standard monitors, anesthesia may be induced either by inhalation or
IV; after induction, at least one large-bore peripheral IV line should be
placed. A second IV line may be placed, or a central venous catheter and
possibly an arterial catheter. In general, if blood loss occurs, it is slow and
steady, but if posterior iliac osteotomies are performed, the blood loss
may become brisk, requiring aggressive fluid resuscitation and
administration of blood products. Beat-to-beat variability of arterial
blood pressure and serial laboratory evaluations are valuable during
goal-directed resuscitation. Appropriate padding, a fluid warmer, and a
forced-air heating system should be standard. The anesthetic prescription
varies with the age of the child and whether the surgery is completed in a
single or staged repair. Postoperatively, children are transferred directly
to the pediatric intensive care unit for recovery.
A general anesthetic in combination with an epidural or caudal
continuous infusion technique is often used if there are no associated
spine abnormalities and if the catheter can be positioned out of the
surgical field.270
Maintenance of anesthesia may be achieved using an inhalation
anesthetic and small doses of opioids such as a remifentanil infusion;
intermittent boluses (0.5–1 mL/kg) or continuous infusions of
bupivacaine (0.125%–0.25%) or ropivacaine (0.2%) with or without
epinephrine through a lumbar or caudal epidural catheter reduce the

3012
need for anesthetic agents. Caudal or epidural analgesia may be
provided by advancing the catheter to the appropriate surgical
dermatomal level after induction of anesthesia. In neonates, epidural
infusions of bupivacaine (0.2 mg/kg per hour) provide excellent analgesia
although blood concentrations of bupivacaine steadily increase by 2 days
(see later text). The catheter is either secured directly to the skin or
tunneled using the epidural insertion needle271; a continuous infusion of
a local anesthetic may be maintained for several days to provide
analgesia and a mild motor blockade that favors immobility. However,
care must be taken to avoid systemic toxicity. Neonates are at increased
risk for developing local anesthetic toxicity because of their low serum
protein concentration and metabolism. Albumin and particularly α1-acid
glycoprotein are reduced in this age group,272 which reduces protein
binding and increases the free fraction of bupivacaine and ropivacaine.
This free form is metabolized to a lesser extent in the neonate because of
immature metabolic pathways and contributes to an increased risk of
cardiac and systemic toxicity.273,274 It is for this reason that the upper limit
of infusion of bupivacaine or ropivacaine is limited to 0.2 mg/kg per hour
(for 48 hours), which is half that used in children older than 6 months to
minimize the risk of local anesthetic toxicity.275–277 Unfortunately, (free
unbound) serum bupivacaine concentrations are difficult to assay and so
lidocaine 0.1% (0.8 mg/kg per hour) or chloroprocaine may be preferable
(see also Chapters 42 and 44).
The outcome of this complex surgery is determined primarily by the
surgical expertise. Urologic incontinence, bladder prolapse, and
epispadias revisions require additional urologic surgery. Renal function
is usually well maintained throughout; complications of complete repair
are reported to be similar to those of the staged repair but usually
involve additional soft tissue defects.278

Bariatric Surgery in Children


Obesity has become a major threat to public health worldwide
(http://www.un.org/en/development/desa/population/pdf/commission/2010/keynote/
The prevalence of pediatric obesity has increased exponentially, affecting
every country over the past few decades. In the United States, the
incidence of obesity in children 2 to 19 years old has increased from ~7%

3013
in 1980 to ~18% in 2010279; one-third of U.S. children are either obese
(BMI ≥95th percentile) or overweight (BMI between the 85th and 95th
percentiles) (http://www.cdc.gov/obesity/data/childhood.html).280 The
causes of obesity are multifactorial: excess caloric intake, poor nutritional
choices, and lack of exercise. However, recent evidence suggests that
obese children are obese before the age of 5 years and that the underlying
causes may have their provenance in early infancy and childhood.281
Furthermore, the serious complications from obesity—for example,
arterial hypertension, cardiovascular disease, fatty liver, diabetes,
asthma, and obstructive sleep apnea (OSA)—are recognized earlier in
childhood,282 often serving as precursors for significant and intractable
obesity in adulthood.283 Multiple studies have reported an increase in
medical/surgical complications in hospitalized obese children.284 Thus
the societal burden of obesity is enormous (estimated at $147 billion
dollars in yearly health care costs)285 and mainly driven by a number of
associated comorbidities (e.g., diabetes, hypertension, sleep apnea, and
depression). If left untreated, obesity may result in earlier mortality.286,287
The degree of obesity can be quantified using several metrics, the most
common being the body mass index (BMI), defined as the weight (in
kilograms) divided by the height squared (in meters squared). In adults,
the degree of obesity can be defined by single BMI values (Table 29.2).
However, in children the degree of obesity is more difficult to quantify
because BMI and other indexes of growth increase nonlinearly with age
and gender. As a result, clinicians now define the degree of obesity in
children based on growth charts for weight-dependent age and gender
(Table 29.2).
TABLE 29.2

Obesity Indexes in Adults and Children

Classification of Obesity Adult (BMI) Children (Percentile on BMI Growth Chart)

Normal weight 18.5–24.9 5–85


Overweight 25–29.9 85–95
Obesity 30–39.9 >95
Severe obesity ≥40 >120% of the 95th percentile

Based on http://www.cdc.gov/obesity/childhood/basics.html and Pan L, Blanck


HM, Sherry B, et al. Trends in the prevalence of extreme obesity among US

3014
preschool-aged children living in low-income families, 1998–2010. JAMA.
2012:308(24);2563–2565.288

To address these worrying statistics, bariatric surgery has emerged as


one means to help the most extreme cases of adolescent obesity,
especially those who are motivated toward weight loss and are
psychologically prepared.285,289 Bariatric surgery is designed to either
bypass part of the small bowel to reduce caloric absorption (Roux-en-Y
procedure) or to minimize the size of the stomach (laparoscopic vertical
sleeve gastrectomy [LSG]). LSG has replaced the more complex Roux-en-
Y gastric bypass surgery as the most commonly performed surgical
procedure for obesity in the United States.290 These surgeries have
proven to be safe, cost-effective means of achieving permanent weight
loss and providing partial resolution of comorbidities, especially diabetes
in adolescents.291–294

Multidisciplinary Approach to Bariatric


Surgery in Adolescents
Developing a successful approach to bariatric surgery in children
requires a multidisciplinary team with collaboration from many pediatric
subspecialists, including bariatric surgeons, anesthesiologists,
endocrinologists, gastroenterologists, cardiologists, pulmonologists,
psychologists, dietitians, and others.295,296 Only children who are
motivated to lose weight and who exhibit a positive psychological
approach to this intensive process should be admitted to these programs.

Anesthetic Implications of Obesity in


Adolescents
Obesity is associated with a number of organ disorders including
cardiovascular disease (hypertension, dyslipidemias),297 respiratory
(asthma and OSA),298,299 renal dysfunction,300 endocrinopathies (diabetes,
metabolic syndrome), liver dysfunction (nonalcoholic fatty liver disease,
and nonalcoholic steatohepatitis.301,302 Childhood obesity is associated
with early pathologic cardiovascular changes. Hypertension, previously
seen most frequently in children with renal disease, is increasingly

3015
identified in obese children. The risk of hypertension in obese children is
3-fold and increases as BMI increases. Cardiac risk factors in obese
children as young as 5 years of age have been described, including
hypercholesterolemia, hypertension, and hyperinsulinemia. LVH has
been identified in obese children as young as 10 years of age and is
common in adolescents presenting for bariatric surgery. Research is
underway to determine if changes in cardiac architecture and function
are reversible with weight loss.
Preoperative preparation for bariatric surgery is an involved and
prolonged process. A thorough and comprehensive preoperative review
and investigation of these organ systems is warranted before proceeding
with anesthesia. Once screening is completed, it is essential that the
children learn about appropriate nutritional and attitudinal principles for
achieving and maintaining weight loss. After evaluating the major organ
systems and optimizing organ dysfunction, a preoperative anesthetic
interview should be completed. During this evaluation, the
anesthesiologist should review the investigations and laboratory results,
ensure compliance of all medications and interventions (e.g., continuous
positive airway pressure [CPAP] device), and then describe the
anesthetic process with the patient and family in detail. Although these
children appear large in size, many are psychologically immature and
very anxious. Anxiolytic premedication should be offered on the day of
surgery. During the interview, the airway should be assessed (see later
text) and if a difficult airway is identified, supplementary airway
equipment should be available. Children with CPAP devices should be
advised to bring them on the day of surgery for possible use
postoperatively. Routine fasting instructions and urine pregnancy testing
for females who have reached menarche should be ordered or if
complete, the results documented. If the child is unable to walk to the
operating room, a wheelchair or stretcher rated for the child's weight
should be used. The upper extremities should be examined for IV access
as this may be difficult in obese patients.303 A vein finder or ultrasound
may be useful to establish venous access. In advance of undertaking
bariatric surgery, appropriately sized operating room tables should be
present. Operating room tables are rated for the patient's weight; for
normal size patients, they may be rated for a maximum of 500 (227 kg) or
600 pounds (272 kg) and for obese patients, 1000 (454 kg) or 1200 pounds
(544 kg). In all cases, the children should be strapped to the table to

3016
prevent the child from rolling off the table. Unlocking the bed and
shifting the table top while the patient is on the table may cause the table
to tip, even if the child's weight is within the table rating. This is more
likely to occur if the table top is shifted off-center, if the child is placed in
reverse orientation, or if a strong force is applied in one direction to the
table
(http://www.apsf.org/newsletters/html/2013/spring/07_tabletipdanger.htm
Designing appropriate drug dosing in obese children requires an
understanding of the scalars that may be used to estimate the correct
dose. Total body weight (TBW) consists of two compartments: fat-free
mass (FFM) and the fat mass (FM). In general, the former is the volume
into which hydrophilic drugs tend to be distributed, whereas the latter is
the volume into which lipophilic drugs are distributed. Because most
drugs have both lipophilic and hydrophilic properties, the pharmacology
of each drug (or category) must be considered to define its
pharmacokinetic properties. Unfortunately, few of the drugs used for
anesthesia in obese children have been studied, thus limiting the
evidence on which to base drug doses. The FFM, a measure similar to
lean body mass, may be considered the sum of the ideal body mass plus
the additional mass required to support the physical and metabolic
demands of the FM. The latter is derived primarily from an increased
muscle mass as well as minor contributions from increased vessel-rich
organ mass (heart, liver) and the fluid compartments. As the TBW
increases, the FM increases in proportion; FFM increases steadily up to a
BMI 40 but then plateaus thereafter.304
The ideal body weight (IBW) of a child can be obtained from tables,
graphs, or simple equations.305 Sample equations include

The lean body weight (LBW) may be estimated using a nomogram305


or from a simple equation:

Dosing is further complicated because the size metric used for a


loading dose, which depends on the volume of distribution, may differ
from that used for maintenance dosing or infusion rates, which depend
on clearance. Most anesthetic drugs are lipophilic and are theoretically
distributed in the TBW. However, drug doses for induction of anesthesia

3017
are not uniformly based on the TBW.306 Drugs used to induce anesthesia
are distributed into the central compartment and throughout the vessel-
rich group of organs (e.g., brain). Even though these drugs are lipophilic,
the induction dose is ideally based on the acute volume of distribution
(Vd), which is more appropriately based on IBW or LBW.307 LBW might
be a good-sized metric for the dose of propofol for induction of
anesthesia,308 but TBW is better for maintenance/infusion.309
Other considerations in obesity include changes in plasma proteins,
liver and renal functions, cytochrome enzyme activities,310 CO, and
regional blood flow.311 Obesity may compromise organ function;
dexmedetomidine clearance is reduced in obese adults.309 The
pharmacokinetics of drugs in obesity depend on the physicochemical
characteristics of the drugs. For the loading dose of drugs, if the Vd/TBW
ratio is reduced in obesity, then the drug is not distributed to the FM and
should be based on the LBW or IBW.304 In contrast, if the Vd/TBW ratio is
unchanged or increased in obesity, then the drug is predominantly
lipophilic and the dose should be based on TBW.312,313 For drugs
administered for maintenance, the dose should be based on its clearance.
If the clearance in obesity is unchanged or decreased, then the dose
should be based on LBW or IBW, whereas if the clearance is increased in
obesity, then the maintenance dose should be based on the TBW. The use
of cerebral function monitors (e.g., bispectral index in teenagers) greatly
simplifies propofol infusion dosing by allowing titration of dose to a
target effect.
Studies reporting the pharmacokinetics/dynamics of specific anesthetic
drugs in obese children are limited.306,314 Based on the best “available”
data, a summary of the doses for induction or maintenance of anesthesia
is presented in Table 29.3315; as more detailed evidence emerges, the basis
for the dose of the drugs continues to evolve.
TABLE 29.3

Dosage of Intravenous Anesthetics in Obese Children

Induction Dose Based Maintenance Dose Based


Drug
on on

Thiopenthal LBW

3018
Propofol LBW TBW
Synthetic opioids (fentanyl, alfentanil, and TBW LBW
sufentanil)
Morphine IBW IBW
Remifentanil LBW LBW
Nondepolarizing neuromuscular blockers IBW IBW
Succinylcholine TBW
Sugammadex TBW

IBW, ideal body weight; LBW, lean body weight; TBW, total body weight.
Reproduced with permission. Mortensen A, Lenz K, Abildstrøm H, Lauritsen TL.
Anesthetizing the obese child. Paediatr. Anaesth. 2011;21(6):623–629.

Inhalational anesthetic agents with low blood-gas solubility are ideal in


this setting. Rapid induction and emergence from anesthesia can be
achieved and facilitated in this population with agents such as desflurane
or sevoflurane combined with remifentanil.316 The context-sensitive half-
life of desflurane is superior to sevoflurane owing to lower solubility in
blood and tissues; thus the former facilitates a more rapid recovery than
the latter, particularly for surgeries longer than 2 hours when inhalation
anesthetics accumulate in fat.317 However, desflurane induces more
bronchoconstriction than sevoflurane; thus in children with asthma or
who smoke cigarettes, sevoflurane's bronchodilatory properties make it
preferable to desflurane.
Airway management should take into consideration several basic
principles. The presence of increased abdominal girth, shallow tidal
volumes, and supine position predispose to rapid desaturation once
anesthesia is induced. To preclude desaturation, patients should be
preoxygenated in a reverse-Trendelenburg position more than 25
degrees.318 This provides an adequate reserve of oxygen during
laryngoscopy and tracheal intubation, mitigating the risk of desaturation
and attenuating atelectasis. To view the glottis aperture, elevation of the
head in the sniffing position should be doubled (from normal distance of
7 cm) to ~14 cm. This may be achieved by combining the ramped position
with the usual head elevation (7 cm).319 The quintessential alignment that
must be ensured to successfully visualize the larynx in obese children is
that the tragus of the ear is at or above the level of the sternal notch in the
sniffing position.320 If the tragus is not elevated above the sternal notch,
then the head must be propped up further. An RSI is not usually
required in most obese children; those who have gastroesophageal reflux
are controlled medically. Gastric fluid volume is increased in obesity, but

3019
the fluid pH is not.321 Therefore the risk of pneumonitis should aspiration
occur is no greater than in a nonobese patient.
Ventilation may be a challenge in obese children, particularly during
laparoscopic surgery. The pulmonary challenges from laparoscopic
surgery are compounded by the large abdominal girth compressing the
basal lungs. Increased inspired oxygen concentration may prevent
hemoglobin desaturation but may lead to absorption atelectasis. Optimal
strategies for ventilation while avoiding barotrauma include low tidal
volumes (6–8 mL/kg based on LBW, not TBW), periodic alveolar
recruitment maneuvers, PEEP (8–15 mm Hg), a respiratory rate to
maintain normocapnia or permissive hypercapnia, particularly during
pneumoperitoneum), and a sufficient inspired oxygen concentration to
maintain an adequate saturation.322–324 The ventilation mode has little
bearing on the outcomes after anesthesia. At the end of surgery and after
extubation, the child should be positioned in the semirecumbent
position, administered oxygen by face mask, and monitored en route to
the recovery room. Postoperative pain may be managed with either
intermittent IV opioids or patient-controlled analgesia.
OSA is common in children who are morbidly obese. Many use nightly
CPAP devices, although obese adolescents exhibit poor compliance with
these devices.325 Given the known risks of OSA causing opioid
sensitivity, opioids should be administered in reduced doses ( – the
usual doses).326–328 Intermittent hypoxia commonly complicates the
postoperative period. Hence, patients should bring their CPAP or nasal
CPAP devices to the hospital for postoperative use. Children may be
discharged home after uncomplicated surgery on postoperative day
3.329–331
Drug dosing after gastric bypass surgery is poorly understood.332,333
After gastric bypass surgery, whether Roux-en-Y or the more popular
LSG surgery, rapid weight loss occurs. This often results in the resolution
of many comorbidities associated with morbid obesity such as systemic
hypertension, diabetes, and so on. However, failure to adjust drug
dosing in parallel with the weight loss, reduced gastric residence time,
reduced exposure to intestinal cytochrome enzymes (CYP3A4; e.g.,
increasing atorvastatin blood concentrations), and the resolution of
severity of the associated diseases may lead to relative drug overdoses
and the associated consequences.333 For example, after Roux-en-Y gastric

3020
surgery, approximately 50% of drugs taken orally have reduced area
under the concentration-time curve, whereas 25% have unchanged and
25% have increased area under the concentration-time curve.334 Oral
morphine kinetics after Roux-en-Y showed dramatically increased blood
concentrations within the first 2 weeks after surgery that persisted for at
least 6 months.335 The risks of an opioid overdose from the changes in
pharmacokinetics not accounting for the effects of comorbidities may be
substantial. In one meta-analysis, the range of postoperative to
preoperative drug exposure ratio extended from a 10-fold greater ratio
for penicillin to a 33% lower ratio for phenytoin and ampicillin.333 The
pharmacokinetics of midazolam after gastric bypass surgery in adults
yielded similar bioavailability after oral dosing but greater clearance one
year after bypass surgery.336
Currently, LSG surgery has eclipsed Roux-en-Y surgery in the United
States in popularity, including in the adolescence age group. LSG surgery
is not associated with the same extent of gastric dumping as Roux-en-Y
surgery, but gastric residence time and absorption with LSG is reduced.
Until practice recommendations are forthcoming, it seems prudent to
titrate all parenteral drugs to effect during the period of rapid weight loss
in particular and to adjust the doses of all oral drugs using therapeutic
drug monitoring and individual clinical responses.

Acknowledgments
The authors thank P.A. Lonnqvist, Yoichi Kondo, Yasuyuki Suzuki,
Richard Banchs, Takako Tamura, Reiko Hayashi, and Katsuyuki
Miyasaka for their prior contributions to this chapter.

References
1. Chandra V, Dutta S, Albanese CT. Robot-assisted pediatric
surgery. Saxena AK, Hollwarth ME. Essentials of Pediatric
Endoscopic Surgery. Springer-Verlag: Berlin; 2009:87–90.
2. Bricker SR, McLuckie A, Nightingale DA. Gastric aspirates after
trauma in children. Anaesthesia. 1989;44(9):721–724.
3. Schmitz A, Thomas S, Melanie F, et al. Ultrasonographic gastric

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