1

ANESTHETIC CONSIDERATIONS IN THE PATIENT

WITH
DOWN SYNDROME

Melissa A. Ehlers, M.D.

Assistant Professor of Anesthesiology
Director of Pediatric Anesthesia
Albany Medical Center
Albany, NY

Address correspondence to:

Melissa A. Ehlers, M.D.

47 New Scotland Ave.
Albany Medical Center
PO Box 131
Albany, NY 12208
(518) 262-4305
(518) 262-4736 (fax)
melissa_ehlers@yahoo.com
 2

Learning Objectives

 Appreciate commonly found disease states in the population with Down syndrome (DS)
 Understand how to quickly evaluate these common diseases
 Learn how to evaluate a potentially unstable cervical spine
 Be aware of the significantly increased mortality from pulmonary infection in the
patient with DS
 Recognize common forms of congenital heart disease (CHD) in this population
 Understand some of the potential complications in the patient with CHD presenting
for non-cardiac surgery
 Be aware of differences in determination of tracheal tube size
 Decide whether endocarditis prophylaxis is necessary
 Evaluate the best method of post-operative pain control
 3

Anesthetic care of the patient with Down syndrome (also known as Trisomy 21) can

present a challenge to even the most seasoned practitioner. These patients may have a

wide range of serious medical problems in combination with varied ability to realize that

they are sick. Unfortunately, they rarely understand why the anesthesia caregivers must

poke and prod them in an effort to “make them better.” In order to provide the best

possible care for them the caregiver must understand the various physical and emotional

characteristics of the patient with DS and be prepared for the special challenges that they

may present.

Pre-operative considerations

Frequently the preoperative screen may be the most challenging part of the entire

anesthetic. The intelligence quotient (IQ) in individuals with DS ranges from 20 to 70,1

therefore commonly little or no detailed information may be obtained from the patient.

Instead, a parent, sibling, other relative or caretaker must frequently be relied upon to

furnish the medical history. Because this may be incomplete or abbreviated, the

anesthesia provider must keep the common disease states associated with DS in mind and

attempt to rule out or further explore as many as possible (see Table 1).
 4

Mental Retardation

The IQ usually ranges from 20 to 70 in these patients and cooperation can be difficult to

obtain. This is further compounded by a 30% incidence of mental illness in the DS

population2 that can make communication even more difficult. For those patients able to

understand, a calm, quiet explanation of the anesthetic plan can be reassuring; appropriate

use of oral or intravenous sedative premedication may make the experience much more

pleasant for everyone involved. With the extremely agitated, uncooperative patient, one

must occasionally resort to intramuscular or rectal sedatives in order to sedate him/her to

the point where the rest of the anesthetic plan can occur smoothly and safely.

Hypo-/hyper- thyroidism

Hypothyroidism may be seen in as many as 10 to 40% of patients with DS, and this

population also has a higher-than-average prevalence of hyperthyroidism.2 Many patients

will have routine screening tests for this condition, but a well-directed history may

elucidate symptoms (i.e., constipation, recent weight gain, sluggishness vs. diarrhea, heat

intolerance, palpitations) that warrant further workup.

 5

Diabetes Mellitus

The prevalence of type 1 diabetes mellitus is as high as 10.6% in patients with DS.3

These patients are often treated with once daily insulin regimens that are surprisingly

efficacious when compared to diabetics without DS who are matched for other variables

(i.e., duration of diabetes, sex, age). If screening for diabetes has not been done recently

in the patient, a history of prolonged flu-like illness, polyuria, polyphagia, and/or weight

loss should raise the suspicion of diabetes.

Obesity

Although infants with DS typically follow height and weight curves lower than those of

the rest of the population, by the age of 2 to 3 years they commonly start to exhibit

excessive weight gain.4 Upon reaching adulthood, as many as 50% of individuals with

DS may be overweight in spite of evidence that their caloric intake may even be reduced.5

Obesity in these patients may contribute to worsening of pre-existing conditions (i.e.,

diabetes, sleep apnea, pulmonary hypertension) or may cause new health conditions

unrelated to the presence of DS, such as hypertension, arthritis, and gallbladder disease.

Additionally, practitioners should be cognizant of the airway, ventilatory, and positioning

challenges posed by the overweight patient.

 6

Sleep Apnea

Obstructive sleep apnea (OSA) has a reported prevalence of 54% to 100% in the

population with DS compared with only 2% in the rest of the population.6 Typical

characteristics of the DS patient, which include midface hypoplasia, micrognathia, small

larynx, obesity and muscular hypotonia, all predispose them to potentially significant

OSA. Besides these potential causes of mechanical obstruction, there is evidence that

some individuals with DS may have centrally mediated sleep apnea, perhaps secondary to

an abnormal respiratory drive with hypoxic stimulation (Ondine’s curse).7 If

adenotonsillar hypertrophy is present, their removal may improve symptoms, but quite

often these patients have relative hypoplasia of these structures and other surgical

alternatives would include uvulopalatopharyngoplasty and/or possible partial tongue-base

resection or genioglossal advancement. Nonsurgical therapy may include continuous

positive airway pressure (CPAP), weight loss programs, and occasionally, medications to

stimulate respiratory drive.6 The consequences of undiagnosed/untreated OSA may

include “minor” symptoms such as daytime sleepiness or behavioral difficulties, but

major problems such as cor pulmonale (especially in this population which is more

susceptible to pulmonary hypertension) or arrhythmias may develop. Obviously, because

of the high prevalence in this population, one should assume the DS patient has OSA and

try to rule it out by a history of surgical or non-surgical treatment or a negative

polysomnography test. Otherwise, anesthetic preparation should include the assumption

that some degree of obstruction may be encountered during most anesthetics, and

pulmonary hypertension may also be present.

 7

Otitis Media/ Hearing Loss

Patients with DS are predisposed to frequent middle ear infections and/or effusions

because of abnormal eustachian tube architecture (including hypotonicity of the

musculature, a decreased diameter, and abnormal cartilage). Stasis within the eustachian

tube permits bacteria to migrate from the nasopharynx.7 Recurrent infections along with

an increased incidence of inner ear abnormalities (progressive ossification of the basal

spiral tract, shortened organ of Corti, decreased spiral ganglion cells) all contribute to the

increased incidence of hearing loss in individuals with DS which may be as high as 78%.8

Whether presenting for middle/inner ear surgery or some other procedure, these patients

may find communication even more difficult because of their hearing loss. Also, they

may be more prone to post-operative nausea and vomiting if their vestibular pathways

have been affected.

Gastrointestinal Disorders

Newborns with DS have up to a 12% prevalence of various gastrointestinal (GI)

atresias,1 which usually necessitate surgery within the first few days of life. Other

frequent GI abnormalities include pyloric stenosis, imperforate anus, tracheo-esophageal

fistula, and Hirschsprung’s Disease. In addition, patients with DS are prone to develop

celiac disease (occurs in 7 to 16%9) that requires lifelong adherence to a special diet in

order to prevent small bowel mucosal damage.

 8

Hematologic Abnormalities

Children with DS are known to have a 10-to 20-fold increase in their risk of developing

acute leukemia,10 although it is still only found in less than 1% of individuals with DS.1

With chemotherapy, the long-term prognosis for acute lymphocytic leukemia (ALL) is

similar to that for non-DS patients, but interestingly, patients with DS have higher

survival rates for the various myeloid leukemias. This has led some authors to speculate

that there may be increased expression of a gene on chromosome 21 that allows improved

chemosensitivity of the dysplastic cells.11 In contrast to the increased risk of ALL, the

risk of developing most solid tumors is lower in the DS population, and breast cancer is

rarely seen.

Approximately 10% of newborns with DS will exhibit transient abnormal myelopoiesis

(TAM) that almost always spontaneously resolves within 3 months.12 Characteristics of

this condition include megakaryocytes in peripheral blood, variable thrombocytopenia,

and rare progression to hydrops fetalis or hepatic fibrosis.13 This disorder is considered to

be indistinguishable from acute megakaryocytic leukemia (AML-M7), and only its

spontaneous regression points to the diagnosis of TAM. Of these patients, approximately

25% will go on to develop AML in the first four years of life, which is fatal if left

untreated.12

Other common hematologic anomalies of the newborn with DS are thrombocytopenia

and thrombocytosis.14 Both appear to spontaneously regress, but severe or symptomatic

thrombocytopenia may need to be treated with transfusion.

 9

Atlanto-axial Instability

Up to 20 % of children with DS have radiographic evidence of atlantoaxial instability

(AAI), but only approximately 1% of all patients with DS will exhibit any symptoms15

(see Table 2).

Pre-operative evaluation of the individual with DS for AAI remains controversial. All

patients should have annual neurologic exams and certainly a patient who exhibits any of

the above symptoms should have neck radiographs taken. Lateral views in extension,

neutral position, and flexion are termed a “Down series”16. A distance 4.5 mm or greater

between the anterior arch of the atlas and odontoid process of the axis is regarded as

potentially unstable.17 The asymptomatic child with DS without previous screening

presenting for tonsillectomy is a common workup dilemma for many practitioners. If the

patient has competed in Special Olympics, screening for AAI is required before

participation and any evidence of AAI should have been communicated to the caregivers.

Otherwise, a brief inquiry as to any evidence of ligamentous laxity or instability

elsewhere (i.e. frequent patellar dislocation, ability to fold thumb back to touch wrist)

should alert one to a greater likelihood that some degree of AAI is present. Regardless,

without overt neurological symptoms, it does not seem reasonable to recommend

radiographs in these asymptomatic patients as long as some standard precautions are

taken, such as minimal manipulation of the head for intubation (ideally one has an

assistant stabilize the head/neck in the neutral position) as well as avoidance of further

surgical manipulation of the head and neck whenever possible. Although many people

assume that extension of the head is most compromising to the spinal cord, lateral or
 10

axial rotation can also cause damage18 and this should be kept in mind when placing

internal jugular venous lines. For those patients who already have diagnosed AAI,

consideration should be given to placing a hard or soft collar perioperatively to protect

the cervical spine.

Recurrent Pulmonary Infections

Numerous studies have found the lungs of patients with DS to be abnormal; grossly they

exhibit a sponge-like appearance due to enlargement of alveoli and terminal bronchioles

that is accompanied by a reduction in the overall number of alveoli.19 Although some

investigators feel that these abnormalities are present antenatally,20 others have found that

these differences only start to occur after birth.21 This has led to the hypothesis that

intrauterine pulmonary development may be mediated by maternal-placental factors,

while post-natal development is mainly genetic in nature.22 Another unusual feature in

these patients is the presence of a double capillary network around the alveoli, a normal

feature of all human fetuses, but abnormal in its persistence into adult life.21

Another abnormality in patients with DS is their state of relative immunodeficiency due

to decreases in both cell and antibody-mediated immunity.23 All of the above factors,

combined with reduced mucociliary clearance and the presence of heart disease in many

patients (which can increase interstitial fluid in the lungs, cause compression of the left-

mainstem bronchus, etc.) all contribute to a mortality from respiratory infection that is

124 times greater than that of the non-DS population.22 For this reason, the presence of

any pulmonary infection in a patient with DS presenting for surgery should be treated
 11

with careful consideration, and the threshold for postponement of an elective case may be

much lower than usual.

Congenital Heart Disease

Cardiac defects are recognized in 40-50% of the population with DS while alive, and in

up to 66% of these patients at autopsy. Additionally, subtle abnormalities of morphology

or cardiovascular function can be seen in almost everyone with DS.24 The most common

type of congenital heart disease (CHD) in this population is complete atrioventricular

septal defect (cAVSD) where the lower part of the atrial septum and the upper part of the

ventricular septum are missing, and there is a common atrioventricular valve that must be

divided into the tricuspid and mitral valves. CAVSD has a prevalence of approximately

50-60% of all CHD cases with DS.25 Ventricular septal defects (VSD) account for about

12%, and Tetralogy of Fallot (TOF) is the third most common being found in

approximately 8% of cases. Interestingly, Chinese and Mexican DS patients are more

likely to have a VSD than cAVSD suggesting that trisomy of the 21st chromosome does

not by itself account for the constellation of heart diseases seen, and instead, there are

other genetic and environmental factors that must be important as well.26 Because the

prevalence of CHD is so high in this population, almost all infants with DS will be

evaluated at birth, preferably by a pediatric cardiologist, for the presence of heart disease.

Since most forms of CHD in the population with DS involve a large left-to-right shunt

(especially with CAVSD and VSDs), signs of pulmonary overcirculation develop quite

quickly. For this reason, these patients tend to have repair fairly early (i.e., within the
 12

first year of life) since morphologic differences in their lung parenchyma make them

much more susceptible to the development of irreversible pulmonary vascular occlusive

disease.27 Even the adult patient with DS and no history of CHD is at greater risk of

acquired heart disease; mitral valve prolapse is found in 50 – 60% of these patients while

valvular incompetence is seen in 17%.2 It is of interest that coronary artery disease is

much less prevalent in individuals with DS than the rest of the population. Recently, a

group from the University of Vermont demonstrated that adult patients with DS have

reduced plasminogen activator type-1 in their blood compared with control subjects, and

they theorize that this may explain the decreased incidence of coronary plaque formation

and rupture in this population.28-

Intraoperative Considerations

In 1957, McKusick29 was the first person to report a hypersensitivity to atropine in the

population with DS and several other authors reported similar findings. Based on these

papers, pediatric manuals for many years warned of the potentially fatal effect of atropine

use in these patients. Starting in 1973, however, other authors30 have shown that there is

no difference in the cardio-acceleratory effects of atropine in the patient with DS

compared to the rest of the population. Interestingly, ophthalmic instillation of atropine

drops does cause a more rapid mydriasis in the patient with DS but the duration and

degree of papillary dilation are the same as in the patient without DS.
 13

If the anesthetic plan includes tracheal intubation, one special consideration in the patient

with DS is the appropriate size of tracheal tube (TT). This population has a higher

incidence of congenital narrowing of the subglottis, and they are also more likely to

develop significant subglottic stenosis following tracheal intubation.6 In a study

performed at the Children’s Hospital Medical Center in Cincinnati, Ohio, Shott31

determined the proper size of TT in 42 children with DS compared to 32 control subjects,

using an audible air leak around the TT between 10 and 30 cm of H2O pressure as the

guide. In addition, the author assessed the tracheal diameter found on MRI of another

18 patients with DS. Shott’s conclusions led her to recommend that for the patient with

DS, the initial intubation should be performed with a TT that is two half-sizes smaller

than that normally used as determined by the standard formula TT size = 4 + age/4.

Since numerous other authors have found very high rates of post-extubation croup (Kobel

et al32) or stridor (Sherry33) in the population with DS, it would seem prudent to follow

these recommendations.

The patient with DS and uncorrected CHD presenting for non-cardiac surgery may be

intimidating for many anesthesiologists. Keeping a few simple things in mind, however,

can enable one to deliver a safe anesthetic. First, one must make sure that the cardiac

anatomy and the direction of any intracardiac shunts are understood. Second, if

intracardiac shunts are present, one must be hypervigilant about not allowing air in the

intravenous line to enter the patient as this may find its way to the left side of the heart

and out into the systemic circulation to possibly cause myocardial or cerebral ischemia.

Third, one must be conscious of the increased propensity in these patients to have a
 14

pulmonary hypertensive crisis that may cause a left-to-right shunt to become right-to-left,

which leads to cyanosis and possibly acidosis. Immediate measures that should be taken

to reverse pulmonary hypertension include increasing the PaO2 and decreasing the PaCO2

(but avoid overdistension of the lungs which can also increase pulmonary vascular

resistance or PVR). In addition, one should consider administration of volume to

increase preload, sodium bicarbonate to reverse any acidosis that may have developed,

phenylephrine to increase the systemic vascular resistance (SVR), or opioids to reduce

sympathetic outflow to the pulmonary bed (care must be taken as this may decrease the

SVR as well). Table 3 shows a more comprehensive list of common causes of an

increase in the PVR, and methods of treatment. Finally, one must not forget to administer

subacute bacterial endocarditis (SBE) prophylaxis when indicated (see Table 4).

Post-operative considerations

Certainly no patient with DS should be denied adequate pain control; however, it must be

recognized that these patients can rarely be treated for pain similarly to their age-matched

counterparts without DS. While formulating the pain control plan, one must keep in mind

the patient’s ability to request pain medications or to use a patient-controlled analgesia

button. When using opioids, it is important to consider the potential need to avoid any

degree of hypercarbia in the patient who has episodes of pulmonary hypertension.

Alternatively, one should consider if the patient is cooperative enough for a nerve-block

technique or if it is possible to incorporate this during a general anesthetic. Another

consideration is the need for a sitter (if no family member will be available) in order to
 15

prevent the patient from possibly injuring him/herself (i.e., pulling out the IV, falling out

of bed, eating something they should not).

Other problems frequently encountered in the immediate post-operative period are airway

obstruction (again, up to 100% may have sleep apnea), agitation from pain as well as the

unfamiliar environment, and control of blood sugar in those patients who have diabetes

mellitus.

Conclusion

Although caring for the patient with DS may cause some anxiety among

anesthesiologists, it usually is a rewarding experience to interact with these typically

happy, warm and outgoing individuals. As our ability to treat congenital heart disease

and pulmonary hypertension improves, we can expect to see more patients with DS

present for surgery.

 16

Table 1. Common Medical Conditions in the Patient with Down Syndrome

Mental Retardation
Hypo/hyper-thyroidism
Diabetes
Obesity
Sleep Apnea
Otitis Media/Hearing Loss
Gastrointestinal Disorders
Hematologic Disorders
Atlantoaxial Instability
Recurrent Pulmonary Infections

Congenital Heart Disease

 17

Table 2. Symptoms of Atlantoaxial Instability

Neck Pain
Gait Disturbance
Incontinence (urinary/fecal)
Motor Weakness
Torticollis
Ataxia
 18

Table 3. Factors that increase PVR Potential Treatment

Acidosis Reverse cause of acidosis, sodium bicarbonate
Hypoxia Increase PaO2 with supplemental O2, FIO2, etc.
Hypercarbia Decrease PaCO2 by RR and TV, etc.
Increased airway pressure Avoid high airway pressures by adjusting
ventilation
Sympathetic stimulation Opioids, deepen anesthesia
 19

Table 4. Indications for Endocarditis Prophylaxis

Artificial valve
> 1+ (trace) valvular regurgitation
Presence of any shunt (except an atrial septal defect)
Congenital heart disease surgery within the past 6 months
Previous history of endocarditis
> Mild coarctation of the aorta
 20

References:
1
American Academy of Pediatrics: Health supervision for children with Down syndrome; Committee on Genetics.

Pediatrics 2001;107:442-449
2
Smith D: Health care management of adults with Down syndrome. Am Fam Physician 2001;64:1031-8
3
Anwar AJ, Walker JD, Frier BM: Type 1 diabetes mellitus and Down's syndrome: prevalence, management and diabetic

complications. Diabet Med 1998;15:160-3

4
Prasher, VP:Overweight and obesity among Down syndrome adults. J of Intel Dis Res 1995; 35:437-441
5
Luke AH, Sutton M, Schoeller DS, Roizen NJ: Nutrient intake and obesity in prepubescent children with Down syndrome.

J Am Diet Assoc 1996; 96:1265-7

6
Kanamori G, Witter M, Brown J, Williams-Smith L: Syndromic and other congenital anomalies of the head and neck:

Otolaryngologic manifestations of Down Syndrome. Otolaryngol Clin North Am 2000;33: 1285-92

7
Bower CM, Gungor A: Pediatric obstructive sleep apnea syndrome. Otolaryngol Clin North Am 2000;33:49-73
6

8
Shott SR, Joseph A, Heithaus D: Hearing loss in children with Down syndrome. Int J Pediatr Otorhinolaryngol

2001;61:199-205
1

9
Van Riper M, Cohen W: Caring for children with Down Syndrome and their families. J Pediatr Health Care 2001;15:123-

131
10
Fong CT, Brodeur G: Down’s syndrome and leukemia; epidemiology, genetics, cytogenetics and mechanisms of

leukemogenesis. Cancer Genet Cytogenet 1987;28:55

1

11
Hasle H: Pattern of malignant disorders in individuals with Down’s syndrome. The Lancet Oncol 2001; 2:429-436
12
Zipursky A, Brown EJ, Christensen H, Sutherland R, Doyle J: Leukemia and/or myeloproliferative syndrome in neonates

with Down syndrome. Semin Perinatol 1997: 21:97-101

13
Zipursky A, Brown EJ, Christensen H, Doyle J: Transient myeloproliferative disorder (transient leukemia) and

hematologic manifestations of Down syndrome. Clin Lab Med 1999; 19:157-67, vii
1

14
http://www.ds-health.com/hemat.htm
15
Harley EH, Collins MD: Neurologic sequelae secondary to atlantoaxial instability in Down Syndrome. Arch of Otolaryn

1994: 120;159-165.
16
Motoyama EK, Davis PJ, ed. Smith’s Anesthesia for Infants and Children, 6th edition. New York, Mosby, 1996. p 868
17
Behrman: Nelson Textbook of Pediatrics, 16th ed. W. B. Saunders Company, 2000. Section 613.2
18
Williams JP, Somerville GM, Miner ME, Reilly D: Atlanto-axial subluxation and Trisomy-21: Another perioperative

complication. Anes 1987;67:253-254

19
Cooney TP, Thurlbeck WM: Pulmonary hypoplasia in Down’s syndrome. N Engl J Med 1982; 307:1170-3
20
Schloo BL, Vawter GF, Reid LM: Down syndrome: patterns of disturbed lung growth. Hum Pathol 1991;22:919-923
21
Cooney TP, Wentworth PJ, Thurlbeck WM: Diminished radial count is found only postnatally in Down’s syndrome.

Pediatr Pulmonol 1988;5:204-9

22
Marino B, and Pueschel S, ed. Heart Disease in Persons with Down syndrome. Baltimore, Paul H Brookes Publishing

Co., 1996, p. 122

2

23
Ugazio AG, Lanzavecchia A, Jayakar S, Plebani A, Duse M, Burgio R: Immunodeficiency in Down’s syndrome. Acta

Paediatr Scand 1978;67:705-708

2

24
Marino B, and Pueschel S, eds. Heart Disease in persons with Down syndrome. Copyright 1996 Paul H Brookes

Publishing Co., pp. 22 and 49.

25
Greenwood RD, Nadas AS: The clinical course of cardiac disease in Down’s synrome. Pediatrics 1976;58:893-897
26
Marino B, and Pueschel S, ed. Heart Disease in persons with Down syndrome. Copyright 1996 Paul H Brookes

Publishing Co. p. 134

27
Marino B, and Pueschel S, ed. Heart Disease in persons with Down syndrome. Copyright 1996 Paul H Brookes

Publishing Co. p. 116

2

28
Hopkins WE, Fukagawa NK, Sobel BE, Schneider DJ. Plasminogen activator inhibitor type 1 in adults with Down

syndrome and protection against macrovascular disease. Am J Cardiol 2000;85:784-6, A9

29
McKusick VA: Mechanisms in genetic diseases of man. Am J of Med 1957;22;676-86
30
Mir GH, Cumming GR: Response to atropine in Down’s syndrome. Arch Dis Child 1971;46:61-5
6
31
Shott S: Down syndrome: Analysis of airway size and a guide for appropriate intubation. Laryngoscope 2000;110:585-

592
32
Kobel M, Creighton RE, Steward DJ: Anaesthetic considerations in Down’s syndrome: experience with 100 patients and a

review of the literature. Can Anaesth Soc J 1982;29:593-598

33
Sherry KM: Post-extubation stridor in Down’s syndrome. Br J Anaesth 1983;55:53-55

Multiple Choice Questions

1. Which of the following is true about weight in the patient with Down syndrome (DS)?
a. Infants with DS tend to follow height and weight curves that are slightly higher than the rest
of the population
b. Once they reach the age of 3, patients with DS start to exhibit a fall off of the normal weight
and height curves
* c. Up to 50% of adult patients with DS are overweight
d. Obesity in the patient with DS rarely contributes to worsening of other pre-existing
conditions

2. Post-operative pain control in the patient with DS

a. is best accomplished with prn opioid administration
* b. might include use of PCA
c. should not include consideration of pulmonary hypertension issues since they are unlikely to
occur
d. is best accomplished with non-opioids since this population is much more sensitive to
opioids

3. Problems frequently encountered in the post-operative period include:

* a. airway obstruction
b. hypothyroidism
 c. poor control of blood sugar
d. excessive salivation after use of atropine (paradoxical effect)

4. Obstructive sleep apnea

a. is seen in approximately 25% of the population with DS
b. may be centrally mediated in some patients with DS
c. is almost always cured by adenotonsillectomy in the patient with DS
* d. may be treated nonsurgically by CPAP or weight loss programs

5. Typical airway characteristics of the patient with DS include:

* a. midface hypoplasia
b. macrognathia
c. muscular hypertonia
d. normal-sized larynx but small pharynx

6. GI abnormalities frequently found in the newborn with DS include:

a. volvulus
* b. pyloric stenosis
c. diarrhea
d. lower esophageal sphincter incompetence

7. Transient abnormal myelopoiesis (TAM) of the newborn with DS

a. spontaneously resolves after approximately one year
b. is differentiated from AML by megakaryocyte count
c. frequently progresses to hydrops fetalis
* d. is characterized by megakaryocytes in peripheral blood

8. All patients with DS should have

a. annual radiographs taken of the c-spine
* b. annual neurological exams
c. evaluation by a neurosurgeon for prophylactic atlantoaxial fusion
d. a soft cervical collar placed before surgery

9. The lungs of patients with DS

a. are usually relatively normal
* b. have a double capillary network around the alveoli
c. display hypoplastic alveoli
d. show increased numbers of alveoli

10. The second most common congenital heart defect seen in the patient with DS is
* a. ventricular septal defect
b. atrial septal defect
c. Tetralogy of Fallot
d. hypoplastic left heart syndrome

11. Most patients with DS and a congenital heart defect

 a. have a large right-to-left shunt
b. have definitive surgical repair only after the age of 5 years
* c. have signs of pulmonary overcirculation
d. are resistant to pulmonary vascular occlusive disease

12. In the older adult patient with DS

a. accelerated coronary artery disease is commonly seen
b. valvular incompetence is rarely seen
c. atrial arrythmias are almost always found
* d. mitral valve prolapse is found in at least 50% of patients

13. According to Shott, the proper size tracheal tube for the patient with DS can be determined by the
formula:
a. (16+age)/4
b. 4 + age/4
* c. age/4 + 3
d. (2+age)/2

14. Factors that increase pulmonary vascular resistance include:

a. hyperpnea
* b. hypercarbia
c. decreased sympathetic stimulation
d. hyperoxia

15. Maneuvers to decrease pulmonary vascular resistance include:

* a. increasing the PaO2
b. adjusting airway pressures to the highest tolerated without barotrauma
c. permissive hypercapnia
d. avoiding opioids