20.
EVA LUATION OF COEXISTING DISEASE
Jacob T. Arnett and Chien-Hsiang Chow
1. A 7-month-old female is referred for a transthoracic
echo due to a murmur that her pediatrician auscul-
tated on physical exam. A 0.4 cm ASD is visualized at
the center of the intra-atrial septum and is believed to
be an ostium secundum defect. The echo report shows a
pulmonary-to-systemic blood flow (Qp:Qs) of 1.2. The
most appropriate treatment at this time is:
A. Open surgical closure as right heart failure is likely
in the near future
B. Medical therapy with indomethacin
C. Observation
D. Percutaneous closure to avoid Eisenmenger’s
syndrome in the future
2. A 1-year-old-male with a history of Tetralogy of Fallot
presents for a sedated transthoracic echocardiogram.
His cardiac surgeon is in the process of surgical plan-
ning and needs a repeat echocardiogram. During an
IM induction the child becomes hypoxic and cyanotic.
You quickly flex his hips and knees and begin a phenyl-
ephrine infusion, in addition to a fluid bolus. His blood
pressure is stable but his oxygen saturations are slow to
increase. The next step in pharmacologic management
includes which of the following?
A. Small boluses of esmolol
B. Deepening the anesthetic by increasing the inhaled
anesthetic concentration
C. Starting a milrinone infusion to reduce pulmonary
artery pressures
D. Small boluses of epinephrine to increase systemic
vascular resistance and reduce pulmonary pressures
3. Which of the following best describes autism?
A. Psychiatric disorder characterized by culturally
inappropriate behaviors

B. Psychiatric disorder characterized by impairment in
development of culturally normal relationships
C. Organic disorder characterized by
developmental delay
D. Organic disorder characterized by seizures of varying
frequency
4. An 11-year-old male with a history of dyskinetic cere-
bral palsy is seen with his parents prior to placement of
a baclofen intrathecal pump. The mother relays that no
medications were taken for 2 days prior to surgery, as he
refused to take them. A peripheral IV is placed in the
induction area and dexmedetomodine is started as a pre-
medication for anxiolysis. Five minutes later the nurs-
ing staff requests your immediate presence. The child
is unresponsive and appears to be having a tonic-clonic
seizure. The most likely cause of the seizure is:
A. Side effect from dexmetetomidine
B. Electrolyte abnormality
C. Subacute meningitis
D. Medication withdrawal
5. Succinylcholine use is likely considered acceptable in
which of the following patients?
A. A 7-year-old male having an open-reduction, internal
fixation of a supracondylar humeral fracture whose
mother has a history of malignant hyperthermia
B. A 13-year-old female with a history of a 70%
BSA deep burn sustained 5 months ago having a
contracture release
C. A 9-year-old male with a history of spastic cerebral
palsy having an Achilles tendon lengthening
procedure
D. An 11-year-old male with notable
pseudohypertrophy of the calves scheduled for a
muscle biopsy under general anesthesia
6. A  9-year-old male with a history of cystic fibro-
sis is evaluated prior to laparoscopic appendectomy.
Preoperative labs show an elevated PT and INR but
normal PTT and platelet values. What is the most likely
cause of the elevated PT/INR?
A. Intestinal malabsorption
B. Warfarin use
C. Perioperative enoxeparin use
D. Hemophilia A
7. A  2-year-old male with a history of Trisomy 21 is
scheduled for an ophthalmic exam under anesthesia.
With regard to planning airway management, which of
the following statements regarding the airway manage-
ment is true?
A. Microglossia may make for a challenging direct
laryngoscopy.
B. Hypertonia may be present, causing upper airway
obstruction.
C. Macrognathia is a common finding.
D. Airway manipulation may be limited due to
potential for cervical spine instability.
8. A 3-year-old child with recurrent tonsillitis is evalu-
ated prior to a tonsillectomy. The parents report that the
child’s pediatrician noted a heart murmur at a recent
visit, but they have no other details. The mother relates
that the child is doing very well and she did not have any
concerns prior to the pediatrician’s visit. You observe
the child smiling and appearing comfortable in her
mother’s arms. When auscultating the heart, you appre-
ciate a II/VI systolic murmur at the left-lower sternal
border. The child moves in her mother’s arms and the
murmur becomes very faint and difficult to appreciate.
Which of the following is most likely true?
A. The child should be referred for an
electrocardiogram (ECG) and a transthoracic echo
prior to anesthesia.
B. The murmur may not be significant during activities
of daily living (ADLs) but will likely be an issue
during an inhalational induction when systemic
vascular resistance decreases.
C. The murmur is most likely not clinically significant
and she can proceed to surgery without further
diagnostic testing.
D. The murmur likely represents significant underlying
cardiac pathology; she should be sent to the
emergency room for admission and a complete
workup.
9. A 9-week-old male is scheduled for a pyloromyotomy
for pyloric stenosis. He was admitted 20 hours prior to

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the time of surgery and was resuscitated in the pediatric
intensive care unit (PICU). The PICU team reports his
electrolytes have normalized, and he appears euvolemic.
In reviewing the child’s labs you notice his hemoglobin
is 10.1 g/dL. This hemoglobin level:
A. Likely reflects an iron-deficient anemia from
malabsorption/chronic vomiting
B. Likely reflects a gastrointestinal bleed with acute
blood loss anemia, which will require blood
transfusions in the operating room
C. Likely reflects hemodilution from the IV fluid
resuscitation
D. Likely represents a normal lab finding for a
9-week-old male
10. A  9-month-old male is seen in preop prior to a
circumcision, which is being completed for cosmetic
reasons. His parents report a normal perinatal his-
tory with no major problems since the time of birth.
During a review of systems, his father reports that
he has been able to sit alone and recently showed the
ability to stand up. He babbles but has yet to speak
any words. He smiles and plays “peek-a-boo” with
his mother but does not seem to play with his twin
brother. The above developmental status of this child
is likely:
A. Normal and appropriate for his age
B. Globally abnormal and indicates
developmental delay
C. Motor development is normal but social
development is delayed
D. Social development is normal but motor
development is delayed
11. With regard to child and parental preoperative
anxiety and emotional stress in the preoperative period,
which of the following is true?
A. Children younger than 7 years experience less
anxiety than older children because they do not
understand the danger of anesthesia/surgery.
B. Midazolam given for preoperative anxiety gives
satisfactory anxiolysis and sedation in approximately
one third of children.
C. Discussion of specific anesthetic risks will
significantly increase parental anxiety in the
preoperative period.
D. Child temperament is generally not indicative of
preoperative anxiety and coping strategies.
12. A 4-year-old female has a broken arm and is sched-
uled for an open reduction internal fixation of the
humerus. During the preoperative evaluation the
mother requests that she be present during induction
of anesthesia. What could be done to adequately pre-
pare the mother for her presence during the induction
of anesthesia?
A. Nothing, it is not routinely recommended
B. Explaining that the most upsetting areas for parents
are when the child goes limp and when the parent
has to leave the child
C. Telling the mother that she should be quiet during
induction and not to distract her daughter as she
goes to sleep
D. Explaining to the mother that studies have shown
unequivocally that pediatric patient anxiety is lowest
with a parent present during induction
13. An 8-year-old male with an anterior mediastinal
mass comes to the preop clinic for preoperative evalu-
ation prior to a mediastinoscopy. On evaluation the
patient is able to tolerate lying supine, but you notice
that he has distended neck veins, facial swelling, and
has been complaining of difficulty breathing. Which of
the following test/s should be ordered in the evaluation
of this mass?
A. Exercise electrocardiogram
B. Gastric motility study
C. Positron emission tomography (PET) scan
D. Pulmonary function tests
14. A  12-year-old male with an anterior mediastinal
mass is scheduled for a mediastinoscopy. On evalua-
tion the patient is able to tolerate laying supine and the
patient’s preoperative echocardiogram shows no signs
of vascular compression. Which of the following is the
most appropriate technique to induce anesthesia in this
patient?
A. Rapid sequence induction with cricoid pressure
B. Intravenous induction with propofol
C. Inhalational induction
D. Intravenous opioid-based induction for
hemodynamic stability
15. An 8-month-old with a history of spina bifida
occulta is scheduled for a repair of a umbilical hernia.
During the initial history the patient’s family states
that the child has had rashes after dental procedures.
During the procedure the child suddenly develops
hives, becomes hypotensive, and difficult to ventilate.
What type of hypersensitivity reaction is responsible
for the patient’s symptoms?
A. Type 1 hypersensitivity reaction
B. Type 2 hypersensitivity reaction
  
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C. Type 3 hypersensitivity reaction
D. Type 4 hypersensitivity reaction
16. Neonates and infants with gastroesophageal reflux
disease (GERD) can present with which of the follow-
ing symptoms?
A. Prolonged PT/INR
B. Failure to thrive
C. Renal insufficiency
D. Rhinorrhea
17. An 8-month-old infant presents to the hospital
for repair of an inguinal hernia. Other than moderate
GERD, the patient is healthy but was born at 29 weeks
gestation. Which of the following would be appropriate
premedication and/or intervention in this child prior to
induction of anesthesia?
A. Proceeding with surgery 4 hours after breast milk
B. Famotidine
C. Pantoprazole
D. Magnesium
18. Which of the following is most consistent with
asthma and management of asthma?
A. Wheezing is produced by a combination of upper
airway inflammation and bronchospasm.
B. Appropriate therapy includes bronchodilators,
anti-inflammatories, mast cell stabilizers, and
leukotriene inhibitors.
C. Immunoglobulin D is associated with the disease
process.
D. Manifestations of the disease include accessory
muscle use, coughing, and prolonged inspiration.
19. A 1-year-old is to undergo a laparoscopic Nissen fun-
doplication in the morning. Three hours prior to surgery
the patient was given formula by her parents. What is the
appropriate fasting interval for this particular situation?
A. 2 hours
B. 4 hours
C. 6 hours
D. 8 hours
20. Which of the following decreases the risk of respi-
ratory complications in the setting of upper respiratory
infection?
A. Delaying surgery 3 weeks
B. Using an LMA in place of an endotracheal tube
C. Preoperative beta agonists (albuterol)
D. Cavity, head, or neck surgery
21. Select the statement most true regarding anesthetic
risk:
A. Risk for both major and minor adverse event in
pediatric patients exceeds that for adult patients.
B. Nausea is the most common adverse event in all
pediatric patients.
C. Children between the ages of 1 and 12 months are at
greatest risk for major adverse event among pediatric
patients.
D. Children between the ages of 1 and 12 months
undergo cardiac surgery more frequently than other
pediatric patients.
22. Please select which of the following qualities is true
in the context of pediatric patient consent?
A. Children younger than 7 years of age are typically
capable of assenting due to their decision-making
ability.
B. Between the ages of 7 and 14 years, children reliably
develop decision-making skills.
C. Children above the age of 14 years may reliably hold
adequate decision-making skills.
D. Children above the age of 14 years have developed
the cognitive and emotional skills required to fully
participate in the process of consent.
23. A 14-year-old male presents to a preoperative clinic
2 weeks prior to a planned knee arthroscopy. The
patient had myringotomy with drainage tubes placed at
2 years of age without incident. The patient has no med-
ical issues aside from his knee. What would be the most
appropriate assessment tool?
A. Complete blood count and basic metabolic panel
B. Complete blood count and electrocardiogram
C. Complete blood count
D. No lab assessment
24. Select the answer which is most consistent with
parental presence on induction of anesthesia (PPI).
A. PPI is absolutely contraindicated above the patient
age of 18 years.
B. PPI is a benign process for the parent.
C. A possible benefit of PPI is reduction in need for
preoperative sedation.
D. A possible benefit of PPI is reduction of parent anxiety.
25. Choose the correct statement regarding psychoso-
cial preparation of pediatric patients preoperatively.
A. Children aged 5–10 years receive little benefit from
preparation programs aimed at enhancing coping
skills.
 
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B. Children under the age of 3 years receive a great
deal of benefit from preparation programs aimed at
enhancing coping skills.
C. Prior hospitalization routinely aids in calming
children undergoing surgery.
D. Parental anxiety is strongly associated with child
anxiety.
26. Which severe disease state is commonly associated
with seizure disorder and is strongly associated with
impaired oromotor function and recurrent aspiration
pneumonia?
A. Idiopathic scoliosis
B. Cerebral palsy
C. Cystic fibrosis
D. Trisomy 21
27. An 11-year-old Caucasian male with cystic fibro-
sis presents with abdominal pain and is scheduled for
a laparoscopic appendectomy. Which of the following
treatment plans optimizes his postoperative outcomes?
A. Cessation of preoperative bronchodilators
B. Prophylactic broad spectrum antibiotics
C. Incentive spirometry and postural drainage
D. Low-calorie diet, continuation of pancreatic enzyme
replacement, and fat-soluble vitamin supplements
28. A  2-month-old infant who was born at 30 weeks
gestation with complete bladder extrophy presents for
exploratory laparotomy and intestinal stricture resec-
tion. This is her fourteenth general anesthetic. Once a
new Foley catheter is placed in the operating room, her
electrocardiogram (EKG) demonstrates sinus tachycar-
dia, a noninvasive blood pressure cannot be obtained,
and the ventilator alarms because her peak airway pres-
sures acutely increase >40  cm H2O. Which of the fol-
lowing is most important in treating this patient?
A. Administer H1 and H2 blockers
B. Administer epinephrine
C. Administer corticosteroid
D. Send blood specimen for mast cell tryptase level
29. A  13-year-old otherwise healthy boy presents for
resection of an anterior mediastinal mass. He and his
parents note that for the last 9 months, he has not been
able to participate during recess at school because of
progressive tiredness, he has a constant dry cough, and
he uses two pillows to sleep comfortably at night. As
you examine him, you notice plethora of his neck and
face. Which of the following preoperative studies best
detects evidence of dynamic airway compression in this
patient?
 A. Chest X-ray
B. CT scan
C. Cardiac echocardiogram
D. Pulmonary function tests
30. A talkative 7-year-old boy with Williams syndrome
presents for tonsillectomy and adenoidectomy for sleep
apnea and recurrent tonsillitis. Which of the follow-
ing findings is most alarming during your preoperative
assessment?
A. Moderate aortic stenosis
B. Rhinorrhea and dry cough
C. Micrognathia with prominent incisors
D. 4 + tonsils
31. A 3-year-old boy with Tetralogy of Fallot is sched-
uled for urgent left groin exploration with possible
orchiopexy versus orchiectomy for left testicular tor-
sion. He has had groin pain for 4 hours and arrives from
the emergency department with three failed intravenous
attempts. As he begins to cry in the preoperative hold-
ing area, you notice his lips turning blue. Bearing in
mind hemodynamic goals for a patient with Tetralogy
of Fallot, what should you do next?
A. Administer oral midazolam and provide oxygen by
facemask.
B. Administer intramuscular ketamine and provide
oxygen by facemask.
C. Place a peripheral intravenous to increase his
intravascular volume.
D. Find a junior partner to do the case for you.
32. If this same 3-year-old boy with Tetralogy of Fallot
and testicular torsion is now in the operating room
and is crying, what is the best way to safely attenuate a
hypercyanotic episode in this patient with Tetralogy of
Fallot and no intravenous access?
A. Use EMLA, then place an intravenous to administer
an anxiolytic, crystalloid, and phenylephrine.
B. Proceed with mask induction with cricoid pressure,
high inspired concentration of sevoflurane, and
100% FiO2 .
C. Proceed with mask induction with cricoid pressure,
high inspired concentration of sevoflurane, and 80%
N2O/20% O2 .
D. Administer intramuscular ketamine and provide
supplemental oxygen.
33. A  chubby 2-year-old with Down syndrome has
severe obstructive sleep apnea with an apnea-hypopnea
index of 42 and presents for tonsillectomy and adenoid-
ectomy. She feeds well, her parents report appropriate
  
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weight gain with no sweating or cyanotic episodes with
feeds, and she walks without difficulty in the preop-
erative holding area. As you examine the patient, you
notice Down facies, macrocephaly, macroglossia, and
you hear a holosystolic murmur on auscultation. What
should you do next?
A. Obtain a chest X-ray.
B. Obtain an EKG and cardiac echocardiogram.
C. Obtain a cardiac stress test.
D. Proceed with surgery.
34. A  13-year-old severely autistic adolescent presents
for dental rehabilitation. Dentists have been unsuccess-
ful in examining his teeth in the office because he is a
“biter.” He has poor dental hygiene because he will not
allow anyone to brush his teeth or place anything near
his face. Which of the following options is most reliable
in inducing general anesthesia in this severely autistic
patient?
A. Intravenous induction with propofol and fentanyl
B. Inhalational induction with sevoflurane
C. Oral midazolam, then inhalational induction with
sevoflurane
D. Intramuscular ketamine, then inhalational
induction with sevoflurane
35. A 14-year-old girl with asthma and idiopathic sco-
liosis with a Cobb angle of 60 degrees presents for
T2-pelvis posterior spinal fusion with instrumentation.
She appears thin, tearful, and nervous in the preopera-
tive area. Her parents inform you that they are Jehovah’s
Witnesses and do not want any transfusion of blood or
blood products to their daughter. She is in the middle
of menses now. What is your most important periop-
erative concern for this patient and her upcoming major
surgery?
A. Discussion of anxiolytic strategy
B. Discussion of blood conservation strategies
C. Discussion of neuromonitoring and possible
wake-up test
D. Discussion of susceptibility to malignant
hyperthermia
36. A neonate with hypertelorism, midface hypoplasia,
seizures, and tetany presents for omphalocele repair.
Given the nature of this major surgery, which will likely
involve large fluid shifts and potential transfusion
of blood products, which of the following is the most
important part of your preoperative evaluation?
A. Focused airway exam
B. Cardiovascular workup
 C. Evaluation of electrolyte abnormalities
D. Ensuring appropriate urine output
37. A full-term 3 kg neonate male with a large, prena-
tally diagnosed myelomeningocele presents for myelo-
meningocele repair and placement of a VP shunt for
hydrocephalus. What is your primary reasoning for
recovering this patient in an ICU-level setting?
A. Fluid resuscitation
B. Postoperative respiratory monitoring
C. Proper patient positioning
D. Maintaining euthermia
38. An ex-28-week, now 6-week-old twin B has a unilat-
eral inguinal hernia. He is now 4 kg. You administered a
single-shot spinal with 0.4 cc 0.5% bupivacaine for your
anesthetic, in your efforts to avoid post-operative apnea
in this former preterm infant. After the hernia sac has
been dissected, the infant starts moving his legs. Since
he is under the surgical drapes, you cannot repeat the
spinal. Which of the following techniques safely pre-
vents postoperative apnea in this baby?
A. Ask the surgeon to supplement with local to the
surgical field.
B. Induce general anesthesia but use fentanyl instead of
morphine.
C. Induce general anesthesia but administer no narcotic.
D. Supplement with ketamine to keep the baby
breathing spontaneously.
39. A full-term, now 6-week-old firstborn male presents
with 4  days of nonbilious projectile vomiting, and an
ultrasound confirms hypertrophic pyloric stenosis. He
is scheduled for laparoscopic pyloromyotomy for hyper-
trophic pyloric stenosis. Which of the following sets of
laboratory values best indicates that this infant is ready
for surgery?
Na+ K+ Cl– HCO3–
A. 130 3.1 89 34
B. 142 3.8 105 25
C. 150 3.2 102 32
D. 142 3.6 92 28

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40. An ex-31-week, now 4-year-old boy presents for
circumcision for phimosis. He has received no vacci-
nations and has been febrile for the past 36 hours. As
you examine him in the preoperative holding area, you
observe rhinorrhea, hear diffuse rales in his lung fields
bilaterally, and see the patient cough up yellow sputum.
How do you proceed?
A. Proceed with GA, use an LMA to avoid intubation,
supplement with a dorsal penile block.
B. Administer albuterol in preop holding, then proceed
with GA.
C. Administer steroids and azithromycin, then proceed
with GA.
D. Postpone this elective procedure for at least 4 weeks.
41. A  16-year-old, African American male with sickle
cell disease presents for laparoscopic cholecystectomy.
He is maintained on chronic folic acid and hydroxyurea
therapy. Which of the following would be most delete-
rious to him in the perioperative period?
A. Liberal use of analgesics
B. Transfusion of phenotype-matched,
leukocyte-reduced blood to target hemoglobin
10 g/dL
C. Hyperventilation to EtCO2 < 25 mm Hg
D. Intraoperative use of inhaled nitric oxide
42. A  5-year-old girl with Beckwith-Wiedemann syn-
drome has hemihypertrophy and presents for left distal
femur epiphysiodesis. Her parents report that she had
a perinatal omphalocele repair with no complications,
and that all of her follow-up screens for somatic tumors
have been negative. You plan a combined general anes-
thetic and femoral nerve block catheter for postopera-
tive analgesia. Which of the following studies would
most strongly influence your perioperative care for this
patient?
A. Fingerstick glucose
B. Liver function tests
C. Cardiac echocardiogram
D. Caffeine-contracture testing for malignant
hyperthermia susceptibility
 $) " 15 &3ु
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1. A N S W E R : C
Atrial septal defects (ASDs) are common congenital cardiac
lesions and can vary in severity and timing of presentation.
The vast majority (75%) of ASDs are ostium secundum
defects that occur at the center of the interatrial septum in
the region formerly occupied by the foramen ovale. Septum
primum ASDs are due to endocardial cushion defects,
which leave a large opening in the center of interatrial sep-
tum. There are also sinus venosum defects, which are rarer.
In the absence of other major cardiac anomalies, ASDs
will initially be acyanotic lesions as blood generally shunts
left to right. Small ASDs, such as in this question, are gener-
ally watched and not intervened upon. They typically create
a systolic ejection murmur at the left upper sternal bor-
der, which may be mistaken for a flow murmur, particularly
in a well-appearing child. Larger lesions (e.g., approaching
2  cm in diameter) can lead to significant shunting caus-
ing increased flow through the pulmonary circulation.
An echo that reveals a Qp:Qs of > 1.5 signifies significant
shunting and may warrant closure, either open or by percu-
taneous intervention. The long-term consequences include
right-heart failure, pulmonary hypertension, and if
untreated, reversal of the shunt if right-heart pressure
exceeds the pressure in the left heart.
Anesthetic considerations for patients with ASDs are
based around management of the SVR:PVR ratio. If the
PVR drops considerably, there will be increased shunting
and worsened right-heart signs/symptoms. Conversely,
if the PVR is allowed to rise due to hypoxia, hypercarbia,
acidosis, or hypothermia, this may lead to transient devel-
opment of a cyanotic lesion. It is imperative to remember
than even if only left-to-right shunting is present on echo,
there is still a risk of paradoxical emboli. Many patients do
well for years, and the ASD may actually be undetected, in
which case they may not present until adulthood.
K E Y FAC T S
r In the absence of other major cardiac anomalies, ASDs
will initially be acyanotic lesions as blood generally
shunts left to right.
r Larger lesions with a Qp:Qs of > 1.5 and significant
shunting may lead to right-heart failure, pulmonary
hypertension, and reversal of shunt flow if untreated.
r Anesthetic considerations for patients with ASDs are
rooted in management of the SVR:PVR ratio.
3 & ' & 3 & /$ & 4
Odegard, Kirsten C., DiNardo, James A., Laussen, Peter C. “Anesthesia
for Congenital Heart Disease” in Gregory GA, Andropoulos DB,
  
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eds. Gregory’s Pediatric Anesthesia. 5th ed. Hoboken, NJ: Blackwell;
2011:642–3.
Franco, Samantha A.; Hines, Roberta L. “Congenital Heart Disease”
in Hines RL, Marschall KE, eds. Stoelting’s Anesthesia and
Co-Existing Disease. 6th ed. Philadelphia, PA:  Saunders Elsevier;
2012:49–51.
2 . A N S W E R : A
Tetralogy of Fallot (TOF) is the most common congenital
cyanotic lesion, accounting for 10% of congenital heart dis-
ease cases. The four components of the lesion are (1) a VSD,
(2) an “overriding” aorta that covers ventricles, (3) RVOT
obstruction, and (4) right ventricular hypertrophy (RVH).
This child is experiencing a hypercyanotic “Tet” spell, indi-
cating that the volume of blood shunting from right to
left has increased. After increasing preload (fluid bolus,
flexing the hips) and increasing afterload (flexing the hips
and using phenylephrine), the next step is to reduce the
obstruction to RV outflow. Though it may seem logical to
reduce PA pressures with milrinone or epinephrine, the
distal pulmonary pressures are generally low and not a sig-
nificant factor in the degree of shunting. The obstruction
to RV outflow is at the level of the RVOT. This may be
subvalvular, valvular, supravalvular, and occasionally at
the pulmonary arterial branches. Esmolol will slow heart
rate to allow for adequate preload and will also relax the
infundibular (subvalvular) region of the RVOT, allowing
more blood to leave the RV into the RVOT. For children
with recurrent Tet spells, propanolol is often indicated for
outpatient use.
An anesthetic for a child with TOF must be well
planned and tailored to the patient. Premedication is often
recommended as crying and struggling can worsen breath-
ing and cause desaturations. Midazolam has been shown to
be effective. The induction of anesthesia on patients with
TOF can cause significant hemodynamic changes, which
can quickly lead to cyanosis. Ketamine is often used as
an induction agent. The increase in SVR with ketamine
will reduce the right-to-left shunt. An inhaled induction
is acceptable, but one must remember that the reduction in
SVR will cause an increase in the shunt. Historically, halo-
thane has been the preferred inhalational agent for induc-
tion as it maintains a relatively stable SVR but causes direct
myocardial depression, helping to relax the infundibulum.
Although still in use in some centers, the popularity of hal-
othane has decreased due to the risk of fulminant hepatic
necrosis. Positive-pressure ventilation, hypercarbia,
and hypoxia will raise pulmonary pressures and increase
the right-to-left shunt, so effective airway management is
imperative when balancing SVR and PVR. Care should
also be taken to avoid high airway pressures and allow ade-
quate time for expiration to avoid increased intrathoracic
pressures. As with all cardiac lesions involving shunts, care
must be taken to avoid air in the IV tubing due to risk of
paradoxical emboli.
 K E Y FAC T S
r TOF is the most common congenital cyanotic lesion
and consists of a VSD, an “overriding” aorta, RVOT
obstruction, and RVH.
r A child experiencing a “tet” spell has increased right-to-
left shunting, and is treated by increasing preload and
afterload as well as reducing the RVOT obstruction.
This can be accomplished by giving beta blockers.
r Positive-pressure ventilation, hypercarbia, and hypoxia
will raise pulmonary pressures and increase the right-to-
left shunt, so effective airway management is imperative
when balancing SVR and PVR.
3 & ' & 3 & /$ & 4
Odegard, Kirsten C., DiNardo, James A., Laussen, Peter C. “Anesthesia
for Congenital Heart Disease” in Gregory GA, Andropoulos DB,
eds. Gregory’s Pediatric Anesthesia 5th ed. Hoboken, NJ: Blackwell;
2011:633–5.
Franco, Samantha A.; Hines, Roberta L. “Congenital Heart Disease” in
Hines RL, Marschall KE, eds. Stoelting’s Anesthesia and Co-Existing
Disease. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:57–9.
Levine MF, Hartley EJ, Macpherson BA, Burrows FA, Lerman J. Oral
midazolam premedication for children with congenital cyanotic
heart disease undergoing cardiac surgery: a comparative study. Can J
Anaesth. 1993;40(10):934–8.
3 . A N S W E R : B
Autism is a poorly understood psychiatric disorder that
manifests in multiple fashions, most centrally as an
inability to develop interpersonal relationships as
considered normal in a given culture. Associated findings
may include developmental delay, seizures, and culturally
inappropriate behaviors but are not central to the diagno-
sis. Behaviors vary among autistic patients and could be
largely considered as either hyperreactive or hyporeac-
tive. Among hyperreactive patients, it is paramount that
the anesthesiologist inquire about triggers for patients in
order to provide a safe environment. Likewise, it is impor-
tant to distinguish hyporeactive behaviors because they
mimic common perioperative issues such as hypoglyce-
mia, anesthetic overdose, and hypothermia. Currently no
curative treatment exists for autism. Life span is normal
with continuation of childhood symptoms into adult-
hood being relatively common.
Substantial consideration must be given to the details
of the operative experience when the patient is autistic in
order to deliver the safest experience possible. Details such
as lighting, stimulation, calming techniques, and agita-
tion triggers are most easily explored with the parent. In
addition, child life specialists are tremendously helpful in
this arena, freeing the anesthesiologist to devote mental
resources to medical concerns such as preoperative sedation
and pain control.

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Emergence from general anesthesia is a particularly dif-
ficult time for autistic patients. Changes in sedation level,
coupled with abrupt changes in location, present a challenge
to all children, but these are particularly challenging for
autistic children. Coupled with surgical pain, temperature
changes, and hunger, emergence and transition through the
postanesthetic care unit can be a time of particular diffi-
culty with autistic patients.
K E Y FAC T S
r Autism is a poorly understood psychiatric disorder
that manifests in multiple fashions, most centrally as
an inability to develop interpersonal relationships as
considered normal in a given culture.
r Behaviors vary among autistic patients and could
be largely considered as either hyperreactive or
hyporeactive. For hyperreactive patients, it is important
to explore the triggers for agitation (lighting, touch,
sounds, etc.) and calming techniques from the parents.
r Coupled with surgical pain, temperature changes,
and hunger, emergence and transition through the
postanesthetic care unit can be a time of particular
difficulty with autistic patients.
3 & ' & 3 & /$ &
Diu, Michelle W.; Mancuso, Thomas J. “Pediatric Disease” in Stoelting
RK, Dierdorf SF, eds. Anesthesia and Co-Existing Disease. 4th ed.
Philadelphia, PA: Churchill Livingstone; 2002:639.
4 . A N S W E R : D
Cerebral palsy (CP) is a nonprogressive neurologic disorder
that is multifactorial in origin but is generally related to a
variety of prenatal, perinatal, and postnatal insults. The
disease process is not well understood, and contributing
factors can range from prematurity to infection to intrace-
rebral hemorrhage. Perinatal asphyxia was once thought to
be the most common etiology but is now believed to cause
less than 10% of CP. The key feature of CP is a disorder
of movement and posture. There may be varying degrees of
mental retardation and developmental delay, depending on
the etiology of the cerebral palsy. Multiple classification sys-
tems have been developed for CP; a commonly used system
is based on the type of neurologic impairment (i.e., spastic,
dyskinetic, atheoid, mixed). Spastic CP is the most com-
mon, accounting for 70% of cases.
Children with CP frequently have seizure disorders
with an incidence of at least 30%. They are often on antiepi-
leptic drugs, in addition to antispasmodic medications, and
withdrawal can trigger seizures as in this scenario. In the
perioperative period it is important to review all medica-
tions and administer preoperatively, if necessary.
 Dexmedetomidine is an alpha-2 agonist often used as
an adjunct for general anesthesia. It has potent sedative
effects and provides analgesia, which can be opioid sparing.
There have been no case reports of seizures/convulsions in
humans.
K E Y FAC T S
r CP is a nonprogressive neurologic disorder that is
multifactorial in origin but is generally related to a
variety of prenatal, perinatal, and postnatal insults. It is a
disorder of movement and posture, with varying degrees
of mental retardation and developmental delay.
r Spastic CP is the most common, accounting for 70%
of cases, and other types are based on the neurologic
impairment (i.e., spastic, dyskinetic, atheoid, mixed).
r Children with CP frequently have seizure disorders with
an incidence of at least 30%.
3 & ' & 3 & /$ & 4
Loepke, Andreas W.; Davidson, Andrew J. “Surgery, Anesthesia, and the
Immature Brain” in Cote CJ, Lerman J, Todres ID, eds. A Practice
of Anesthesia for Infants and Children. 4th ed. Philadelphia, PA:
Saunders Elsevier; 2009:492–4.
Diu, Michelle W.; Mancuso, Thomas J. “Pediatric Disease” in
Hines RL, Marschall KE, eds. Stoelting’s Anesthesia and
Co-Existing Disease. 6th ed. Philadelphia, PA: Saunders Elsevier;
2012:604–5.
Lerman J. Perioperative management of the paediatric patient with coex-
isting neuromuscular disease. Br J Anaesth. 2011;107(S1):i79–89.
Miller RD, Eriksson LI, Fleisher LA, Wiener-Kronish JP, Young
WL, eds. Miller’s Anesthesia. 7th ed. Philadelphia, PA:  Chuchill
Livingstone; 2010:752–4, 2248.
5 . A N S W E R : C
Gastroesophageal reflux disease (GERD) is quite com-
mon in patients with cerebral palsy (CP) and clinicians
often opt for an RSI prior to intubation. Due to CP being
a neuromuscular disease, anesthesia trainees often assume
depolarizing NMBs are contraindicated, but this is not
true. There have been no case reports of hyperkalemia after
succinylcholine administration despite many years of use.
This is due to only minimal upregulation of ACh recep-
tors around the neuromuscular junction despite having
spastic paresis.
Answers C (burns) and D (myopathy that is classic for
Duchenne muscular dystrophy) represent conditions that
may lead to hyperkalemia following succinylcholine use due
to upregulation of ACh receptors.
Malignant hyperthermia (MH) is a condition involv-
ing abnormal intracellular handling of calcium, often
related to the ryanodine receptor at the sarcoplasmic reticu-
lum. Succinylcholine is one of the known triggering agents
and should be avoided in patients with a known or sus-
pected family history of MH, as in Answer B.
  
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K E Y FAC T S
r Conditions that cause upper or lower motor neuron
defects, burns, and prolonged immobilization can lead
to upregulation of ACh receptors, causing hyperkalemia
after succinylcholine use.
r Despite the neuromuscular disease, succinylcholine use
does not seem to cause hyperkalemia in CP patients.
r If there is concern for malignant hyperthermia,
succinylcholine should be avoided.
3 & ' & 3 & /$ & 4
Parness, Jerome; Lerman, Jerrold; Stough, Robert C. “Malignant
Hyperthermia” in Cote C, Lerman J, Todres ID, eds. A Practice
of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:847–66.
Lerman J. Perioperative management of the paediatric patient with coex-
isting neuromuscular disease. Br J Anaesth. 2011;107(S1):i79–89.
Martyn J, Richtsfeld M. Succinylcholine-induced hyperkalemia in
acquired pathologic states. Anesthesiology. 2006;104:158–69.
6 . A N S W E R : A
Cystic fibrosis (CF) is an autosomal recessive disorder that
affects 1 in 2000 Caucasian births in the United States and
is the most common fatal inherited disease in white indi-
viduals. The genetic basis for the disease is a mutation in the
CFTR gene on chromosome 7. This gene codes for a chlo-
ride transporter located on epithelial cells in several organs,
including the lungs, pancreas, liver, and gastrointestinal
tract. Decreased chloride transport results in decreased
sodium and water transport into the luminal surface of the
organs. This yields thick, dehydrated secretions that cause
luminal obstruction with subsequent inflammation and
scarring. The lungs are generally affected at a young age
with decreased clearance of the thick secretions being the
impetus for recurrent bacterial infections, with later find-
ings of bronchiectasis and emphysema. The lungs account
for nearly 90% of the morbidity and mortality of CF.
The GI tract is affected with pancreatic exocrine insuf-
ficiency, poor GI motility, and altered enterohepatic cir-
culation. More than 90% of CF patients have pancreatic
dysfunction, and most are prone to recurrent episodes of
pancreatitis. Due to malabsorption and liver dysfunction,
CF patients may have vitamin K deficiency and reduced
levels of clotting factors II, VII, IX, and X.  The deficien-
cies would prolong the PT but generally not the PTT and
would not affect platelet levels. In the absence of clinically
significant bleeding, there are no screening labs that must
be obtained prior to surgery, but one should keep vitamin
K deficiency in mind when evaluating a patient with CF.
K E Y FAC T S
r CF is an autosomal recessive disorder that affects 1
in 2000 Caucasian births in the United States and
 is the most common fatal inherited disease in white
individuals.
r Decreased chloride transport results in decreased
sodium and water transport into the luminal surface of
the organs, yielding thick, dehydrated secretions that
cause obstruction with subsequent inflammation and
scarring.
r Due to malabsorption and liver dysfunction, CF
patients may have vitamin K deficiency and reduced
levels of clotting factors II, VII, IX and X, causing a
prolonged PT.
3 & ' & 3 & /$ & 4
Al-Ruzzeh, Sharif; Kurup, Viji. “Respiratory Diseases” in Hines RL,
Marschall KE, eds. Stoelting’s Anesthesia and Co-Existing Disease.
6th ed. Philadelphia, PA: Saunders Elsevier; 2012:196.
Huffmyer JL Littlewood KE, Nemergut EC. Perioperative man-
agement of the adult with cystic fibrosis. Anesth Analg.
2009;109(6):1949–61.
7. A N S W E R : D
Down syndrome (Trisomy 21) is relatively common occur-
ring in 1:700–800 live births, and affected children may
present for a variety of procedures. In addition to a com-
plete history and physical, one should concentrate on the
airway examination. Brachycephaly, macroglossia, micro-
gnathia, hypotonia, and subglottic tracheal stenosis are
common findings, which may make positioning, direct
laryngoscopy, and successful intubation difficult. The nar-
rowed subglottic diameter makes postintubation croup
more common, so consideration should be given to using a
smaller endotracheal tube.
In addition to airway abnormalities, there may be con-
siderable laxity in the cervical spine, with both occiput-C1
and C1-C2 instability having been described. Parents
should be questioned about the child’s history of cervi-
cal spine instability, complaints of neck pain, inability
to move the neck, gait abnormalities, sensorimotor defi-
cits, and bowel/bladder incontinence. The medical record
should be reviewed for prior cervical spine films, which
typically include lateral flexion-extension plain films.
Symptomatic children are screened via radiographs
between 3 and 5 years of age in accordance with recom-
mendations from the American Academy of Pediatrics,
while the guidelines do not recommend routine radio-
graphs of asymptomatic children. The anesthesiologist
should be careful to minimize neck hyperextension
during mask ventilation or direct laryngoscopy, even in
the absence of a history that suggests cervical instability.
Positioning during the case should also be a point of con-
cern, particularly during head/neck procedures where the
surgeon may ask for a position that could compromise the
cervical spine.

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K E Y FAC T S
r Down syndome (Trisomy 21) is a common occurrence
and is likely to be encountered by a pediatric
anesthesiologist.
r Airway abnormalities include brachycephaly,
macroglossia, micrognathia, hypotonia, and subglottic
tracheal stenosis.
r Cervical spine laxity may be present, and a focused
history and physical as well as review of the medical
record are important to avoid subluxation.
3 & ' & 3 & /$ & 4
American Academy of Pediatrics. Health supervision for children with
Down syndrome. Pediatrics. 2011;128(2):393–406.
Weaver, R.  Grey Jr., Tobin, Joseph R. “Opthalmology” in Cote C,
Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:688–9.
Diu, Michelle W.; Mancuso, Thomas J. “Pediatric Disease” in Hines
RL, Marschall KE, eds. Stoelting’s Anesthesia and Co-Existing
Disease 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:634–634.
8 . A N S W E R : C
Murmurs are often appreciated on children during pre-
operative examinations, and the pediatric anesthesiolo-
gist will frequently be tasked with guiding what, if any,
diagnostic testing must be completed prior to undergoing
surgery. In this scenario, the murmur likely represents a
so-called innocent murmur. The term “innocent” implies
that there are no cardiac abnormalities present; the mur-
mur is caused by flow through normal anatomic structures.
Because these are flow dependent, they will change with
position or maneuvers that alter venous return and car-
diac output. In contrast, a fixed murmur would imply a
structural abnormality. Innocent murmurs are exclusively
systolic, not graded more than III/VI on auscultation.
A common example is Still’s murmur, which is low pitched
and typically appreciated at the left lower sternal bor-
der without radiation across the precordium. A  Valsalva
maneuver will greatly reduce the murmur or make it dis-
appear entirely. No clicks or other abnormal heart sounds
can be appreciated with Still’s murmur. The rest of the
physical exam, including the pulmonary exam, peripheral
pulses, and signs of normal perfusion (e.g., capillary refill
and skin color) should be normal.
The evaluation of the murmur should begin with a good
history from the child’s parents. They should be questioned
about the birth history, perinatal events, issues with feed-
ing, episodes of cyanosis, ability to perform physical tasks
similar to children of their age, and any complaints from
the child related to chest pain, syncope/presyncope, or dys-
pnea. A family history of significant heart disease should be
elicited. Children with a completely normal history are less
likely to have significant underlying cardiac pathology.
 In this case, the fact that the child’s movement changed
the intensity of the murmur and her general state of
well-being make an innocent murmur the most likely etiol-
ogy, and further workup is generally not required prior to
anesthesia.
K E Y FAC T S
r Innocent murmurs are exclusively systolic, not graded
more than III/VI on auscultation. A common example
is Still’s murmur, which is low pitched and typically
appreciated at the left lower sternal border without
radiation across the precordium.
r The rest of the physical exam, including the pulmonary
exam, peripheral pulses, and signs of normal perfusion
(e.g., capillary refill and skin color) should be normal.
3 & ' & 3 & /$ & 4
Biancaniello T. Innocent murmurs. Circulation. 2005;111:e20–e22.
Slesnick, Timothy C.; Gertler, Ralph; Miller-Hance, Wanda C.
“Essentials of Cardiology” in Cote CJ, Lerman J, Todres ID, eds. A
Practice of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:307–8.
Litman RS. Basics of Pediatric Anesthesia. Tablet ed. Philadelphia,
PA: Ronald S. Litman; 2013:118–9.
9. A N S W E R : D
Though all of the choices are possible, hemoglobin (Hb) of
10 g/dL represents a normal finding for an infant at 9 weeks
of age. A term neonate typically has a hemoglobin level of
17–18 g/dL. This is caused by very high erythropoietin
(EPO) levels in fetal circulation due to the low oxygen ten-
sion in the uterine environment and the leftward position
of the fetal hemoglobin-oxygen dissociation curve. The
elevated EPO levels cause Hb levels considerably higher
than in older children or adults. As oxygen levels rise in the
neonate, there is a sharp decline in EPO and hematopoiesis
is reduced. In addition, fetal hemoglobin (HbF) production
is reduced as adult hemoglobin (HbA) production begins to
rise. The nadir of the resultant anemia is generally seen at
8–12 weeks of age with Hb levels in the 10–12 g/dL range.
This is referred to as a physiologic anemia, indicating that
normal physiology accounts for the laboratory values with-
out underlying pathology.
Of note, there are no guidelines that have established
“safe” Hb levels for pediatric surgery. For routine cases not
expected to cause significant bleeding, checking hemo-
globin in healthy children prior to surgery is generally
not indicated. Several authors have studied preoperative
Hb/Hct values in large numbers of children and found
very low rates of anemia. Preoperative coagulation studies
rarely delay surgery or change management; the utility of
these tests is often questioned. Evidence also indicates that
  
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r

the abnormal lab values are often ignored without being
followed by repeat studies or interventions to correct the
abnormalities. In addition to adding to increasing health
care costs, the unwarranted testing exposes the child to
undue stress and needle sticks. Despite these findings, sig-
nificant numbers of physicians continue to order screening
labs prior to surgery.
K E Y FAC T S
r Term neonates have elevated Hb levels in the
15–20 g/dL range.
r Due to reduced EPO levels and a reduction in HbF
synthesis, Hb levels drop to 10–12 g/dL with the nadir
in the 8- to 12-week range.
r A further workup for this physiologic anemia is not
generally indicated.
r Routine laboratory testing for minor procedures is
not warranted unless there are specific concerns to be
addressed.
3 & ' & 3 & /$ & 4
Ghazal, Elizabeth A.; Mason, Linda J.; Coté, Charles J. “Preoperative
Evaluation, Premedication, and Induction of Anesthesia” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:59–62.
Motoyama, Etsuro K.; Finder, Jonathan D. “Respiratory Physiology
in Infants and Children” in Davis PJ, Cladis FP, Motoyama EK,
eds. Smith’s Anesthesia for Infants and Children. 8th ed. St. Louis,
MO: Mosby Elsevier; 2011:395–417.
Miller, Bruce E.; Hendrickson, Jeanne E. “Coagulation, Bleeding, and
Blood Transfusion” in Gregory GA, Andropoulos DB, eds. Gregory’s
Pediatric Anesthesia. 5th ed. Hoboken, NJ: Blackwell; 2011:233–4.
O’Connor ME, Drasner K. Preoperative laboratory testing of children
undergoing elective surgery. Anesth Analg. 1990;70:176–80.
Patel RI, DeWitt L, Hannallah RS. Preoperative laboratory testing in
children undergoing elective surgery:  analysis of current practice.
J Clin Anesth. 1997;9(7):569–75.
10 . A N S W E R : A
The scenario represents normal development for a healthy
child. Inquiring about the development of a pediatric patient
is extremely important in assessing the child’s overall health
status. One very commonly used screening method to assess
development makes use of milestones as defined by the Denver
Developmental Screening Test (DDST). The milestones
are described in broad categories:  gross motor (e.g., sitting,
standing), fine motor (pincer grasp, playing with blocks), lan-
guage (e.g., talking), and personal-social (e.g., smiling, separa-
tion anxiety, playing with others). The American Academy of
Pediatrics recommends a developmental assessment using the
milestones at 9 months, 18 months, and 30 months, but many
practitioners assess aspects of developmental at all well-child
visits. When used as a screening method, the sensitivity and
specificity of the milestones are >70%.
 While the anesthesiologist may not frequently com-
plete a full developmental screening test, understanding
the expected norms based on a child’s age will help in plan-
ning an anesthetic tailored to the child. This will make for a
smoother induction, avoid postoperative behavioral issues,
and potentially change the pharmacologic approach to the
anesthetic. Behavioral and developmental delays are incred-
ibly common, present in 15%–18% of pediatric patients.
It is important to remember that while missing a specific
milestone may not affect an anesthetic plan, it may be a clue
that a larger issue is present.
Table 8–1 in Nelson’s Essentials of Pediatrics, sixth edi-
tion (copyright 2011), is a good overview of the milestones
and provides the salient information without excessive
detail.
3 & ' & 3 & /$ & 4
Niezgoda, Julie. “Behavioral Development” in Davis PJ, Cladis FP,
Motoyama EK, eds. Smith’s Anesthesia for Infants and Children. 8th
ed. St. Louis, MO: Mosby Elsevier; 2011:10–21.
Levine, David A. “Growth and Development” in Kliegman RM,
Behrman RE, Marcdante K, eds. Nelson’s Essentials of Pediatrics.
6th ed. Philadelphia, PA: Saunders Elsevier; 2011:19–21.
11. A N S W E R : D
Preoperative anxiety and the need for proper psychosocial
preparation represents one of the most important aspects of
pediatric anesthesia. Up to 50% of all children undergoing
anesthesia will have high levels of anxiety regarding the pro-
cess. In addition to stress on the child, this complicates the
anesthetic induction and puts considerable distress on the
parents. It may also lead to emergence agitation and post-
operative behavioral issues. Understanding the causative
factors for preoperative anxiety will help identify those at
risk and guide treatment. Children younger than 7  years
generally experience more anxiety than older children, as
the older children tend to develop coping mechanisms to
deal with the anxiety. Several studies have demonstrated
that shy, withdrawn children will experience more anxiety
than other children their age. This makes it important to
observe the child and question the parents regarding the
child’s personality and reactions to stressful situations.
A  history of a negative experience with anesthesia or sur-
gery will likely influence the preoperative anxiety as well,
making it important to inquire about past anesthetic
experiences.
Treatment of preoperative anxiety is typically mul-
timodal and should be tailored to the child. Research on
the subject has been done for years, but no solid guidelines
exist for treatment. Preoperative education (i.e., pamphlets,
hospital tours, role playing) completed in the days before
surgery is effective, but it must be timed well. One author
reported that children who underwent preoperative
 
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preparation 5–7 days prior to surgery experienced low lev-
els of anxiety, whereas those who underwent the program
24 hours preoperatively experienced the highest levels of
anxiety. Distraction techniques such as music, television,
and video games are also valuable methods for reducing
anxiety.
Premedication is commonly used, with midazolam
and clonidine being the best studied agents. Midazolam is
effective due to its sedating and anxiolytic effects, as well as
the anterograde amnesia that results from administration.
A recent study reported satisfactory results in 75% of chil-
dren who were given oral midazolam preoperatively. Other
studies have shown that there may be a higher incidence of
postoperative behavioral issues, when midazolam has been
administered, so one must be careful to use it only when
indicated.
K E Y FAC T S
r Up to 50% of all children undergoing anesthesia will
have high levels of anxiety regarding the process.
r Children younger than 7 years generally experience
more anxiety than older children, as the older children
tend to develop coping mechanisms to deal with the
anxiety.
r Midazolam is effective due to its sedating and anxiolytic
effects, as well as the anterograde amnesia that
results from administration. A recent study reported
satisfactory results in 75% of children who were given
oral midazolam preoperatively.
3 & ' & 3 & /$ & 4
Ghazal, Elizabeth A.; Mason, Linda J.; Cotè, Charles J. “Preoperative
Evaluation, Premedication, and Induction of Anesthesia” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:39–46.
Davidson, Andrew; Howard, Kelly; Browne, William; Harbre, Walid;
Lopez, Ursula. “Preoperative Evaluation and Preparation, Anxiety,
Awareness, and Behavior Change” in Gregory GA, Andropoulos
DB, eds. Gregory’s Pediatric Anesthesia. 5th ed. Hoboken,
NJ: Blackwell; 2011:283–9.
Kain ZN, Mayes LC, Caramico LA. Preoperative preparation in chil-
dren: a cross-sectional study. J Clin Anesth. 1996;8(6):508–14.
Kogan A, Katz J, Efrat R, Eidelman LA. Premedication with mid-
azolam in young children: a comparison of four routes of adminis-
tration. Paediatr Anaesth. 2002;12:685–9.
Tan L, Meakin GH. Anaesthesia for the uncooperative child. Contin
Educ Anaesth Crit Care Pain. 2010;10(2):48–52.
12 . A N S W E R : B
Parental presence during anesthesia has long been a topic of
controversy. The benefits of parental presence are thought
to minimize the need for premedication and to avoid the
trauma and struggling that comes with separation from
the parents. In addition, there may be more controversial
benefits like decreasing the child’s anxiety as well as the
long-term stigma that is associated with surgery and anes-
thesia. It is clear that multiple studies have proven that
parents and children generally prefer to be together during
procedures, but this does not necessarily make it a desirable
situation or one that has improved patient outcomes.
If parental presence is allowed, the patient and family
must be counseled during the preoperative evaluation about
the induction of anesthesia and how they can comfort their
child. It must be stressed that being present is not a right
but a privilege and the parent may be removed at any time
if there is new or additional risk to the child. It is crucial
to explain specific events during the process to decrease
the anxiety parents may feel during the induction. After
explaining to the parents what they might see during induc-
tion, it is not unusual for the parents to no longer wish to be
present during induction. At no time should the parents be
coerced or forced to be present in the operating room.
K E Y FAC T S
r The benefits of parental presence during induction over
premedication is still a topic of debate.
r It is crucial to explain specific events during the
induction process that are normal to decrease the
anxiety parents may feel during the induction.
3 & ' & 3 & /$ &
Kain, Zeev N.; MacLaren, Jill; Maes, Linda C. “Perioperative Behavior
Stress in Children” in Cote CJ, Lerman J, Todres ID, eds. A
Practice of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:2.
13 . A N S W E R : D
This patient is exhibiting signs of SVC syndrome such as
distended neck veins, facial swelling, and dyspnea. The
underlying pathology, however, is vascular congestion
resulting from compression of the SVC by the mass. This is
one of the many unique anesthetic challenges that anterior
mediastinal masses can create for anesthesiologists and thus
they must be worked up in a logical manner.
An anterior mediastinal mass (AMM) can be primary
or metastatic, with the most common being lymphoblastic
lymphoma and Hodgkin’s lymphoma. Other less com-
mon tumors that may be the cause include teratomas, thy-
momas, parathyroid, mesenchymal, and thyroid tumors. In
addition, because non-Hodgkin’s lymphoblastic lymphoma
has a 12-hour doubling time, there often is a rush to obtain
a tissue diagnosis and to begin treatment.
When evaluating a patient with an AMM, one should
look for signs and symptoms of vascular and/or airway
compromise, which may include dyspnea, pain, coughing,
  
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SVC syndrome, orthopnea, and/or pleural effusions.
Patients should then undergo testing, which would include
computed tomography (CT), echocardiography, and PFT
test when feasible. Vascular and/or airway compression
detected on a CT is associated with a high incidence of
serious complication during the induction of anesthesia.
Echocardiogram detects the compression of the SVC and
the pulmonary outflow tract, while PFTs detect evidence of
the dynamic compression of the airways.
Substantial effort should be made to obtain a tissue
diagnosis using minimally invasive techniques such as
lymph node biopsy or bone marrow biopsy, so that gen-
eral anesthesia can be avoided. If obvious signs of vascular
and/or airway compromise exist, one must consider per-
forming the mediastinoscopy under local, initiating chemo/
radiation to shrink the tumor prior to induction or giving a
short dose of corticosteroid therapy, which can also shrink
the size of the tumor. These treatments are not without con-
sequence, however, and may actually make it more difficult
to obtain an adequate amount of tissue for a diagnosis; thus,
the oncologist may choose to avoid such an option.
K E Y FAC T S
r An AMM can be primary or metastatic, with the most
common being lymphoblastic lymphoma and Hodgkin’s
lymphoma.
r When evaluating a patient with an AMM, you should
look for signs and symptoms of vascular and/or
airway compromise, which may include dyspnea, pain,
coughing, SVC syndrome, orthopnea, and/or pleural
effusions.
r If obvious signs of vascular and/or airway compromise
exist, one must consider doing the mediastinoscopy
under local, initiating chemotherapy or radiation to
shrink the tumor prior to induction or giving a short
dose of corticosteroid therapy, which can also shrink the
size of the tumor.
3 & ' & 3 & /$ &
Hammer, Gregory B. “Anesthesia for Thoracic Surgery” in Cote CJ,
Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:290–2.
14 . A N S W E R : C
Patients with anterior mediastinal mass (AMM) can have
primary or metastatic disease, with the most common
being lymphoblastic lymphoma and Hodgkin’s lym-
phoma. Other less common tumors that may be the cause
of the patient’s pathology include teratomas, thymomas,
parathyroid, mesenchymal, and thyroid tumors. In addi-
tion, because non-Hodgkin’s lymphoblastic lymphoma has
a 12-hour doubling time, there often is a rush to obtain a
tissue diagnosis and to begin treatment.
When evaluating a patient with an AMM, one should
look for signs and symptoms of vascular and/or airway
compromise, which may include dyspnea, pain, cough-
ing, SVC syndrome, orthopnea, and/or pleural effusions.
Patients should then undergo testing, which would include
CT, echocardiography, and pulmonary function tests when
feasible. Vascular and/or airway compression detected on a
CT is associated with a high incidence of serious complica-
tions during the induction of anesthesia. Echocardiogram
detects the compression of the SVC and the pulmonary
outflow tract, while PFTs detect evidence of the dynamic
compression of the airways.
Even if a patient has undergone testing and shows no
evidence of vascular and/or airway compromise, severe air-
way and circulatory collapse may still occur with the induc-
tion of anesthesia. In light of this fact, it is recommended
that an inhalational induction be performed with main-
tenance of spontaneous respiration. In addition, the use
of continuous positive airway helps maintain functional
residual capacity lost during the induction of anesthesia and
may help keep the airway from collapsing. Elevating the
patient’s head to 30–45 degrees may decrease the deleteri-
ous effects of supine positioning. Neuromuscular blockers
decrease chest wall tone and should be avoided because
this can increase the chance of airway compromise. Finally,
some have advocated using heliox to decrease the airway
resistance and to promote laminar flow.
One must be prepared for the chance of airway/vascular
compromise should the event arise. In circumstances where
this has occurred, quick repositioning to a prone or lat-
eral position can be lifesaving. In a situation where airway
collapse is the main issue, placing a rigid bronchoscope
distal to the point of obstruction may also be necessary.
Finally, in catastrophic situations, it has been advocated to
perform a median sternotomy and initiate cardiopulmo-
nary bypass. Cardiopulmonary bypass would, however,
take some time to initiate and would be impractical unless
access for partial bypass was done prior to the induction of
anesthesia.
K E Y FAC T S
r Patients with AMM can have primary or metastatic
disease, with the most common being lymphoblastic
lymphoma and Hodgkin’s lymphoma.
r When evaluating a patient with an AMM, you should
look for signs and symptoms of vascular and/or
airway compromise, which may include dyspnea, pain,
coughing, SVC syndrome, orthopnea, and/or pleural
effusions.
r Severe airway and circulatory collapse can still occur
with induction. It is recommended that an inhalational
 
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3 & ' & 3 & /$ &
Hammer, Gregory B. “Anesthesia for Thoracic Surgery” in Cote CJ,
Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:290–2.
15 . A N S W E R : A
Latex allergy is extremely common and tends to develop
after repeated exposures to latex through urinary cath-
eterizations, surgical gloves, and other products found in
the hospital. While there has clearly been a more intensive
effort at recognizing the significance of the problem and
thus more products have become latex-free, latex still exists
in many products and some facilities are unable to accom-
modate latex-free cases.
The first step in avoiding such a reaction is taking an
adequate history and in particular looking for reactions
with exposure to latex or rubber (toy balloon). Nearly 50%
of patients with a latex allergy will have their first reac-
tion at the dentist’s office, where there is a tendency to use
more latex-containing equipment. Finally, one should be
cognizant of the link between spina bifida patients and an
increased incidence of latex allergy, as with this patient.
The presentation of latex allergies is extremely variable.
Some patients manifest with a simple contact reaction (type
4), while others can manifest with anaphylaxis. These
patients can manifest with variable degrees of rash, bron-
chospasm, and/or circulatory collapse. This reaction is due
to a type 1 hypersensitivity reaction.
Treatment of an anaphylactic reaction involves quickly
recognizing the problem and washing out the source of
contamination. The patient should be given 100% oxy-
gen and volume loaded with a balanced salt solution
(10–20 mL/kg) with additional boluses given to main-
tain blood pressure. Epinephrine should be given as soon
as possible at 0.01–0.02 mg/kg and additional vasoactive
medications used to maintain blood pressure. In addition,
beta-agonists can be used to relieve bronchoconstric-
tion, corticosteroids to diminish mediator formation and
release, and antihistamines (H1 and H2 antagonists) to
block the action of histamine.
K E Y FAC T S
r Nearly 50% of patients with a latex allergy will have
their first reaction at the dentist’s, where there is a
tendency to use more latex-containing equipment.
r There is an association between spina bifida and an
increased incidence of latex allergy.
r Anaphylaxis is a type 1 hypersensitivity reaction.
 3 & ' & 3 & /$ &
Everett, Lucinda L.; Fuzaylov, Gennadiy; Todres, I. David. “Pediatric
Emergencies” in Cote CJ, Lerman J, Todres ID, eds. A Practice
of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:779.
16 . A N S W E R : B
Gastroesophageal reflux disease (GERD) is common in
neonates and preterm infants, with up to 40% of new-
borns regurgitating their food. This is thought to be due
at least partially to the inability to coordinate swallowing
and respiration, which does not fully develop until about
4–5 months gestational age. In addition, lower esophageal
sphincter pressures are diminished and take 3–6 weeks to
achieve adult levels. Neonatal duodenal maturity does
not occur until 29–32 weeks and may be a relevant con-
tributor in the preterm infant. Finally, CNS changes may
also contribute.
GERD must be initially differentiated from develop-
mental abnormalities such as pyloric stenosis, duodenal
stenosis, annular pancreas, malrotation, tracheoesophageal
fistula, and esophageal atresia. In general, developmental
abnormalities usually present within the first 24–36 hours
of life—upper intestinal abnormalities generally present as
recurrent vomiting and regurgitation, while lower abdomi-
nal abnormalities present with abdominal distention and
failure to pass meconium. The exception would be pyloric
stenosis, which typically presents in the third week of life.
The diagnosis of GERD often takes an accurate history and
physical examination as well as one or more of the follow-
ing tests: (1) barium swallow, (2) PH probe, (3) upper GI
endoscopy, (4) gastric emptying study.
GERD in the infant and neonate typically presents
with irritability, persistent vomiting, failure to thrive,
and in severe cases hematemesis and anemia and can be
occasionally complicated with stricture formation. In addi-
tion, reflux into the pulmonary system may lead to recur-
rent pneumonia, irritability, wheezing, and stridor.
K E Y FAC T S
r GERD is common in neonates and preterm infants,
with up to 40% of newborns regurgitating their food.
r Developmental abnormalities usually present within
the first 24–36 hours of life—upper intestinal
abnormalities generally present as recurrent vomiting
and regurgitation, while lower abdominal abnormalities
present with abdominal distention and failure to pass
meconium.
r GERD typically presents with irritability, persistent
vomiting, failure to thrive, and in severe cases
hematemesis and anemia, and can lead to recurrent
pneumonia, irritability, wheezing, and stridor.
  
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3 & ' & 3 & /$ & 4
Cote CJ. Pediatric anesthesia. In: Miller RD, Eriksson LI, Fleisher LA,
Wiener-Kronish JP, Young WL, eds. Miller’s Anesthesia. 7th ed.
Orlando, FL: Churchill Livingstone; 2009:xx–xx.
Insoft, Robert M.; Todres, I.  David. “Growth and Development” in
Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:21.
17. A N S W E R : B
This question tackles many of the issues that are associ-
ated with patients with GER in the peri-induction period.
Gastroesophageal reflux (GER) is common in neonates and
preterm infants, with up to 40% of newborns regurgitating
their food. In general, infants with the diagnosis are other-
wise healthy but may occasionally have concomitant devel-
opmental abnormalities. This is a more common scenario.
It is also important to note that the risk of aspiration on
induction is increased in all children that are developmen-
tally delayed, have had a previous esophageal surgery,
have had a difficult airway, were obese, underwent trau-
matic injury, and have coexisting GER.
Whether the patient has GER or not, it is important to
recognize the recommended fasting periods for elective sur-
gery in healthy patients and their limitations. Keep in mind
that the goal of fasting is to reduce gastric volume and that
because children are often induced via inhalation their risk
for aspiration is often protracted. Table 20.1 shows recom-
mendations that are found in ASA practice guidelines.
Premedication for GERD and the prevention of aspi-
ration pneumonitis classically involves antacids, H2 antago-
nists, proton pump inhibitors, and gastrointestinal motility
agents. Antacids work by increasing pH, and nonparticulate
antacids should be used such as sodium citrate. Particulate
antacids can cause a severe pneumonitis if aspirated and
should be avoided. H2 antagonists work by inhibiting
acid secretion in the gastric mucosa leading to increased
pH and decreased residual volume. Proton pump inhibi-
tors work in a similar manner; however, no PPI has been
approved in children below the age of 1  year. It is impor-
tant to recognize that H2 blockers and PPI need at least
30 minutes to be effective and 45 minutes–1 hour for peak
Table 20.1 "4"
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Todres ID, eds. A Practice of Anesthesia for Infants and Children.
4th ed. Philadelphia, PA: Saunders Elsevier; 2009:167.
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effect. Promotility agents such as metoclopramide work
by increasing lower esophageal tone, relaxing the pyloric
sphincter, and promoting gastric motility while decreasing
gastric volume. Metoclopramide works 1–2 minutes after
IV administration. Table 20.2 shows the recommended
dosing in the pediatric population.
3 & ' & 3 & /$ & 4
Cote CJ. Pediatric anesthesia. In: Miller RD, Eriksson LI, Fleisher LA,
Wiener-Kronish JP, Young WL, eds. Miller’s Anesthesia. 7th ed.
Orlando, FL: Churchill Livingstone; 2009:xx–xx.
Insoft, Robert M.; Todres, I.  David. “Growth and Development” in
Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:532.
18 . A N S W E R : B
Asthma is a complicated disease process that is likely a fam-
ily of related disease processes which present with a com-
mon finding of wheezing.
Critical to the understanding of asthma is an apprecia-
tion that lower airway reactivity and spasm are merely
components of the overall disease process. Inflammation
is a critical component underlying intermittent broncho-
spasm. Chronic inflammation leads to loss of appropri-
ate bronchoalveolar interaction and permanent airway
remodeling.
Therapy for asthma is directed at reducing under-
lying inflammation while controlling acute symptom-
atology. Beta agonists as well as ipratropium decrease
smooth muscle contraction and thus reduce bronchospasm.
Methylxanthines such as theophylline additionally reduce
bronchospasm but are associated with toxicity and are
becoming historical rather than practical. Corticosteroids,
both inhaled and systemic, are commonly prescribed and
used in asthmatic patients. These agents reduce swelling
and are well tolerated but are associated with hypergly-
cemia, hypertension, and loss of endocrine homeostasis.
It is useful to note that inhaled corticosteroids do induce
systemic effects as the dose escalates in comparison to
patient size. Infants may be at risk for systemic effects of
inhaled corticosteroids. Leukotriene inhibitors and mast
 
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cell stabilizers are two final classes of agents commonly
encountered in this patient population.
Immunoglobulin E–mediated atopy is a common find-
ing among asthmatics. Atopic asthmatics tend to have less
well-controlled disease as compared to nonatopic patients.
Nonatopic asthmatics may, in fact, have a subtly different
Asthmatics present with a variety of symptoms, ranging
from subtle chest tightness to respiratory failure. Patient
presentation may include air hunger, wheezing, acces-
sory muscle use, coughing, pneumothorax, mental status
change, and prolonged expiration among other associated
symptoms. Asthma, like other obstructive respiratory dis-
eases, does not manifest as prolonged inspiration.
K E Y FAC T S
r Asthma is a complicated disease that involves chronic
inflammation and lower airway reactivity.
r It commonly presents with air hunger, wheezing,
accessory muscle use, coughing, pneumothorax, mental
status change, and prolonged expiration.
r Treatment includes beta-agonists, corticosteroids,
leukotriene inhibitors, and mast cell stabilizers.
3 & ' & 3 & /$ &
Firth, Paul G.; Kinane, T.  Bernard. “Essentials of Pulmonology” in
Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:229–32.
19. A N S W E R : C
Infants and children as well as adults are fasted prior to
operations to decrease gastric volume and minimize the
risk of aspiration. The thinking behind this is that in the
fasted child the only thing that would remain in the stom-
ach would be basal gastric secretions. The risk and incidence
of aspiration in routine procedures without risk factors is
small, and it is recommended that in children without addi-
tional risk factors be fasted for 2 hours after the consump-
tion of clear liquids. These recommendations come from
studies that showed no additional decrement in risk when
fasting a child greater than 2 hours. Clear liquids include
water, fruit juices without pulp, carbonated beverages,
clear tea, and black coffee. Finally, the gastric residual
half-life of clear liquids is approximately 15 minutes, and
this information could be used to guide additional fast-
ing time for patients with some risk factors. The benefits
of fasting for 2 hours but not longer include a decreased
risk for hypoglycemia, hypovolemia, decreased thirst,
Table 20.3 13 &01&3 "5*7&
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IPVST tions in the setting of URI. Interestingly, positive-pressure
*Include only fluids without pulp, clear tea, or coffee without milk products.
†
Some centers allow plain toast (no dairy products) up to 6 hours prior to
induction.
SOURCE: Reprinted with permission from Warner MA, Caplan RA,
Epstein B. Practice guidelines for preoperative fasting and the use of
pharmacologic agents to reduce the risk of pulmonary aspiration: application
to healthy patients undergoing elective procedures: a report by the American
Society of Anesthesiologist Task Force on Preoperative Fasting. Anesthesiology.
1999;90:896–906.
decreased hunger, decreased risk for hypotension on
induction, and improved child cooperation.
Breast milk has a high lipid content, which delays gas-
tric emptying and can cause significant pulmonary injury
if aspirated. Furthermore, studies have shown that gastric
emptying times for breast milk when compared to clear
liquids are substantially greater. In addition, gastric empty-
ing times for formula are substantially greater than breast
milk. It is because of these studies that the ASA recom-
mended guidelines, as shown in Table 20.3.
3 & ' & 3 & /$ &
Ghazal, Elizabeth A.; Mason, Linda J.; Cotè, Charles J. “Preoperative
Evaluation, Premedication, and Induction of Anesthesia” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:37–8.
2 0 . A N S W E R : B
The issues surrounding the child with upper respiratory
infection have best been described as vexing. While man-
agement of children on the extreme ends of the infectious
spectrum is fairly straightforward from the surgical plan-
ning perspective, the majority of children present with
nuanced symptoms such as rhinorrhea.
Postponement of surgery is an option in children with
any infectious symptomatology. Such a stance is, however,
rather restrictive. Given that 20% to 30% of children will
present with upper respiratory infection (URI) symptoms,
the distinct possibility of another cold occurring during
convalescence is present. Layering on top of this informa-
tion, the very nature of many pediatric surgeries is aimed at
reducing the frequency and severity of infection. Examples
of such surgery include adenoidectomy, tonsillectomy, and
myringotomy. While postponing surgery 7 weeks (the time
frame required for spirometry studies to normalize) will
  
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reduce the chance of respiratory complications, this is a
practice that may not be compatible with real life and may
even put the anesthesiologist in the position of postponing
surgery that could ultimately break the cycle of infection.
Noninvasive airway management (mask or LMA) has
demonstrated a benefit in reducing respiratory complica-
ventilation alone appears to increase the risk of respiratory
complication in such patients, placing a premium on spon-
taneous ventilation.
While pharmacologic intervention is tempting for the
patient with a URI, few medications have demonstrated a
positive effect. Bronchodilators given preoperatively in oth-
erwise healthy children having minor surgery have shown
no protective effect. Anticholinergics have shown little
added benefit in this arena as well. Humidification of gasses
as well as adequate vascular hydration have some benefit in
reducing respiratory complications.
The nature of the surgery is distinctly important in
the frequency of respiratory complications in children
with a URI. Head and neck surgery as well as thoracic or
intra-abdominal surgery is associated with more frequent
respiratory complications in affected children.
A discussion of degree of illness is warranted. Viral
respiratory illness appears to be less severe than bacterial
illness. Discerning the difference between these two states
is at times difficult. Generally speaking, presence of a pro-
ductive, moist cough is associated with more severe bac-
terial infection. Additionally, fever greater than 38.5°C,
wheezing or rales on lung exam, and behavioral changes
are associated with severe disease. In addition, formerly pre-
mature children and young (less than 1 year) children tend
to have more severe responses to URIs and should be con-
sidered separately from the otherwise healthy children dis-
cussed thus far. Household smoking also appears to worsen
the outcome in affected patients.
K E Y FAC T S
r Noninvasive airway management (mask or LMA)
has demonstrated a benefit in reducing respiratory
complications in the setting of a URI.
r Positive-pressure ventilation alone appears to increase
the risk of respiratory complication in such patients,
placing a premium on spontaneous ventilation.
r Fever greater than 38.5°C, productive cough, wheezing
or rales on lung exam, and behavioral changes are
associated with severe disease.
3 & ' & 3 & /$ &
Firth, Paul G.; Kinane, T. Bernard. “Essentials of Pulmonology” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:226–9.
21. A N S W E R : A lack of decision-making ability. Between the ages of 7 and
14 years, children may develop decision-making skills, but it
Anesthetic risk for children differs from that of adult patients.
is unlikely that a patient in this age range will reliably dem-
In fact, adverse events (major and minor collectively) have
onstrate such skill. Children above the age of 14 years may
been reported to occur in 35% of pediatric cases as compared
reliably hold adequate decision-making skills. It is critical
to 17% in adult anesthesia. This difference in risk is multi-
that the practitioner not confuse this ability with the abil-
faceted and includes patient characteristics as well as surgical
ity to engage in informed consent because children of this
characteristics. Children present a number of anatomic and
age lack cognitive and emotional skills required to fully
physiologic challenges for the anesthesiologist. These chal-
participate in the process of consent.
lenges generally become more pronounced with decreasing
There are four fundamental steps to informed con-
patient age and size. Empirically, children 1  month of age
sent. First, the patient or surrogate (often a parent) must be
and younger have been reported to have the highest rate of
supplied adequate information to understand both his or
adverse event. Importantly, the same database that yielded
her medical condition and the procedure planned for him
this finding also reported that children under 1  month of
or her. Second, the practitioner must assess the patient or
age underwent cardiac and major vascular surgery more fre-
surrogate for understanding of the information laid out
quently than did children in other age groups.
in the first step. Third, the practitioner must assess the
Particular organ systems appear to be more prone to
patient/surrogate for capacity (cognitive, emotional, and
adverse event than others. In a second database, respira-
decision making) to engage in consent. Fourth, the practi-
tory adverse events accounted for 53% of all intraoperative
tioner must deliver an assurance that the patient/surrogate
events. Infants, patients undergoing ENT surgery, patients
may make the decision free of coercion.
in whom the trachea was intubated, and patients with
Pediatric informed consent is further complicated by
ASAPS scores of 3 and above were more likely to have a
several unique situations. First, the surrogate may (and
respiratory adverse event. Cardiac adverse events accounted
often does) ask the physician what the physician would do
for 12.5% of all such events. Nausea occurred in 6% of cases
in a given setting. This question is fundamentally flawed
and appeared (in both databases) to occur in older children.
because the child simply is not the physician’s. Any declara-
Cohen reports that one third of 5-year-old patients experi-
tive answer to this question is simply a guess. No physician
enced vomiting in recovery.
knows his or her patient well enough to claim to know or
understand all the complexity of the parent–child rela-
K E Y FAC T S tionship. The best answer to this question will be one that
addresses the goals that the family is attempting to
r Adverse events have been reported to occur in 35% of
achieve and the values they hold. Second, some children
pediatric cases, as compared to 17% in adult cases.
obtain emancipated minor status via the legal system. These
r Children 1 month of age and younger have been
individuals are typically adolescent and either married,
reported to have the highest rate of adverse event.
parents, in the military, or economically independent
r Most adverse events are respiratory (53%) or cardiac
of their parents for any of a variety of reasons. Each state
(12.5%) in nature.
handles these individuals and their rights uniquely, but
in general they are recognized as holding the right to par-
3 & ' & 3 & /$ & 4 ticipate in informed consent. Third, there exist religious
and cultural differences between patients and families. Of
Cohen MM, Cameron CB, Duncan PG. Pediatric anesthesia mor- common concern are children of Jehovah’s Witnesses.
bidity and mortality in the perioperative period. Anesth Anelg. Without great detail, such families generally refuse blood
1990;70(2):160–7.
Murat I, Constant I, Maud’Huy H. Perioperative anesthetic morbid-
transfusion. Practitioners may or may not agree with this
ity in children:  a database of 24,165 anesthetics over a 30-month choice. Furthermore, it is debatable whether the child must
period. Pediatr Anesth. 2004;14:158–66. be treated as an informed practitioner of the religion or cul-
tural practice. Each clinician must decide on a case-by-case
basis whether to support this decision. Consultation with
22 . A N S W E R : C
representatives of the legal system prior to surgery may be
Informed consent, a complicated concept for adult patients, wise prior to surgery if a conflict exists between the practi-
is significantly more complicated in the arena of pediatrics tioner and the family/patient regarding such religious and
because children are widely deemed to lack the cognitive, cultural norms and their impact on medical care.
emotional, and decision-making skills required to partici-
pate alone through the consent process. Assent, however,
K E Y FAC T S
is obtainable according to the child’s capability. A  “rule
of sevens” aids in this assent process. Children younger r Children above the age of 14 years may reliably hold
than 7 years of age are typically incapable of assent due to a adequate decision-making skills, but they often lack

 
r
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 the cognitive and emotional skills required to fully
participate in the process of consent.
r An emancipated minor (adolescents that are married,
parents, in the military, or economically independent) is
generally recognized as holding the right to participate
in informed consent.
r Consultation with representatives of the legal system
prior to surgery may be wise if a conflict exists between
the practitioner and the family/patient regarding
such religious and cultural norms and their impact on
medical care.
3 & ' & 3 & /$ &
Waisel, David B. “Ethical Issues in Pediatric Anesthesiology” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:73–6.
23 . A N S W E R : D
Lab testing and other studies such as EKG and lung func-
tion tests are certainly useful in aiding the assessment of
the appropriate patient. Metabolic and electrolyte testing is
useful in patients with disorders of the cardiac, gastrointes-
tinal, and genitourinary systems as well as patients receiving
psychiatric medications. Blood counts are useful in the
setting of significant blood loss and hematologic disor-
ders as well as children with oncologic disorders. More
important than memorizing tests is that the practitioner
arrive at a point where he or she understands why a test is
being used and when it is warranted.
A fundamental understanding of the patient’s current
physiology and the anticipated surgical procedure are essen-
tial in driving preoperative assessment. A  useful starting
point is to obtain laboratory studies that will either (a) yield
information regarding the patient’s current status, (b) alter
further assessment (such as blood typing and screening for
anemic patients), or (c)  alter the patient’s surgical plan-
ning (such as electrolyte testing for dehydrated patients).
A discussion regarding echocardiography in particular is
warranted. In the context of congenital heart disease (CHD)
it is often useful to obtain a recent echocardiogram. Given
that children with CHD undergoing surgery often mani-
fest symptoms consistent with cardiac failure, visualization
of current anatomy and function provides the practitioner
a wealth of information. Cardiac failure in children may
manifest much more obscurely than in adults. Simple failure
to match peers in growth raises many questions regarding a
neonate’s cardiac status. Rarely is the same true for adults.
Given that cardiac dysfunction is difficult to diagnose clini-
cally in pediatrics, empiric evaluation via echocardiogra-
phy is frequently warranted. Children with cardiac disease
often have undergone repeated echocardiography, which
may be useful in assessment. A discussion with the patient’s
cardiologist is often helpful to this end.
  
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Given that the patient described in this question has no
comorbidities and is undergoing a benign surgical procedure
with little anticipated blood loss, no testing is warranted.
3 & ' & 3 & /$ & 4
Ghazal, Elizabeth A.; Mason, Linda J.; Cotè, Charles J. “Preoperative
Evaluation, Premedication, and Induction of Anesthesia” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:43–4.
Miller-Hance, Wanda C. “Anesthesia for Noncardiac Surgery in
Children with Congenital Heart Disease” in Cote CJ, Lerman J,
Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th
ed. Philadelphia, PA: Saunders Elsevier; 2009:467–8.
2 4 . A N S W E R : C
Parent presence on induction of anesthesia (PPI) is a process
of variable popularity that holds many potential advantages
as well as disadvantages. Each practitioner and institu-
tion must carefully develop and adopt a position regard-
ing the practice of PPI; no national standard is available.
Commonly, patient age comes under question in develop-
ment of such a plan. While it is intuitive that adult patients
often do not require or request PPI, unique patients may
benefit from PPI. Such patients may be developmentally
delayed or even demented, calling into question the appro-
priateness of more broad practices such as family present
induction for the elderly.
Both benefits and challenges exist in regard to PPI.
Potential benefits include a lessened requirement for preop-
erative sedation medications, less frequent emergence delir-
ium, and a more benign induction process. Importantly,
data regarding these benefits are mixed, and it cannot be
conclusively stated that these benefits in fact exist across all
patients. Disadvantages of PPI include requirement of addi-
tional staff, possible disruption of the operating room and
sterile area, and harm either to or inflicted by the parent.
The parent, it must be recognized, is not an inert cog in this
machine but brings with him or her a degree of unpredict-
ability. The parent must be counseled regarding what will be
seen in the operating room and what each individual’s role
is in the event of an emergency, parent included. Beyond the
possibility that the parent may disrupt the operative envi-
ronment, the operative environment may harm the parent!
Data exist describing elevations in heart rate, blood pres-
sure, and skin conductance in parents participating in PPI.
While no harm has been reported to parents during PPI, it
is important that practitioners fully recognize the gambit of
outcomes that may develop in the context of PPI.
3 & ' & 3 & /$ &
Kain, Zeev N.; MacLaren, Jill; Maes, Linda C. “Perioperative Behavior
Stress in Children” in Cote CJ, Lerman J, Todres ID, eds. A Practice
 of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:28–31.
25 . A N S W E R : D
Psychosocial preparation of the pediatric patient is multi-
faceted in regard to both breadth and patient applicability.
Common techniques aim at creating trusting relationships
with health care providers, using age-appropriate terminol-
ogy, and developing coping skills.
Of interest, patient age is an important consideration
in psychosocial preparation programs. The fundamental
consideration appears to be the child’s ability to discern
between fantasy and reality. At approximately 3 years of
age, children become able to differentiate between these
two states. With this development, the child becomes able
to benefit from simulation meant to prepare him or her for
upcoming surgery. As such, children younger than 3 years
may not benefit from such programs.
Older children demonstrate an interesting nuance to
preparatory programs as well. Children aged 6  years and
older have demonstrated an increase in anxiety leading up
to the date of preoperative preparation. In fact, this anxi-
ety peaked in the days surrounding such preparation and
fell below baseline levels 5 days following preparation.
Given this trend, it is critical that older children be sched-
uled for preparation a week in advance of surgery.
Regarding a parent’s influence on a child’s level of anxi-
ety, it is not surprising that the values positively correlate.
Anxious parents beget anxious children. Preparation must
include the family in addition to the patient. Furthermore,
ideal preparation will clearly convey to the parent how
directly their state of mind influences that of their child.
Children who have been hospitalized in the past are a
unique population. A trend toward an enhanced emotional
response to hospitalization is present in children who
have been hospitalized in the past. A prior negative medi-
cal experience additionally promotes anxiety. Appropriate
preparation for such patients may be best targeted at spe-
cific experiences.
3 & ' & 3 & /$ &
Ghazal, Elizabeth A.; Mason, Linda J.; Cotè, Charles J. “Preoperative
Evaluation, Premedication, and Induction of Anesthesia” in
Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:39–41.
2 6 . A N S W E R : B
Cerebral palsy, a static encephalopathy, is the most com-
mon childhood movement disorder (Table 20.4). Cerebral
palsy has an incidence of 1/7000 births and a prevalence
of 1/5000. Often there are no identifiable risk factors, and
the diagnosis is made when a patient exhibits delayed motor
development. Despite the static nature of neurologic dam-
age, the clinical picture for this disease state is variable
and may change. It is thought that insults to the imma-
ture CNS or congenital anomalies cause cerebral palsy, as
birth asphyxiation, prematurity, and intrauterine growth
retardation are known associations. Approximately 75%
of cases involve in utero insults to the immature CNS,
10% of cases occur during birth, and the remaining 15%
of cases occur shortly after birth. Despite immature CNS

Table 20.4 $&3 &#3 "-ु1"-4:

TYPE/ETIOLOGY MOTOR DEFICIT DISTR IBUTION COMPLICATIONS

Hypotonic -PX
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EJTBCJMJUZ

4ZOESPNJD 7BSJBCMF
MJNCुUPOF $POUSBDUVSFT
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UFOEPO
SFĔFYFT
VTVBMMZ
&QJMFQTZ
*OTVMUुIZQPYJBJTDIFNJB JODSFBTFE 'FFEJOH
EZTGVODUJPO
)FBSJOHWJTJPO
JNQBJSNFOU
3FTQJSBUPSZ
JOGFDUJPOT
Spastic *ODSFBTFE
UPOFुQZSBNJEBMुUZQF .POPQBSFTJT
*OTVMUुIZQPYJBJTDIFNJB
*ODSFBTFE
EFFQ
UFOEPO
SFĔFYFT %JQBSFTJT
WBTDVMBS )FNJQBSFTJT
5SJQBSFTJT
5FUSBQBSFTJT
Choreoathetoid *OWPMVOUBSZ
NPWFNFOUुPĕFO
.BZ
CF
EJĈVTF
UFUSBQBSFTJT

PS
)FBSJOH
JNQBJSNFOU
*OTVMUुIZQPYJBJTDIFNJB
B
NJYUVSF
PG
DIPSFB
BUIFUPTJT
DPOđOFE
UP
POF
PS
NPSFुMJNCT $POUSBDUVSFT
OFPOBUBM
IZQFSCJMJSVCJOFNJB
EZTUPOJB 0ĕFO
DPFYJTUT
XJUI
TQBTUJDJUZ *OUFMMFDU
PĕFO
NBJOUBJOFE
NFUBCPMJD
Ataxic "UBYJB
VTVBMMZ
HFOFSBMJ[FE
.BZ
CF
EJĈVTF
CVU
PĕFO
BTTPDJBUFE
'FX
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CF
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SOURCE: Reprinted with permission from: Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:493.

 
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injury, cognitive impairment is not a consistent feature of
cerebral palsy.
Cerebral palsy is classified according to its severity, dis-
tribution, and nature of motor deficit. The four major classi-
fications of cerebral palsy are spastic, athetoid or dyskinetic,
ataxic, and mixed. Without the normal gravitational
muscular stresses, abnormal skeletal muscle development
ensues, and patients with cerebral palsy often require multi-
ple orthopedic surgeries to loosen tight muscles and joint
contractures, and to straighten abnormal long bone twists
and kyphoscoliosis.
Although severe scoliosis compromises respiratory
reserve, idiopathic scoliosis by itself is not strongly associ-
ated with impaired oromotor function or recurrent aspira-
tion pneumonia.
Similarly, pulmonary reserve is compromised in
patients with cystic fibrosis (CF), but severe CF does not
affect oromotor function. Rather, severe CF disrupts elec-
trolyte transport in epithelial cells of sweat ducts, airway,
pancreatic duct, intestinal, biliary tree, and vas deferens.
Although Trisomy 21 is associated with duodenal atresia
and generalized hypotonia, severe cerebral palsy is more
often associated with severe seizure disorder, impaired
bulbar function, and recurrent aspiration pneumonia.
With severe bulbar dysfunction, some patients with cere-
bral palsy have difficulties feeding and speaking. Coupled
with significant gastroesophageal reflux disease and poor
laryngeal reflexes, patients with cerebral palsy can have
recurrent aspiration pneumonia and subsequent reduced
pulmonary reserve.
3 & ' & 3 & /$ & 4
Crean P, Hicks E. “Essentials of Neurology and Neuromuscular Disease”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:492–3.
Anderson B, Wilton N. “Orthopedic and Spine Surgery” in Cote CJ,
Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:651–2.
Urban, MK. “Anesthesia for Orthopedic Surgery” in Miller RD,
Eriksson LI, Fleisher L, Wiener-Kronish JP, eds. Miller’s Anesthesia.
7th ed. Philadelphia, PA: Churchill Livingstone; 2010:2248.
27. A N S W E R : C
Cystic fibrosis (CF) demonstrates autosomal recessive
inheritance and occurs in 1/2000 Caucasian births. The
pathophysiology for CF involves chronic infection,
mucus plugging, inflammation, and epithelial injury,
as the mutation in the gene encoding CF transmembrane
conductor regulator disrupts electron transport via chlo-
rine channel in various epithelial cells. This renders CF
patients to the symptoms of productive cough, hemop-
tysis, recurrent respiratory infections, atelectasis, and
maldigestion.
  
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Treatment for CF focuses on nutritional reple-
tion with high-calorie diets, continuation of pancreatic
enzyme replacement, and fat-soluble vitamin supple-
ments; and relief of airway obstruction, including pre- and
postoperative bronchodilator use, incentive spirometry,
postural drainage, and pathogen-specific antibiotics
for lung infection. Other areas currently being evaluated
for treatment of CF include anti-inflammatory medication
and lung transplant. Answer A is incorrect because preop-
erative bronchodilators should be continued to optimize
postoperative outcomes. Answer B is incorrect because
pathogen-specific antibiotics should be administered. In
addition, prophylactic broad-spectrum antibiotics may har-
bor multidrug resistance. Answer D is incorrect because
postoperative outcomes are optimized when CF patients
continue high-calorie diets, coupled with replacement of
pancreatic enzymes and fat-soluble vitamin supplements.
3 & ' & 3 & /$ & 4
Firth P, Haver K. “Essentials of Pulmonology” in Cote CJ, Lerman J,
Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th
ed. Philadelphia, PA: Saunders Elsevier; 2009:233–5.
Holzman RS, Mancuso TJ, Sethna NF, DiNardo JA. “Respiratory
System” in Holzman RS, Mancuso TJ, Sethna NF, DiNardo JA,
eds. Pediatric Anesthesiology Review. New  York, NY:  Springer;
2010:48–9.
2 8 . A N S W E R : B
The best way to prevent latex allergy is to remove latex
from hospitals. To this end, many children’s hospitals are
converting to completely latex-free environments. Latex
allergy is IgE mediated, and clinical diagnosis requires a
high index of suspicion.
The following individuals are at increased risk of ana-
phylaxis to latex:  children with neural tube defects such
as spina bifida and myelomeningocele, children with
urinary tract anomalies requiring frequent bladder cath-
eterization, children with an immunologic predisposition,
children undergoing multiple surgical procedures, individ-
uals with an allergy to tropical fruits such as banana and
kiwi, and health care workers exposed to latex.
Although it has been demonstrated that pretreatment
with antihistamine and corticosteroid can prevent a reac-
tion to intravenous contrast, these preventative treatments
do not reliably prevent anaphylaxis to latex. Thus, Answers
A and C are incorrect.
If anaphylaxis is clinically suspected, the most impor-
tant treatment is intravenous epinephrine (1–10 µg/kg)
to prevent further mast cell degranulation. In addition,
the offending agent should be removed or diluted, 100%
oxygen should be administered, the patient should be intu-
bated if not already, acute volume loading should occur, and
the patient may require an infusion of epinephrine or other
vasoactive mediations to support the patient’s blood pres- anterior mediastinal mass, this patient may demonstrate
sure. If possible, a blood sample should be sent for mast cell extrathoracic or intrathoracic variable or fixed obstruc-
tryptase test to confirm anaphylaxis, but this is certainly tion, and this would be apparent on flow-volume studies
not the first or most important step in treating anaphylaxis. (see Fig. 20.1).

3 & ' & 3 & /$ & 3 & ' & 3 & /$ & 4

Everett LL, Fuzaylov G, Todres ID. “Pediatric Emergencies” in Cote CJ,
Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:779.
29. A N S W E R : D
Firth PG, Haver KE. “Essentials of Pulmonology” in Cote CJ, Lerman
J, Todres ID, eds. A Practice of Anesthesia for Infants and Children.
4th ed. Philadelphia, PA: Saunders Elsevier; 2009:227.
Hammer GB. “Anesthesia for Thoracic Surgery” in Cote CJ, Lerman
J, Todres ID, eds. A Practice of Anesthesia for Infants and Children.
4th ed. Philadelphia, PA: Saunders Elsevier; 2009:291.

Pediatric anterior mediastinal masses may be primary or

metastatic tumors of the lung, mediastinum, and pleura.
Associated signs and symptoms are typically due to air-
way or vascular compression and include stridor, dys-
pnea, orthopnea, cough, pain, pleural effusion, and SVC
syndrome. This patient demonstrates dyspnea, orthopnea,
cough, and SVC syndrome.
Preoperative CT scan, cardiac echocardiogram, and
pulmonary function tests should be obtained for anterior
mediastinal masses. However, this question asks which
preoperative study is most appropriate to detect dynamic
airway compression. A plain chest X-ray does not detect
dynamic airway compression. Images from CT scan are
static and may not necessarily identify dynamic airway
compression, which may occur with the induction of gen-
eral anesthesia. Cardiac echocardiogram can show SVC
or pulmonary outflow tract compression, but flow-volume
studies are most effective in detecting evidence of dynamic
airway compression. Depending on the location of this
3 0 . A N S W E R : A
Although potential symptoms of a URI and a potential
difficult airway should alert any pediatric anesthesiologist,
the cardiovascular assessment of this patient with Williams
syndrome supersedes other perioperative concerns. With
only rhinorrhea and a dry cough, some would argue that
this child does not have an active URI. Micrognathia with
prominent incisors does not necessarily equate with diffi-
cult airway, but it would be prudent to have advanced and
alternative airway equipment readily available to intubate
this patient.
Williams syndrome occurs in 1/20,000 children and
involves a deletion of chromosome 7, which alters the
elastin gene. Patients with Williams syndrome have charac-
teristic elfin facies, outgoing personalities, endocrine abnor-
malities, mental retardation, growth deficiency, and altered
neurodevelopment. Associated cardiovascular pathology

(A) (C)
Exhalation

6.0
Flow (L/sec)

0.0
6.0

(D)
Inhalation

–6.0

Spirometry Prebron- Observed Postbron- Observed Percent

Parameter Units chodilator Percent Pred chodilator Percent Pred Change
FVC Liter 3.53 102 3.63 106 3
FEV1 Liter 2.63 89 2.65 90 0
FEV1/FVCa Percent 74.7 72.8 –3

Pulmonary function test from a child with an intrathoracic airway obstruction (vascular ring). (A)The flow-volume curve shapes suggest
'JHVSFु
a fixed expiratory obstruction. The shape of the inspiratory link is normal; the expiratory flow limb is flattened on both the prebronchodilator
and postbronchodilator flow-volume curves. (B) A magnetic resonance angiogram accompanies the flow loop. (C) Slit-like tracheal compression
before repair. (D) Note the marked improvement in the tracheal lumen after division of the vascular ring. Post, postbronchodilator; Pre,
prebronchodilator; Pred, predicted. (Reprinted with permission from Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:227.)

 
r
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Echocardiogram displaying the classic supravalvar aortic
'JHVSFु
narrowing in a patient with Williams syndrome (arrows). Ao V, aortic
valve; Asc Ao, ascending aorta.
includes valvular and supravalvular aortic stenosis, and
coarctation of the aorta. These children also demonstrate
arteriopathy that may involve the origin of the coronary
arteries.
Children with Williams syndrome are predisposed to
increased anesthetic morbidity and potential mortality in
the form of coronary artery stenosis and severe biven-
tricular outflow tract obstruction, which may occur with
the induction of general anesthesia. The need for surgery
and general anesthesia should be seriously considered, and
thorough cardiac evaluation should be pursued, with special
attention to cardiac echocardiogram (Fig. 20.2) and, if pos-
sible, cardiac catheterization data. With potential coronary
artery stenosis in the setting of aortic stenosis with already
compromised coronary artery filling during diastole, this
patient is at a greatly increased risk of cardiac morbidity and
mortality with anesthesia.
K E Y FAC T S
r Williams syndrome occurs in 1/20,000 children and
involves a deletion of chromosome 7, which alters the
elastin gene.
r Associated cardiovascular pathology includes valvular
and supravalvular aortic stenosis, and coarctation of
the aorta.
r Children with Williams syndrome are predisposed to
increased anesthetic morbidity and potential mortality
in the form of coronary artery stenosis and severe
biventricular outflow tract obstruction, which may
occur with the induction of general anesthesia.
  
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0 ' ु $ 0 & 9 * 4 5 * / (
% * 4 & " 4 &
r

3 & ' & 3 & /$ &
Slesnick TC, Gertler R, Miller-Hance WC. “Essentials of Cardiology”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:303–4.
31. A N S W E R : A
Many children with congenital heart disease will present for
noncardiac surgeries, so pediatric anesthesiologists should
be well versed in the appropriate hemodynamic goals and
appropriate management of these patients. This scenario
presents an urgent case where a young boy may lose a testis
if his testicular torsion is not corrected in a timely manner.
Tetralogy of Fallot (TOF) is the most common cya-
notic cardiac condition and is characterized by the tet-
rad of pulmonary stenosis, right ventricular outflow tract
obstruction, overriding aorta, and ventricular septal defect
(Fig. 20.3). With TOF, right-to-left shunt results in cya-
nosis, and these patients are prone to “tet spells” or hyper-
cyanotic episodes. Tet spells occur when pulmonary blood
flow is decreased in patients who already have significant
right ventricular outflow tract obstruction. Crying and
Ao
SVC
PA
LA
RA
LV
Pulmonary
stenosis
Right
ventricular
hypertrophy
RV
IVC
Tetralogy of Fallot. This diagram shows the features of the
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tetralogy: VSD, overriding aorta, right ventricular hypertrophy, and
pulmonary stenosis. Shown here is both pulmonary and subpulmonary
obstruction. The result is right-to-left shunting resulting in cyanosis.
AVC, superior vena cava; RA, right atrium; RV, right ventricle; LA, left
atrium; LV, left ventricle; PA, pulmonary artery; Ao, aorta. (Reprinted
with permission from: Cote CJ, Lerman J, Todres ID, eds. A Practice of
Anesthesia for Infants and Children. 4th ed. Philadelphia, PA: Saunders
Elsevier; 2009:348.)
inadequate depth of anesthesia are known triggers for tet
spells and exacerbate right-to-left shunting.
Nonpharmacological treatment of tet spells includes
Valsalva maneuvers and assuming the squatting position.
Further treatment of tet spells includes increasing anes-
thetic depth, systemic vascular resistance, intravascu-
lar volume, and inspired oxygen concentration. SVR is
typically increased pharmacologically with phenylephrine.
Crystalloid or colloid may be used to increase intravascu-
lar volume. Patients already under general anesthesia may
also benefit from lowered inspiratory ventilator pres-
sures or beta blockade, as these decrease pulmonary vas-
cular resistance and infundibular spasm, respectively.
With regard to the answer choices listed, oral midazolam
has great bioavailability, but it requires a cooperative patient
and 10–15 minutes for maximal sedating effects. With supple-
mental oxygen, this benzodiazepine will sedate the child so he
will separate from his parents easily and tolerate mask induc-
tion, all within the safe time frame to begin this urgent surgery.
The sympathomimetic effects of intramuscular ket-
amine and the intramuscular route of administration are
not ideal in this situation. Although an argument could be
made that intramuscular ketamine will sedate the child,
the intramuscular route of administration will likely cause
more crying, and the sympathomimetic properties of ket-
amine may worsen infundibular spasm in this child having
a hypercyanotic episode.
Further attempts at awake peripheral intravenous place-
ment are also likely to cause additional pain and crying in
this patient, who is already experiencing a hypercyanotic
episode. Although increasing intravascular volume is part
of the management strategy for this patient, it is more
important to increase the patient’s level of sedation and
provide increased inspired oxygen content in a timely man-
ner. It would be irresponsible to not immediately treat this
patient who requires urgent surgery.
3 & ' & 3 & /$ &
McEwan A. “Anesthesia for Children Undergoing Heart Surgery”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:348.
32 . A N S W E R : B
A TOF patient having a hypercyanotic episode, no intra-
venous access, and presenting for urgent surgery demands
thoughtful perioperative consideration. The urgency of this
surgery should be reiterated, as the patient’s torsed testis
may potentially be saved if its blood supply is restored soon.
Most testes can be saved if surgery is performed within
6 hours of the onset of pain. This salvage rate decreases to
50% if surgery is performed between 6 and 12 hours of the
onset of pain.
 
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Patients with testicular torsion should be considered to
have full stomachs. To safely anesthetize this TOF patient
with a full stomach, no intravenous access, and an urgent sur-
gery requires balancing of anesthetic goals. The most impor-
tant considerations are to increase depth of anesthesia and
inspired oxygen concentration in a timely and safe manner.
Although inhalation induction will likely be slower in
this patient because of reduced pulmonary blood flow
given his TOF physiology, the best way to attenuate this
child’s tet spell with the given answer choices is to increase
FiO2 and inhaled concentration of sevoflurane. In efforts
to prevent regurgitation of gastric contents, cricoid pres-
sure should be used during inhalation induction. After the
induction of anesthesia, an intravenous should be quickly
placed and the patient’s airway secured.
A eutectic mixture of local anesthetics (EMLA; 2.5%
lidocaine and 2.5% prilocaine) requires 30 minutes to have
maximal benefit, so this is not the best choice to relieve a
tet spell in this patient already in the operating room, as
there will not be enough time to allow this medication to
work. If EMLA was not used, or a peripheral intravenous
was placed prior to maximal effectiveness of EMLA, this
patient would likely continue crying, which would worsen
the infundibular spasm already associated with this
hypercyanotic episode.
Similarly, an intramuscular dose of ketamine would
increase the patient’s level of sedation but at the expense of
worsening the hypercyanotic episode, as the intramuscu-
lar route of drug delivery would likely worsen crying and
infundibular spasm because of pain and ketamine’s sympa-
thomimetic properties.
An inhalational induction could theoretically be
expedited via second gas effect from nitrous oxide and
sevoflurane, but decreased FiO2 is not congruent with
the management strategy for a child having a tet spell, so
Answer C is incorrect.
3 & ' & 3 & /$ &
Lönnqvist P, Lerman J. “General Abdominal and Urologic Surgery”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:591.
33 . A N S W E R : B
Although tonsillectomy and adenoidectomy are commonly
performed ENT procedures, it would be irresponsible for
the anesthesiologist caring for this child to not know the
extent of cardiac defect that this child has prior to adminis-
tering a general anesthetic.
Trisomy 21 was first described by John Langdon Haydon
Down in 1887 and is the most common chromosomal
abnormality. It has an incidence of approximately 1/800
live births. Advanced maternal age is a known risk factor
for Down syndrome, and Down syndrome may affect up to
4% of children born to women over the age of 40.
Roughly 40%–50% of patients with Down syndrome
have some sort of cardiac abnormality. These range from
innocent childhood murmurs to complete atrioventricu-
lar septal defects (AVSDs). Atrioventricular septal defects,
or endocardial cushion defects, may be partial or complete.
Complete AVSDs typically consist of a large septal defect
with atrial and ventricular components and a common atrio-
ventricular valve, and may include conduction abnormalities.
Although a plain chest X-ray may give information
regarding cardiomegaly, which may reflect congestive heart
failure, this preoperative study does little to confirm the
patient’s functional or cardiovascular status and is more use-
ful when compared to prior imaging. Moreover, congestive
heart failure is not suspected in this patient who is growing
well and has no sweating or cyanotic episodes with feeds.
Similarly, a cardiac stress test is unlikely to yield sub-
stantial clinically important data, as there are no immedi-
ate concerns regarding myocardial ischemia in this child.
A cardiac stress test would be more pertinent in a patient
with coronary artery abnormalities, aortic stenosis, or
exercise-induced arrhythmias.
Based on the pertinent history obtained by the parents,
this child does not have overt signs of congestive heart fail-
ure, as she feeds well and does not sweat or become cyanotic
with feeds. Children with congestive heart failure may have
nasal flaring, grunting with respirations, chest retrac-
tions, poor feeding and growth, and diaphoresis.
The most appropriate answer selection is to clarify the
child’s cardiac defect and physiology by obtaining an EKG
and cardiac echocardiogram. This information should be
used to tailor this child’s anesthetic, as a Down syndrome
patient with complete atrioventricular septal defect may
have severe pulmonary hypertension, may have severe
conduction abnormalities, and may require inotropic sup-
port (Fig. 20.4). Elucidation of the extent of this patient’s
cardiac defect affects anesthetic technique and postopera-
tive recovery placement.
Additional cardiovascular concerns for Down syn-
drome patients include the propensity for bradycardia
with the induction of general anesthesia, and the potential
for pulmonary hypertension, either from cardiac pathol-
ogy or chronic hypoxia from upper airway obstruction,
especially in this patient who also has severe obstructive
sleep apnea.
Other major perioperative concerns for Trisomy 21
include airway and vascular access concerns. Patients with
Trisomy 21 often have chronic pulmonary infections, mac-
roglossia with anterior, smaller airways, the potential for
atlanto-occipital dislocation, increased incidence of
postextubation stridor, and potential for difficult vascu-
lar access.
K E Y FAC T S
r Roughly 40%–50% of patients with Down syndrome
have some sort of cardiac abnormality.
r Children with congestive heart failure may have nasal
flaring, grunting with respirations, chest retractions,
poor feeding and growth, and diaphoresis.

(A) (B)

Ao

PA LA

LA Common A-V
RA value
RA
LV
LV

RV
RV

(A) Diagram of ventricular septal defect showing left-to-right shunt. (B) Diagram of complete atrioventricular septal defect (AVSD)
'JHVSFु
with great vessels removed showing left-to-right shunt through both atrial and ventricular components of the defect and also a single common
atrioventricular valve. RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; PA, pulmonary artery; Ao, aorta. (Reprinted
with permission from: Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th ed. Philadelphia, PA: Saunders
Elsevier; 2009:346.)

  
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r Additional cardiovascular concerns for Down syndrome
patients include the propensity for bradycardia with the
induction of general anesthesia and the potential for
pulmonary hypertension, either from cardiac pathology
or chronic hypoxia from upper airway obstruction.
3 & ' & 3 & /$ &
Miller-Hance WC. “Anesthesia for Noncardiac Surgery in Children
with Congenital Heart Disease” in Cote CJ, Lerman J, Todres
ID, eds. A Practice of Anesthesia for Infants and Children. 4th ed.
Philadelphia, PA: Saunders Elsevier; 2009:303, 346, 473–4.
3 4 . A N S W E R : D
Patients with autism spectrum disorder have a unique set
of perioperative concerns. Disease states along the autism
spectrum include milder forms like Asperger’s syndrome
and severe autism, which may be associated with schizo-
phrenia. When assessing patients with autism spectrum
disorders preoperatively, the patient’s cognitive capacity
and potential effects of psychiatric medications should be
considered.
Answer A  is not the most reliable method of induc-
ing general anesthesia because intravenous placement is
often one of the most challenging aspects of pediatric
anesthesiology, especially for an awake autistic teenager
who will not even allow caretakers to brush his teeth. This
patient likely will not cooperate with an awake intravenous
placement.
Similarly, inhalational induction is not always reli-
able in this patient population. Parental presence or use of
familiar comfort devices such as headphones may ease an
autistic patient and make an inhalational induction pos-
sible, but this is not the most reliable means of inducing
general anesthesia in this patient either.
Although oral midazolam has good bioavailability, it
requires a cooperative patient who will reliably ingest this
potentially bitter-tasting medication, and it requires 10–15
minutes to work. If this patient does not take the com-
plete oral dose of midazolam or spits it out, as may occur
with severely autistic children, then this medication will
not work. So Answer C is not the most reliable method of
inducing general anesthesia for this child.
Given these answer choices, the most reliable way to
induce general anesthesia with this severely autistic patient is
with intramuscular premedication with ketamine, then
inhalational induction with sevoflurane after the patient
is sedated. Alternatively, one could also place a peripheral
intravenous once the patient is adequately sedated, then
proceed with an intravenous induction. Dosing of intra-
muscular ketamine should take into account the antici-
pated length of surgery, so as to avoid prolonged hospital
recovery or time to discharge from overzealous ketamine
administration.
 
r
$ - * / * $ " -
4 $ * & / $ &
0 ' ु " / & 4 5 ) & 4 * "
3 & ' & 3 & /$ & 4
Ghazal EA, Mason LJ, Coté CJ. “Preoperative Evaluation,
Premedication, and Induction of Anesthesia” in Cote CJ, Lerman J,
Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th
ed. Philadelphia, PA: Saunders Elsevier; 2009:57–8.
Reves JG, Glass PSA, Lubarsky DA, McEvoy MD, Martinez-Ruiz R.
“Intravenous Anesthetics” in Miller RD, Eriksson LI, Fleisher L,
Wiener-Kronish JP, eds. Miller’s Anesthesia. 7th ed. Philadelphia,
PA: Churchill Livingstone; 2010:746–7.
35 . A N S W E R : B
Teenagers having surgery are commonly anxious; judicious
use of preoperative anxiolytics may allay this nervousness.
Oral or intravenous anxiolytics may be administered in
the preoperative area. Although important to ensure the
smooth induction of a general anesthetic, anxiolysis is not
the most important concern for this patient and her upcom-
ing major surgery.
Thoughtful discussions of blood conservation strategies
are the most important part of this patient’s perioperative
discussion and would have ideally begun weeks prior to this
scheduled surgery. Had this discussion begun weeks prior,
then the patient could have been started on erythropoi-
etin and iron supplements to increase her production of
red blood cells.
Major blood loss can occur with posterior spinal sur-
gery because of bleeding from epidural vessels during the
posterior surgical approach. Proper positioning of patients
in the prone position without undue abdominal pressure
will avoid venous congestion and may minimize intraop-
erative blood loss attributable to positioning. Other poten-
tial strategies to prevent or limit blood loss in a Jehovah’s
Witness include the aforementioned preoperative iron and
erythropoietin supplementation, appropriate prone posi-
tioning, intraoperative cell savage, use of colloid volume
expanders, use of antifibrinolytics epsilon aminocaproic
acid (EACA, Amicar) or transexamic acid, maintenance
of normothermia, surgical use of fibrin glue, and staged
surgery or agreeing to stop surgery after a predefined total
blood loss or hematocrit have been determined.
Some Jehovah’s Witness patients are agreeable to intra-
operative cell savage so long as the entire system remains
in continuity with their vasculature. Whether or not this
patient and her family agree to cell savage is an important
aspect of this patient’s surgical care that should be eluci-
dated preoperatively. Similarly, the potential use of albumin
should be discussed preoperatively. Jehovah’s Witnesses
may object to albumin because it is prepared from pools of
human plasma, so other plasma expanders like the synthetic
colloid hetastarch should be considered. Hetastarch may
be associated with renal insufficiency in select patients, so
its use is controversial.
Antifibrinolytics are lysine analogs that bind plasminogen
and plasmin and effectively prevent fibrinolysis. They have
been shown to significantly decrease intraoperative bleeding with malignant hyperthermia, but idiopathic scoliosis is
in major surgeries like posterior spinal fusion and open heart not (see Box 20.1).
surgery. Transexamic acid has 6 to 10 times the potency of
epsilon aminocaproic acid and has a longer half-life.
Other blood conservation strategies for patients who Box 20.1 463(*$"-
"/%
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are willing to accept transfusion of blood or blood prod- 13*/$*1-&4
0'ु#-00%-&44
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ucts include preoperative autologous donation, acute ."/"(&.&/5
normovolemic hemodilution, and deliberate hypo-
tension. Autologous blood units may be stored for up to
42  days, but this is not a viable option for patients with Preoperative Assessment and Planning
ischemic heart disease or active infections. With acute Management of anemia
normovolemic hemodilution, units of blood are removed Management of anticoagulation and congenital and
at the beginning of surgery and replaced with crystalloid drug-induced coagulopathies
and colloid so that fewer red blood cells are lost per vol- Prophylactic interventional radiology and embolization
ume of blood lost during surgery. These units of blood Prescribing and scheduling of cell salvage apparatus
are then available for transfusion toward the latter por- Restricted diagnostic phlebotomy
tion of surgery. This strategy intentionally decreases
the oxygen-carrying capacity and reduces the margin Intraoperative Blood Conservation
of safety to prevent cerebral and spinal cord ischemia.
Deliberate hypotension is meant to decrease surgical Meticulous surgical hemostasis
bleeding by intentionally decreasing MAP. However, this Blood salvage
technique may risk both cerebral and spinal cord ischemia Hemodilution
and end-organ damage. Moreover, it is unknown exactly Pharmaceutical enhancement of hemostasis
what minimum MAP is required or is safe, as lower MAPs Maintenance of normothermia
also affect neuromonitoring signals. Surgical positioning to minimize blood loss and hypertension
Advances in neuromonitoring have made posterior
spinal surgery safer, but no single neuromonitoring test is Postoperative Blood Conservation
100% reliable. Somatosensory evoked potentials monitor Blood salvage
the integrity of the dorsal columns, and motor evoked Tolerance of anemia
potentials monitor the integrity of the anterior motor por- Optimum fluid and volume management
tion of the spinal cord. Motor evoked potentials have been Restricted diagnostic phlebotomy
associated with complications such as cognitive defects, sei- Adequate analgesia, maintenance of normothermia
zures, bite injuries, intraoperative awareness, scalp burns, Maintain appropriate fluid resuscitation. Significant normo-
and arrhythmias. volemic anemia is well tolerated in hemodynamically stable
Neuromonitoring is affected by anesthetics, so a com- patients.
monly employed balanced anesthetic technique includes In actively bleeding patients, the first management priority
infusions of propofol and fentanyl with <0.5 MAC of vol- must be to stop the bleeding. Avoid attempts to normalize
atile agent for these surgeries. If neuromonitoring signals blood pressure until bleeding is stopped.
are lost intraoperatively, another way to test the integrity Prevent or treat coagulation disorders promptly.
of the patient’s neurologic circuitry is a wake-up test. For a Oral or parenteral iron may be used to improve iron stores.
wake-up test, the patient’s plane of anesthesia is lightened Exogenous erythropoietin therapy effectively increases red
until the patient can follow simple directions and move blood cell mass.
all extremities to command. Once this was demonstrated,
the plane of anesthesia would then be quickly deepened, Hematology and Oncology
and surgery would continue. This test requires a coop-
erative patient and only evaluates gross motor function. Aggressive exogenous erythropoietin therapy and iron ther-
Potential complications of the wake-up test include extu- apy for the prophylaxis of anemia
bation, dislodgment of invasive lines, venous air embo- Individualized chemotherapy protocols to minimize hema-
lism, dislodgment of surgical instrumentation, patient tologic toxicity
injury from falling off the surgical frame, and intraopera- Pharmacologic prophylaxis and treatment of bleeding
tive awareness. Tolerance of anemia
Idiopathic scoliosis does not automatically place this Restricted diagnostic phlebotomy
patient at an increased risk of malignant hyperthermia. Source: From Miller RD, Eriksson LI, Fleisher L, Wiener-Kronish JP,
Central core disease, King-Denborough syndrome, and eds. Miller’s Anesthesia. 7th ed. Philadelphia, PA:  Churchill Livingstone;
Duchenne and other muscular dystrophies are associated 2010:1791.

  
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r


 K E Y FAC T S
r Blood preservation strategies include preoperative iron
and erythropoietin supplementation, appropriate prone
positioning, intraoperative cell savage, use of colloid
volume expanders, use of antifibrinolytics epsilon
aminocaproic acid (EACA, Amicar) or transexamic acid,
maintenance of normothermia, surgical use of fibrin
glue, and staged surgery or agreeing to stop surgery after
a predefined total blood loss or hematocrit has been
determined.
r Other blood conservation strategies include preoperative
autologous donation, acute normovolemic hemodilution,
and deliberate hypotension.
3 & ' & 3 & /$ & 4
Gertler R, Andropoulos DB. “Cardiopulmonary Bypass and
Management” in Cote CJ, Lerman J, Todres ID, eds. A Practice
of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:402–3.
“Orthopedics:  Scoliosis” in Holzman RS, Mancuso TJ, Sethna NF,
DiNardo JA, eds. Pediatric Anesthesiology Review. New  York,
NY: Springer; 2010:351–5.
Goodnough LT, Monk TG. “Autologous Transfusion, Recombinant
Factor VIIa, and Bloodless Medicine” in Miller RD, Eriksson LI,
Fleisher L, Wiener-Kronish JP, eds. Miller’s Anesthesia. 7th ed.
Philadelphia, PA: Churchill Livingstone; 2010:1487–90, 1791.
3 6 . A N S W E R : B
The neonate introduced in the question stem has DiGeorge
syndrome. If the clinical diagnosis of DiGeorge syndrome
was not immediately made, the fact that this neonate
requires omphalocele repair should prompt a thorough
cardiac evaluation.
Omphalocele occurs in 1/6000 births and is com-
monly associated with congenital heart disease, bladder
extrophy, and Beckwith-Widemann syndrome. The pri-
mary defect with omphalocele is a midline failure of gut
migration from the yolk sac into the abdomen. Large fluid
shifts often occur with omphalocele repairs, and although
ensuring appropriate volume status and urine output are
important, this is not as important as a thorough preopera-
tive cardiac evaluation for this patient with both DiGeorge
syndrome and omphalocele.
DiGeorge syndrome, conotruncal face, and velocardio-
facial syndromes are encompassed by 22q11.2 deletion syn-
drome, which is also known as CATCH 22 syndrome. The
acronym CATCH 22 includes cardiac defects, abnormal
facies, thymic hypoplasia, cleft palate, and hypocalcemia,
and involves deletions of chromosome 22.
DiGeorge syndrome occurs in approximately
1/3000–5000 live births and is the second most common
genetic cause of congenital heart defects. Patients with
DiGeorge syndrome have a variety of congenital heart
 
r
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defects, including interrupted aortic arch, vascular rings,
and hypoplastic left ventricle. The extent and magnitude
of congenital cardiac defects must be elucidated preopera-
tively with cardiac echocardiogram to tailor the neonate’s
appropriate anesthetic technique.
Approximately 60% of DiGeorge patients have charac-
teristic facial abnormalities, including small dysplastic ears,
hypertelorism, short downward-slanted palpebral fissures,
midface hypoplasia, retrognathia, high arched palate, and
bifid uvula. These neonates often present with tetany and
seizures from hypocalcemia. A detailed electrolyte evalua-
tion is helpful to confirm hypocalcemia in this patient who
has seizures and tetany, but it is not the most important pre-
operative evaluation in this patient.
Large fluid shifts are often associated with major neo-
natal abdominal surgeries like omphalocele repair, but the
distractor of potential transfusion of blood products in a
hypocalcemic patient should not lead the reader to incorrectly
choose Answer C instead of Answer B. Similarly, a focused
airway examination is important, as the majority of patients
with DiGeorge syndrome have dysmorphic facies and may
have challenging airways, but the most important preopera-
tive evaluation of this patient is a thorough cardiac evaluation.
Patients with DiGeorge syndrome should be considered
immunocompromised because thymic hypoplasia dimin-
ishes the number and function of their T-cells. Neonates
with DiGeorge syndrome often have recurrent infections
within the first 6 months of life. With this upcoming major
surgery, blood products that are CMV-negative and irradi-
ated should be available to prevent potential graft versus
host disease (GVHD) from blood product transfusion. In
addition, strict aseptic technique should be followed during
vascular access.
K E Y FAC T S
r Omphalocele occurs in 1/6000 births and is commonly
associated with congenital heart disease, bladder
extrophy, and Beckwith-Widemann syndrome.
r DiGeorge syndrome occurs in approximately
1/3000–5000 live births and is the second most
common genetic cause of congenital heart defects.
r Patients with DiGeorge syndrome should be considered
immunocompromised because thymic hypoplasia
diminishes the number and function of their T-cells.
3 & ' & 3 & /$ & 4
“DiGeorge Syndrome” in Bissonnette B, Luginbuehl I, Marciniak
B, Dalens B, eds. Syndromes:  Rapid Recognition and Perioperative
Implications. Online ed. New York, NY: McGraw-Hill; 2006.
Roberts Jr JD, Romanelli TM, Todres ID. “Neonatal Emergencies”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:304, 765.
37. A N S W E R : B
Most children with myelomeningocele present for closure
within the first 48 hours of life to minimize risk of infec-
tion. As with this neonate, many are scheduled for surgical
repair prenatally because the defect is usually detected on
ultrasound. Major anesthetic considerations for this sur-
gery include positioning, potential for massive fluid and
blood loss, and postoperative respiratory difficulties.
With such a large posterior defect, it is important to
ensure that there is no direct pressure on the myelome-
ningocele during the induction of general anesthesia and
while securing the neonatal airway. Depending on the size
of the defect, general anesthesia may be induced with the
area surrounding the patient’s defect well padded in the
supine position, or alternately induction and intubation
may occur in the lateral decubitus position. For very large
defects, it may be safest to induce general anesthesia and
intubate with the patient in the lateral decubitus position,
so as to avoid undue pressure on the lesion.
With myelomeningocele repair, there is the potential
for considerable blood and fluid loss. Because a large por-
tion of the baby’s skin surface area will be exposed for sur-
gery, maintaining adequate volume status and euthermia
are important perioperative considerations, and third space
losses can be considerable.
All of the answer choices are valid concerns for a neo-
nate undergoing myelomeningocele repair and VP shunt
placement, but the most important reason for recovering
this patient in the ICU is to monitor his respiratory status
postoperatively.
Patients with myelomeningocele often have
sleep-disordered breathing, central apnea, hypoventila-
tion, and obstructive sleep apnea. Since this baby is <60
weeks postconceptual age, he is already at an increased risk of
postoperative apnea. Coupled with potential postoperative
respiratory challenges following myelomeningocele repair
and VP shunt placement, it would be advisable to monitor
this patient in an ICU level setting postoperatively, since he
now has multiple reasons to have sleep-disordered breathing.
Hydrocephalus, tethered cord, and type II
Arnold-Chiari malformations often accompany lesions
like myelomeningocele. Moreover, if this neonate also has a
type II Arnold-Chiari malformation that contributes to his
hydrocephalus and need for a VP shunt, then his ventila-
tory response to hypoxia and hypercarbia may be dimin-
ished or absent postoperatively. In addition, depending on
the degree of hydrocephalus, this neonate may have the
potential for brainstem herniation and may also have a
cranial nerve palsy that would result in inspiratory stridor.
This patient thus has several reasons to have his respiratory
status closely monitored in the ICU setting postoperatively.
Patients with myelodysplasias like myelomeningocele
are at an increased risk of developing latex sensitiv-
ity because of repeated exposures to latex, such as occurs
  
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r
 
with repeated bladder catheterization. Strict adherence to
latex-free environments must be followed.
K E Y FAC T S
r Major anesthetic considerations for repair of
myelomeningoceles include positioning, potential
for massive fluid and blood loss, and postoperative
respiratory difficulties.
r For very large defects, it may be safest to induce general
anesthesia and intubate with the patient in the lateral
decubitus position, so as to avoid undue pressure on the
lesion.
r Hydrocephalus, tethered cord, and type II Arnold-
Chiari malformations often accompany lesions like
myelomeningocele, and ventilatory responses to
hypoxia and hypercarbia may be diminished or absent
postoperatively.
3 & ' & 3 & /$ & 4
McClain CD, Soriano SG, Rockoff MA. “Pediatric Neurosurgical
Anesthesia” in Cote CJ, Lerman J, Todres ID, eds. A Practice
of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders Elsevier; 2009:530–2.
“Neuroanesthesia” in Holzman RS, Mancuso TJ, Sethna NF, DiNardo
JA, eds. Pediatric Anesthesiology Review. New York, NY: Springer;
2010:182–3.
Coté CJ. “Pediatric Anesthesia” in Miller RD, Eriksson LI, Fleisher L,
Wiener-Kronish JP, eds. Miller’s Anesthesia. 7th ed. Philadelphia,
PA: Churchill Livingstone; 2010:2589.
3 8 . A N S W E R : A
This question highlights why a single-shot spinal is consid-
ered a preferred anesthetic technique for a former preterm
infant. By using a complete regional technique, one can
potentially avoid administering narcotics and a general
anesthetic, both of which may contribute to postoperative
apnea in this former premature twin whose postconcep-
tual age is <60 weeks. Unfortunately, it appears that his
single-shot spinal has worn off.
Answer A  would prevent postoperative apnea in this
patient. It should be clearly communicated to the surgeon
what dose of local anesthetic is safe and appropriate for this
4  kg infant, as a small volume of local anesthetic quickly
becomes a toxic dose for small infants receiving bupiva-
caine. Since the infant has already received 0.4 cc of 0.5%
bupivacaine in the subarachnoid space, the surgeon should
be limited to 3.2 cc of 0.25% bupivacaine for infiltration.
If no narcotics are administered, the patient will be at no
increased risk of postoperative apnea with this strategy.
Answer B does not prevent postoperative apnea. It
makes no difference what narcotic is administered. Any
administration of narcotic has been associated with
postoperative apnea in former preterm infants with a
postconceptual age <60 weeks.
 Answer C does not reliably prevent postoperative apnea
either. Even if no narcotic medication is administered, for-
mer preterm infants with a postconceptual age <60 weeks
undergoing general anesthesia are at increased risk of
postoperative apnea.
Answer D is attractive since the N-methyl-D-aspartate
(NMDA) antagonist ketamine is often used to maintain
spontaneous respirations while also providing analgesia.
However, when ketamine is used to supplement neonatal
spinal anesthesia, the incidence of postoperative apnea is
actually greater than with a general anesthetic.
Spinal anesthesia is an attractive option for this patient
and surgery because of its association with less postopera-
tive apnea, and timing of surgery meshes well with unilat-
eral inguinal hernia repair. There is a lower risk of total
spinal in neonates, but there are several technical consid-
erations when performing a neonatal spinal. Among these
considerations is the appropriate positioning and bracing
of the baby while the spinal is being performed. Excessive
neck flexion should be avoided, as this may obstruct
the patient’s airway. Since the conus medullaris is located
at L3 in neonates and infants, spinal anesthesia should be
performed at the L4/5 or L5/S1 level. When the subarach-
noid space has been entered, the local anesthetic should be
administered slowly with a tuberculin syringe.
Any repositioning of the infant after placement of the
spinal should be performed carefully. In particular, any
Trendelenburg position should be avoided, as sometimes
occurs when a neonate’s legs are lifted to place an electro-
cautery pad on the patient’s back. This seemingly innocu-
ous maneuver could potentially cause cephalad spread of
the local anesthetic and a resultant high spinal.
K E Y FAC T S
r By using a complete regional technique, one can
potentially avoid administering narcotics and a
general anesthetic, both of which may contribute to
postoperative apnea in this former premature twin
whose postconceptual age is <60 weeks.
r Any administration of narcotic has been associated with
postoperative apnea in former preterm infants with a
postconceptual age <60 weeks.
r Former preterm infants with a postconceptual age <60
weeks undergoing general anesthesia are at increased risk
of postoperative apnea.
r When ketamine is used to supplement neonatal spinal
anesthesia, the incidence of postoperative apnea is
actually greater than with a general anesthetic.
3 & ' & 3 & /$ & 4
Everett LL, Fuzaylov G, Todres ID. “Pediatric Emergencies” in Cote
CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for Infants
 
r
$ - * / * $ " -
4 $ * & / $ &
0 ' ु " / & 4 5 ) & 4 * "
and Children. 4th ed. Philadelphia, PA:  Saunders Elsevier;
2009:877–9.
Welborn LG, Rice LJ, Hannallah RS, Broadman LM, Ruttimann UE,
Fink R. Postoperative apnea in former preterm infants:  prospec-
tive comparison of spinal and general anesthesia. Anesthesiology.
1990;72:838–42.
39. A N S W E R : B
Pyloric stenosis is a medical emergency, not a surgical emer-
gency. It is common in firstborn males within the first 3
to 6 weeks of life. Perioperative concerns for this patient
include full stomach precautions and severe dehydration.
Volume status for patients with pyloric stenosis coming to
the operating room must be adequate, and metabolic alka-
losis from repeated vomiting should be corrected with
vigorous fluid resuscitation prior to surgical correction.
Answer B best indicates that the patient is ready for
surgery, as his electrolyte imbalances have been corrected.
Specifically, his hypokalemia, hypochloremia, and
increased bicarbonate level from his contraction alkalo-
sis have been appropriately corrected with fluid resuscita-
tion. Other signs of adequate volume status in this patient
include appropriate urine output (wet diapers), normal
behavior (not irritable or lethargic), normal skin turgor,
tears if the child is crying, and anterior fontanelles that are
not depressed.
Answer A is incorrect because the patient’s potassium,
chloride, and bicarbonate levels have not yet been cor-
rected. These laboratory values are consistent with meta-
bolic alkalosis and likely are the values that this infant
with pyloric stenosis initially presented with. Neither
Answer C nor D is correct because the bicarbonate levels
have not been appropriately corrected with adequate fluid
resuscitation.
Patients with pyloric stenosis have a full stomach that
may contain radiocontrast dye from confirmatory imag-
ing studies. It is advisable to suction the stomach with a
large-bore catheter with the patient in the supine, right,
and left lateral decubitus positions. One study found that
this technique removes 98% of gastric contents. After
the stomach has been suctioned, options for intubation
include awake or rapid sequence intubation. Either
technique is reasonable, but a study comparing awake
versus rapid sequence intubation demonstrated increased
success with first pass intubation when muscle relaxant
was used.
Postoperative analgesia for laparoscopic or open pyloro-
myotomy typically consists of local anesthetic infiltration
and acetaminophen. Narcotics are not routinely required
for postoperative analgesia for this surgery.
K E Y FAC T S
r Perioperative concerns for patients with pyloric stenosis
include full stomach precautions and severe dehydration.
r Metabolic alkalosis (hypokalemia, hypochloremia, and
increased bicarbonate) from repeated vomiting should
be corrected with vigorous fluid resuscitation prior to
surgical correction.
r Signs of adequate volume status in this patient include
appropriate urine output (wet diapers), normal behavior
(not irritable or lethargic), normal skin turgor, tears if
the child is crying, and anterior fontanelles that are not
depressed.
3 & ' & 3 & /$ & 4
Cook-Sather SD, Liacouras CA, Previte JP, Markakis DA, Schreiner
MS. Gastric fluid measurement by blind aspiration in pae-
diatric patients:  a gastroscopic evaluation. Can J Anaesth.
1997;44:168–72.
Roberts Jr JD, Romanelli TM, Todres ID. “Neonatal Emergencies”
in Cote CJ, Lerman J, Todres ID, eds. A Practice of Anesthesia for
Infants and Children. 4th ed. Philadelphia, PA: Saunders Elsevier;
2009:761–2.
Coté CJ. “Pediatric Anesthesia” in Miller RD, Eriksson LI, Fleisher L,
Wiener-Kronish JP, eds. Miller’s Anesthesia. 7th ed. Philadelphia,
PA: Churchill Livingstone; 2010:2591.
4 0 . A N S W E R : D
Pediatric anesthesiologists must be able to anticipate and
recognize signs and symptoms of active upper respiratory
infections. This former preterm child has several symptoms
of an active URI. Since this surgery is elective, it should be
postponed at least 4 weeks until symptoms of this active
URI have resolved. Moreover, since this child has not
received any vaccinations, he may also have a more serious
illness like varicella or measles, and this should be investi-
gated further.
Other predictors for an acute URI include parental
reports of the child having a “cold,” nasal congestion, snor-
ing, exposure to secondhand smoking, and parental reports
that the child appears ill, which indicate changes in mental
status or behavior.
Having an active URI increases this child’s risks of
perioperative airway complications such as broncho-
spasm, laryngospasm, and major desaturation episodes
up to 10-fold. Furthermore, since he is a former preterm
infant, his risks of respiratory complications are increased.
These risks should be highlighted and discussed with the
child’s parents in the preoperative area, as cancellation of
surgery may impose emotional and economic burdens on
the parents with the challenges of rescheduling elective
surgery.
Answer A is incorrect because this patient has an active
URI and has other risk factors that increase his likelihood
of having perioperative respiratory complications, even if a
laryngeal mask airway is used instead of tracheal intubation.
Bronchospasm has been found to occur more frequently
in patients with active URIs who are intubated. Overall
  
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incidence of laryngospasm in patients with active URIs is
similar independent of endotracheal tube versus LMA use.
LMA use may decrease this child’s propensity for broncho-
spasm, but it will not decrease the chance of laryngospasm
in this patient with an active URI.
Answer B is incorrect. A  study found that healthy
children with URIs undergoing surgery not involving the
airway or body cavity did not benefit from preoperative
bronchodilator administration of either albuterol or
ipratropium.
Answer C is incorrect. There are no data to support the
routine use of steroids and broad-spectrum antibiotics in
children with active URIs.
Unless this surgery is urgent or emergent, it should be
postponed. Active URIs in children scheduled for cardiac
surgery warrant special consideration, as there are risks that
the child’s heart or clinical disease process will deteriorate
while awaiting the child to recover from an active URI. The
child scheduled for cardiac surgery should be optimized
preoperatively, and a thorough discussion of the periopera-
tive risks of an active URI with general anesthesia should be
explained to the child’s parents.
An active URI was determined to be an independent
risk factor for postoperative infections and other post-
operative complications in children undergoing cardiac
surgery. In a prospective study of children scheduled for
cardiac surgery, overall incidence of respiratory adverse
events, multiple postoperative complications, and bacte-
rial infection were significantly higher in children with
active URIs. Postoperative ICU duration was higher
in the URI group, but duration of hospital stay was not
significantly increased among children with or without
active URIs.
There are rare instances when postponing surgery is
not an option, such as occurs with solid organ transplant,
and the risks should be explained and documented, and
vigilance should be employed to watch for potential
respiratory complications. Recognizing URI signs and
symptoms and appropriately balancing risks and ben-
efits for each patient are fundamental in the clinical
acumen of pediatric anesthesiologists (see Box 20.2 and
Table 20.5).
3 & ' & 3 & /$ & 4
Ghazal EA, Mason LJ, Coté CJ. “Preoperative Evaluation,
Premedication, and Induction of Anesthesia” in Cote CJ, Lerman J,
Todres ID, eds. A Practice of Anesthesia for Infants and Children. 4th
ed. Philadelphia, PA: Saunders Elsevier; 2009:60–2.
Malviya S, Voepel-Lewis T, Siewert M, Pandit UA, Riegger LQ, Tait
AR. Risk factors for adverse postoperative outcomes in children pre-
senting for cardiac surgery with upper respiratory tract infections.
Anesthesiology. 2003;98:628–32.
Rolf N, Coté CJ. Frequency and severity of desaturation events during
general anesthesia in children with and without upper respiratory
infections. J Clin Anesth. 1992;4:200–3.
 Box 20.2 %*''&3 &/5*"-
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peripheral perfusion. The accumulation of sickled eryth-
$)*-%
8*5)ु"
36//:ु/04& rocytes causes ischemia, further sickling, and eventually
end-organ failure.
Noninfectious Causes Many organ systems are affected by sickle cell disease,
and patients with this disease are predisposed to acute chest
Allergic rhinitis:  seasonal, perennial, clear nasal discharge,
syndrome (ACS), recurrent episodes of vaso-occlusive cri-
no fever
ses, infection, renal insufficiency, osteonecrosis, and chole-
Vasomotor rhinitis: emotional (crying), temperature
lithiasis. Chronic pulmonary and neurologic diseases like
changes
stroke are causes of significant morbidity and mortality in
this patient population.
Infectious Causes
In the perioperative period, the most common compli-
Viral infections cations in children with SCD include ACS, fever, infec-
Nasopharyngitis (common cold) tion, vaso-occlusive crisis, and transfusion-related
Flu syndrome (upper and lower respiratory tract) events. Because this patient is predisposed to ACS and
Laryngotracheal bronchitis (infectious croup) vaso-occlusive crisis, liberal analgesics should be adminis-
Viral exanthems tered, and regional techniques should be considered.
Measles Similarly, hypoxia should be avoided by ensuring
Chickenpox adequate oxygenation and oxygen-carrying capacity of
Acute bacterial infections hemoglobin, usually on the order of 10 g/dL. Transfusion
Acute epiglottitis of blood corrects anemia, dilutes the concentration of HbS
Meningitis erythrocytes, and can prevent severe complications like
Streptococcal tonsillitis stroke. Since sickle cell patients often receive multiple trans-
fusions of blood products, it is important that the blood
they receive is phenotype matched, leukocyte reduced,
Table 20.5 '"$5034
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and screened for sickle cells, as these patients have a high
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rate and risk of alloimmunization.
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Aside from hypoxia, acidosis and circulatory stasis also
contribute to sickling in susceptible individuals. Acid-base
PRO CON status should be closely monitored, and circulatory status
$IJME
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OP
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can be optimized by providing appropriate fluid resuscita-
PUIFS
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tion and avoiding excessive or prolonged tourniquet use.
DPVHI

QPPS
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KVTU
Because patients with SCD are already at an increased
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risk of developing stroke, hyperventilating to an end-tidal
OJHIU
CO2 < 25  mm Hg is not advised, as that would further
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compromise already tenuous cerebral blood flow.
$MFBS
SIJOPSSIFB 1VSVMFOU
OBTBM
EJTDIBSHF Older patients with SCD like this patient may develop
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SBMFT restrictive lung disease and pulmonary hypertension.
0MEFS
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FYQSFFNJF This results from repeated ACS-like injury, chronic inflam-
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mation, and deficiency of nitric oxide. In addition, chronic
UP
CF
BXBZ
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XPSL

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NBKPS
intravascular hemolysis decreases the production of NO,
XJMM
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PVU PQFSBUJPO

FOEPUSBDIFBM
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and increased scavenging of NO reduces its bioavailability.
SFRVJSFE Endothelial dysfunction and NO deficiency cause com-
SOURCE: Reprinted with permission from: Cote CJ, Lerman J, Todres ID, plications like pulmonary hypertension, priapism, and skin
eds. A Practice of Anesthesia for Infants and Children. 4th ed. Philadelphia,
PA: Saunders; 2009: 61.
ulceration. Studies have shown that inhaled NO amelio-
rates the symptoms of ACS, so inhaled nitric oxide would
not be deleterious for this patient and may potentially
41. A N S W E R : C improve ventilation (see Box 20.3).
Sickle cell disease (SCD) encompasses a group of inher-
ited hemoglobinopathies, with HbSC (sickle cell/HbC)
and HbSD (sickle cell/HbD) having the greatest potential 3 & ' & 3 & /$ & 4
for significant clinical sickling. This sickling occurs when
Haberkern CM, Webel NE, Eisses MJ, Bender MA. “Essentials of
sickled erythrocytes accumulate in the microvascular of Hematology” in Cote CJ, Lerman J, Todres ID, eds. A Practice
susceptible patients in the setting of factors that promote of Anesthesia for Infants and Children. 4th ed. Philadelphia,
the deoxygenation of hemoglobin and interferes with PA: Saunders Elsevier; 2009:182–6.

 
r
$-* / *$ " -
4$* & /$&
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Preoperative
Screening if unknown status in at-risk children
Admission to hematology service (under most circumstances)
History of acute chest syndrome, vaso-occlusive pain crises,
hospitalizations, transfusions, transfusion reactions
Neurologic assessment (strokes, cognitive limitations)
History of analgesic and other medication use
Hematocrit
Oxygen saturation (room air), chest radiograph
Pulmonary function tests (when appropriate)
Echocardiography (when appropriate)
Neurologic imaging (when there are recent changes)
Renal function studies
Transfusion crossmatch (antibody-matched, leukocyte
reduced, sickle negative)
Transfusion to correct anemia (under most circumstances)
Parenteral hydration while NPO
Pain management
Aggressive bronchodilator therapy
Appropriate antibiotic therapy, including pre-splenectomy
antibiotics and immunizations, where indicated
Intraoperative
Maintenance of oxygenation, perfusion, normal acid-basis
status, temperature, hydration
Availability of appropriately prepared blood (when
appropriate)
Replacement of blood loss
Anesthetic technique appropriate for procedure and postop-
erative analgesic requirements
Physiologic effects of laparoscopy on circulatory and respira-
tory function
Appropriate antibiotic therapy
Judicious use of tourniquets, cell saver, and cardiopulmonary
bypass
Postoperative
Management by hematology service
Monitoring for complications, especially acute chest syn-
drome and vaso-occlusive pain crises
Maintenance of oxygen saturation monitoring and supple-
mentation as needed, including supplemental oxygen the
first 24 hours regardless of oxygen saturation
Appropriate hydration (oral + parenteral)
Appropriate antibiotic therapy
Aggressive pain management
Early mobilization
  
& 7" - 6"5 * 0 /
0 ' ु $ 0 & 9 * 4 5 * / (
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r
 
Fischer SP, Bader AM, Sweitzer B. “Preoperative Evaluation” in Miller
RD, Eriksson LI, Fleisher L, Wiener-Kronish JP, eds. Miller’s
Anesthesia. 7th ed. Philadelphia, PA:  Churchill Livingstone;
2010:1028.
4 2 . A N S W E R : C
Beckwith-Wiedemann syndrome usually occurs spo-
radically and consists of exomphalos, macroglossia,
gigantism, organomegaly, and hypoglycemia. It has
an incidence of approximately 1.5 per 100,000 with an
increased incidence in West India, and occurs as a result
of failure of normal biparental inheritance of chromo-
some 11p15, which contains growth-promoting and
tumor-suppressor genes, resulting in increased insulin-like
growth factor 2 (IGF-2). This increased IGF-2 is responsi-
ble for somatic overgrowth, the predisposition for tumors,
and hypoglycemia.
As with this patient, early visceromegaly occurs, and
neonates with Beckwith-Wiedemann syndrome often pres-
ent for repair of omphalocele, anomalies of intestinal rota-
tion, and diaphragmatic eventration. Sometimes patients
with Beckwith-Wiedemann syndrome have midface hypo-
plasia, so a full preoperative airway evaluation is impor-
tant, as securing this patient’s airway may be challenging. It
would be prudent to have a difficult airway cart and vari-
ous LMAs available for instrumenting this child’s airway.
Several organ systems may be involved with
Beckwith-Wiedemann syndrome. Cardiomegaly, hepa-
tosplenomegaly, visceromegaly, and several malignant
tumors have been associated with Beckwith-Wiedemann
syndrome. When patients with Beckwith-Wiedemann
syndrome have hemihypertrophy, an increased incidence
of malignant tumors like adrenal carcinoma, Wilms
tumor, hepatoblastoma, thoracic neuroblastoma, rhab-
domyosarcoma, and congenital gastric teratoma has been
reported. Proper preoperative workup of this patient with
Beckwith-Wiedemann syndrome and hemihypertrophy
thus includes screens for somatic tumors and a full car-
diac workup.
Answer C is correct, as a cardiac echocardiogram will
evaluate for cardiomegaly, cardiomyopathy, congenital
cardiac lesions, or cardiac hamartoma. Data from this
study will more strongly influence decisions regarding this
patient’s anesthetic and choice of intraoperative medica-
tions. Inotropes and bacterial endocarditis prophylaxis may
be indicated for this patient, depending on what her cardiac
echocardiogram shows.
Answer A  is incorrect. Although it would be nice to
know the patient’s fasting glucose level, this data will not
significantly alter the patient’s anesthetic, as this data
point is not absolutely required to maintain intraoperative
euglycemia.
Similarly, Answer B is incorrect. Beckwith-Wiedemann
syndrome is associated with hepatosplenomegaly, but
knowing the patient’s LFTs will not significantly alter her
anesthetic plan. If a neuraxial block were planned for post-
operative analgesia, then coagulation studies and a platelet
count could alter the anesthetic plan. However, this data
will not change the decision regarding the planned femoral
peripheral nerve block catheter.
Answer D is a distractor, as Beckwith-Wiedemann syn-
drome has not been associated with susceptibility to malig-
nant hyperthermia.
K E Y FAC T S
r Beckwith-Wiedemann syndrome usually occurs
sporadically and consists of exomphalos, macroglossia,
gigantism, organomegaly, and hypoglycemia.
r Beckwith-Wiedemann syndrome is associated with
midface hypoplasia, and airway management can be
difficult.
  
r
$ - * / * $ " -
4 $ * & / $ &
0 ' ु " / & 4 5 ) & 4 * "
r Proper preoperative workup of this patient with
Beckwith-Wiedemann syndrome and hemihypertrophy
thus includes screens for somatic tumors and a full
cardiac workup.
3 & ' & 3 & /$ & 4
“Beckwith-Wiedemann Syndrome” in Bissonnette B, Luginbuehl I,
Marciniak B, Dalens BJ. eds.Syndromes:  Rapid Recognition and
Perioperative Implications. New  York, NY:  McGraw-Hill; 2006.
http://accessanesthesiology.mhmedical.com/content.aspx?bookid=
852&Sectionid=49517273. Accessed July 11, 2014.
Wheeler M, Coté CJ, Todres ID. “The Pediatric Airway.” Cote CJ,
Lerman J, Todres ID. A Practice of Anesthesia for Infants and
Children. 4th ed. Philadelphia, PA: Saunders Elsevier; 2009:274.