Obstructive Sleep Apnea in Children With Down Syndrome

Obstructive

Sleep

Apnea

in Children

With

Down

Syndrome

Carole

L. Marcus,

MBBCh;

Thomas

G. Keens,

MD; Daisy

B. Bautista,

RPFT;

Walter

S. von Pechmann;

and Sally

L. Davidson

Ward,

MD

From the Division of Neonatology and Pediatric Pulmonology, Childrens Hospital of Los Angeles, Department of Pediatrics, University of Southern California School of Medicine, Los Angeles

ABSTRACT. Children with Down syndrome have many

predisposing factors for the obstructive sleep apnea

syn-drome (OSAS), yet the type and severity of OSAS in this population has not been characterized. Fifty-three

sub-jects with Down syndrome (mean age 7.4 ± 1.2 [SE]

years; range 2 weeks to 51 years) were studied. Chest wall

movement, heart rate, electrooculogram, end-tidal Po2 and Pco,, transcutaneous P02 and Pco2, and arterial oxygen saturation were measured during a daytime nap

polysomnogram. Sixteen ofthese children also underwent

overnight polysomnography. Nap polysomnograms were

abnormal in 77% of children; 45% had obstructive sleep

apnea (OSA), 4% had central apnea, and 6% had mixed apneas; 66% had hypoventilation (end-tidal Pco, >45 mm Hg) and 32% desaturation (arterial oxygen satura-tion <90%). Overnight studies were abnormal in 100% of children, with OSA in 63%, hypoventilation in 81%, and desaturation in 56%. Nap studies significantly underes-timated the presence of abnormalities when compared to overnight polysomnograms. Seventeen (32%) of the

chil-dren were referred for testing because OSAS was clini-cally suspected, but there was no clinical suspicion of OSAS in 36 (68%) children. Neither age, obesity, nor the presence of congenital heart disease affected the mci-dence of OSA, desaturation, or hypoventilation. Polysom-nograms improved in all 8 children who underwent

ton-sillectomy and adenoidectomy, but they normalized in

only 3. It is concluded that children with Down syndrome frequently have OSAS, with OSA, hypoxemia, and hy-poventilation. Obstructive sleep apnea syndrome is seen frequently in those children in whom it is not clinically

suspected. It is speculated that OSAS may contribute to

the unexplained pulmonary hypertension seen in children

Received for publication Sep 29, 1989; accepted Sep 25, 1990.

This paper was presented, in part, at the annual meeting of the

American Thoracic Society, Cincinnati, OH, May 1989; and the

Eighth Conference on Apnea of Infancy, Palm Springs, CA, January 1990.

Reprint requests to (S.L.D.W.) Division of Neonatology and

Pediatric Pulmonology, Childrens Hospital of Los Angeles, 4650

Sunset Blvd, Los Angeles, CA 90027.

PEDIATRICS (ISSN 0031 4005). Copyright © 1991 by the

American Academy of Pediatrics.

with Down syndrome. Pediatrics 1991;88:132-139; Down

syndrome, sleep-disordered breathing, obstructive sleep

apnea.

ABBREVIATIONS. OSAS, obstructive sleep apnea syndrome;

Petco2, end-tidal carbon dioxide tension; Sao2, arterial oxygen

saturation; OSA, obstructive sleep apnea.

The obstructive sleep apnea syndrome (OSAS), which consists of complete and partial sleep apnea, hypoventilation, and arterial oxygen desaturation, may result in morbidity in children and is fre-quently undiagnosed. It has been shown to result in neurodevelopmental problems such as excessive

daytime somnolence, behavioral disturbances,

school failure, and developmental delay.’ Failure to thrive has been reported.’ Chronic hypoxemia and

hypoventilation may result in pulmonary

hyperten-sion, congestive heart failure,’ and death.2

Children with Down syndrome have many pre-disposing factors for OSAS. These include

midfa-cial and mandibular hypoplasia,3 glossoptosis, an abnormally small upper airway with superficially positioned tonsils and relative tonsillar and aden-oidal encroachment,4’5 increased secretions, an in-creased incidence of lower respiratory tract anom-alies,5 obesity, and generalized hypotonia with re-sultant collapse of the airway during inspiration.

A few case reports describing patients with Down

syndrome and OSAS have been published in the

literature.610 In a group of 12 infants and young children with Down syndrome, Southall et a14 found that 50% had OSAS. However, the type and severity of sleep-disordered breathing in a larger group of

char-acterized. Therefore, we studied 53 children with

Down syndrome of all ages, including obese and

nonobese children and those with and without con-genital cardiac disease, to better ascertain the na-ture and severity of OSAS in the population.

SUBJECTS

AND

METHODS

Children with Down syndrome were recruited

from the Down Syndrome Parents’ Group as well

as hospital subspeciality services. In addition, chil-dren with Down syndrome who were referred to the sleep laboratory by their physicians for evaluation

of clinically suspected OSAS during the study

period were included. Eight normal, healthy control children were recruited from the general

popula--. tion. Children with acute intercurrent infections at

the time of study were excluded. Informed consent was obtained from the parents or legal guardians of each child. This study was approved by the Insti-tutional Review Board of Childrens Hospital of Los Angeles.

A history was obtained from the parents of each child. The history was considered to be suggestive of OSAS ifthe child, in addition to habitual snoring, was frequently noted to have difficulty breathing during sleep or to stop breathing during sleep.

Polysomnographic studies were performed during a daytime or evening nap, in a quiet dark room with an ambient temperature of 24#{176}Cin the Sleep Phys-iology Laboratory of Childrens Hospital of Los Angeles. Children were studied for 1 to 2 hours.

Those who did not sleep spontaneously were

se-dated lightly with chloral hydrate. An initial dose

of 50 mg/kg body weight (maximal dose of 1000

mg) was administered by mouth. Occasionally, a

second dose of 25 mg/kg (maximum of 500 mg) was given if the child failed to sleep after a 30-minute observation period.

To ascertain whether nap studies adequately

rep-resented overnight polysomnograms, and to help

distinguish the effects (if any) of sedation, a con-secutively enrolled, representative sample of sub-jects underwent both daytime nap and overnight polysomnography on separate occasions. In addi-tion, eight normal, healthy control children under-went overnight polysomnography. No sedation was used for the overnight studies.

The following parameters were measured and

recorded continuously on a Gould 16-channel strip chart recorder during both nap and overnight stud-ies: chest wall movement, by thoracic impedance; heart rate, by electrocardiogram; inspired and end-tidal Po2 and Pco2 (PetcO2), sampled at the nose or mouth at a rate of 60 mL/min by mass spectrom-etry (Perkin-Elmer medical gas analyzer) (in

addi-tion, airflow was also monitored at the opposite position with a thermistor [Physitemp, Clifton, NJ] in some subjects.); transcutaneous Po2 and

Pco2,

elec-trode (SensorMedics Transcend Cutaneous Gas

System, SensorMedics, Anaheim, CA); arterial oxy-gen saturation (Sao2), by pulse oximetry (Nellcor

N 200 Pulse Oximeter, Hayword, CA); oximeter

pulse tracing; and electrooculogram. Subjects were also monitored and recorded on videotape, using an infrared video camera. The children were continu-ously observed by a technician trained in polysom-nography. Observations of the child’s sleep behav-ior and respiratory events were recorded directly on the strip chart paper by the technician.

Polysomnograms were defined as abnormal if

they demonstrated one or more of the following: any episode of obstructive sleep apnea (OSA) last-ing for two or more consecutive breaths”; central apnea >15 seconds duration and associated with desaturation or bradycardia’2; mixed apneas (ap-neic episodes with both obstructive and central

components); hypoventilation (PetcO2 >45 mm

Hg’3); or hypoxemia (Sao2 <90%). Subjects with significant cardiac disease were considered to have sleep-related hypoxemia only if they displayed a fall in SaO, of greater than 5% from wakefulness

to sleep, in addition to a sleeping Sa02 of less than 90%.’’ Saturation measurements associated with poor pulse tracings were discarded.

The results of all sleep studies were made avail-able to the child’s primary physician. Therapeutic decisions were made on an individual basis by the patient’s family and primary physician. Follow-up polysomnography was obtained following any sur-gical intervention.

All data are expressed as means ± SEM. To

detect differences between children with Down syn-drome who were referred for clinically suspected OSAS, and those who were prospectively recruited, population characteristics and the incidence of po-lysomnographic abnormalities were compared, using x2 analysis. To detect differences between children with Down syndrome who underwent both

nap and overnight polysomnography, and those

who underwent nap polysomnography alone,

pop-ulation characteristics and the incidence of poly-somnographic abnormalities were compared, using

RESULTS

Population Characteristics

Fifty-four children with Down syndrome were studied, of whom one was excluded from analysis

because he had pneumonia at the time of study. Seventeen (32%) of the children were referred to the sleep laboratory by their physicians for evalu-ation of clinically suspected OSAS. The number of children clinically referred increased during the study period, probably because of a raised level of awareness of OSAS in this population in our insti-tution as the study progressed.

Population characteristics for the Down

syn-drome children are shown in Table 1. The mean age was 7.4 ± 1.2 years. Ages ranged from 2 weeks to 51 years, with eight subjects younger than 1 year of age and four subjects 18 years or older. Eight control children underwent overnight polysomnog-raphy only.

Associated medical conditions in the Down syn-drome children were determined from parental his-tory and a review of medical records. Twenty-three children (44%) had congenital heart disease. 5ev-enteen of these had either minor lesions (no clini-cally significant shunt or cyanosis) or fully repaired

lesions. Six children had clinically significant

car-diac disease. Both height and weight measurements were available for 48 children. Of these, 40% were obese with weights greater than 120% of their ideal weight for height.’6 Two children had mild asthma

but were asymptomatic at the time of study; no

other child had pulmonary disease. Three children had previously undergone tonsillectomy and aden-oidectomy. One child had cerebral palsy and a

sei-TABLE 1. Results of Nap Polysomnography in

Chilnd-rome*

No. (%)

No. of children 53

Age, y (mean ± SEM) 7.4 ± 1.2

Male 32 (60)

OSAS suspected by physician 17 (32)

OSAS suspected by parents 19 (39)t

Obstructive apnea 24 (45)

Central apnea 2 (4)

Mixed apnea 3 (6)

Hypoventilation 35 (66)

Range of highest Petco,, mmHg 34-57

Desaturation 17 (32)

Range of lowest SaO2, % 58-100

Multiple abnormalities 26 (49)

Abnormal polysomnogram 41 (77)

* OSAS, obstructive sleep apnea syndrome; Petco2,

end-tidal carbon dioxide tension; SaO,, arterial oxygen

satu-ration.

t Data available for 49 subjects.

zure disorder in addition to Down syndrome and

was receiving anticonvulsants at the time of study; another child was receiving amitriptyline, pheny-tom, and trihexyphenidyl HC1 for autistic behavior. Three children were receiving thyroxine supple-mentation, all of whom were euthyroid at the time of study. No control children had any illness or were receiving any medications.

Nap Studies

The data for the children referred clinically by

their physicians because of suspected OSAS and

the volunteers were group together as there were no statistically significant differences between the two groups. The groups were similar in composition (no difference in mean age, sex, presence of obesity, or significant cardiac disease). There were no sig-nificant differences between the two groups in the use of sedation, number of children with OSA, central apneas, hypoventilation, desaturation, mul-tiple abnormalities or total number of abnormal studies, or mean highest Petco2 and mean lowest

Sao2.

Polysomnograms were abnormal in 41 (77%) of

the 53 children. The number and types of abnor-malities are shown in Table 1. Of all children stud-ied, 24 (45%) had obstructive apnea. This was as-sociated with desaturation in 1 1 and hypoventila-tion in 18. Three children (6%) had mixed apneas.

Two children (4%) had prolonged central apnea

associated with desaturation; both of these children also had obstructive and mixed apneas. Hypoven-tilation was present in 35 children (66%) and was the commonest abnormality detected. Desaturation was present in 17 patients (32%).

of the 41 children with abnormal polysomno-grams, most (63%) had multiple types of abnor-malities. However, 12 children had persistent hy-poventilation alone, 2 had multiple obstructive ap-neas alone, and 1 had multiple episodes of desaturation alone. There was no significant rela-tionship between the presence of desaturation, hy-poventilation, OSA, or abnormal polysomnograms and sex, obesity, or the presence of congenital heart disease.

A typical example of a polysomnography tracing obtained from one of the children with Down syn-drome is shown (Fig 1). A normal tracing is shown for purposes of comparison (Fig. 2).

Comparison

Between

Nap and Overnight

Pnlvsnmnnnrnhv

chil-Cs.., WaS Msn.ms,e J

250 OO] :Zi:---- - .- - 200

! ‘-+:f-t- +

L::-

-

:‘:

: iii

a&

Chest WettMonimsnt i’ ‘-_.‘ju\ AW\v’-”JA1/vV.’ ...\/

Th.n::: ETT1TTJTTT

Fig 1. Sample tracing from a polysomnograph of a child

with Down syndrome. The scale for transcutaneous

oxy-gen tension (Tco,) is the same as that for end-tidal oxygen tension (Peto,); and the scale for transcutaneous carbon dioxide tension (Tcco2) is the same as that for

end-tidal carbon dioxide tension (Petco,). Several

epi-sodes of obstructive apnea (of up to 15 seconds duration)

are shown by the presence of chest wall movement with

cessation of airflow. This is associated with desaturation

(to 75%) and hypercarbia (to 49 mm Hg). Sao,, arterial

oxygen saturation; EOG, electrooculogram.

]niico:-:1

wmii0

o,- Pu.. ss

-

-

-t-.” t

Fig 2. Sample tracing from a normal polysomnograph

of a child with Down syndrome. All scales and

abbrevia-tions are the same as in Fig 1. There is no obstructive

apnea, desaturation, or hypoventilation.

then did not differ significantly with regard to age, sex, or clinical suspicion of OSAS. None of this group had significant cardiac disease. The mean

time between the two studies was 17 ± 4 days.

Thirteen children (81%) were sedated with chloral hydrate for the nap study; none were sedated for the overnight study. During the overnight studies, the mean percentage of sleep time with hypoventi-lation was 38 ± 8%. The mean percentage of sleep time with desaturation was 4 ± 2%. The mean percentage of sleep time with obstructive apnea was

1 ± 0%. Similar percentages were not calculated for nap studies as normative data are unavailable. The number of children with abnormal studies, OSA,

hypoventilation, desaturation, and multiple

abnor-malities on nap and overnight studies were

com-pared. Twelve children (75%) had abnormal nap

studies, whereas all 16 children had abnormal over-night polysomnograms.

The most common abnormality during overnight polysomnography was hypoventilation, found in

81% of children. Multiple abnormalities during

overnight polysomnography were observed in 63%. In comparison, 50% of children with abnormal nap studies displayed multiple abnormalities. The

de-grees of hypoventilation and desaturation were

sig-nificantly greater during the overnight studies (Table 2). Thus, nap studies underestimated abnor-malities. There were no instances where abnormal-ities were present on the nap studies but not the

overnight studies.

Eight control children underwent overnight po-lysomnography only (Table 2). No sedation was used for these studies. Their mean age was 9.2 ±

1.7 (range 1.3 to 15.3) years, which was not signif-icantly different from the Down syndrome children

undergoing overnight polysomnography. One

con-trol child had a single, 9-second OSA, which was

not associated with either hypoventilation or de-saturation. No control children had any episodes of hypoventilation or desaturation.

Histories from parents were available for 49 chil-dren. Of these, 19 (39%) had histories suggestive of

OSAS. Polysomnograms were abnormal in all 19

(100%). However, 18 (60%) of the 30 children with negative histories also had abnormal polysomno-grams. Physicians’ suspicions of OSAS were based on clinical referrals of patients for polysomnogra-phy. Of the 17 children referred by physicians be-cause of suspected OSAS, 16 (94%) had abnormal studies.

Response

to Tonsillectomy

and Adenoidectomy

Tonsillectomy and adenoidectomy were per-formed in eight children with Down syndrome. Subsequent polysomnograms were performed at 3.8

± 1.6 (mean ± SEM) months postoperatively (Fig

3). Although most patients improved, polysomno-grams normalized completely in only three. In ad-dition, three patients were studied only following tonsillectomy and adenoidectomy. These three pa-tients had all had surgery in other institutions in the past and were referred to us because of

persist-ent symptoms suggestive of OSAS. They all had abnormal studies.

DISCUSSION

Our study shows that significant OSAS is com-mon, and often unsuspected, in children with Down syndrome. Of the children studied, 77% had abnor-mal polysomnograms. Hypoventilation was the

most frequent abnormality demonstrated, being

TABLE 2. Comparison of Nap and Overnight Polysomnography*

Down Syndrome

0 Before I S A

. After I S A

Children

with

OSA

(hb)

LJ

Surgery

PETCO2

(mmhg)

60

50

40

30

7

100

90

5002 80

70

60 50

E

Atter

Surgery

Control Subjects: Overnight, No.

(%) Nap, No.

(%)

.

Overnight,

No. (%)

No. of children 16 16 8

Sedation 13 (81) 0 (0) 0 (0)

Obstructive sleep apnea 7 (44) 10 (63) 1 (13)

Hypoventilation 11 (69) 13 (81) 0 (#{216})d

Desaturation 5 (31) 9 (56) 0 (0)’

Abnormal polysomnogram 12 (75) 16 (100)#{176} 1(13)d

Multiple abnormalities 8 (50) 10 (63) 0 (0Y

Highest Petco,, mm Hg

Mean ± SEM 49 ± 1 52 ± 2b 43 ± ld

Range 41-58 42-64 38-45

Lowest Sa02, %

Mean ± SEM 89 ± 2 82 ± 3’ 95 ± d

Range 68-97 57-97 94-97

* Down syndrome nap vs overnight polysomnography: ap < .025; 1) < .05; ‘ J3 < .005.

Down syndrome overnight vs control overnight polysomnography: dJ3 < .001; ep < .002

Fig 3. The percentage of Down syndrome children with obstructive sleep apnea (OSA) before and after

tonsillec-tomy and adenoidectomy (T & A) are shown in the left

panel. The end-tidal carbon dioxide tension (PetCo2) and arterial oxygen saturation (Sao2) before and after T & A in individual patients are shown in the middle and right panel, respectively. Although most patients improved following T & A, one patient showed an increase in Pet

Co2.

needed to determine the clinical importance of

these abnormalities.

It is possible that our results are affected by selection bias. However, only 32% of the children studied were referred by physicans. Most of the children were recruited from the Down Syndrome Parents’ which is not affiliated with a hospital, and their parents were unaware of the objectives of our study. Furthermore, 57% ofall children in our study did not have a history suggestive of OSAS. There was no difference in polysomnography results be-tween those children referred by physicians and

those recruited from the Down Parent Support

Group. We therefore do not believe that selection bias significantly affected our results. The results

of our study are consistent with the preliminary data recently reported in abstract form by Stebbens et al,’7 which showed evidence of upper airway obstruction in 41% of a geographically based pop-ulation of Down syndrome children.

Nap studies may underestimate abnormalities during sleep because of the shorter duration of the studies, the lack of inclusion of all sleep stages, and circadian variation in sleep patterns.’8 Further-more, sedation was required to induce sleep in the majority of children studied during daytime naps, which may have affected the results of the studies. Although chloral hydrate does not affect either the hypoxic or hypercarbic respiratory drive,’9’20 animal data suggest that it may depress upper airway mus-cle tone.2’ Therefore, a consecutively enrolled, rep-resentative sample of subjects underwent both day-time nap and overnight studies to validate our methods. Our results show that the nap studies significantly underestimated abnormalities, even when chloral hydrate was used. There were no individual cases where nap studies were more ab-normal than overnight studies. This confirms the findings of previous investigators.2224 Therefore, it

is probable that most of the children had more severe OSAS than was demonstrated on the nap

studies.

Normal parameters for polysomnography in chil-dren have not been clearly defned. We considered obstructive apneas lasting from two or more

con-secutive breaths to be abnormal, as obstructive apneas of any length are only rarely demonstrated

asso-ciated with snoring and retractions. This may have led to an underestimation of the severity of obstruc-tive events. Although central apneas of greater than

15 seconds duration are considered abnormal in neonates and infants,’2 this may not be true for older children.25’26 Therefore, we classified central apneas as abnormal only if they were greater than 15 seconds duration and associated with either bradycardia or desaturation. Both of the children with pathological central apnea in our study also had obstructive and mixed apneas, and their central apnea was therefore probably related to upper air-way obstruction.25

We considered Sao2 <90% as abnormal. Children with clinically significant heart disease were con-sidered to have sleep-related desaturation only if they showed a sleep-related fall in Sao2 of greater than 5% in addition to this. The mean maximal drop in nocturnal oxygen saturation in normal chil-dren studied by Chipps et al’4 was 2.2 ± 1.2%, with no drops greater than 4%, while Tabachnik et al’5 showed a maximal fluctuation of 1.5%. None of our controls had Sa02 <90% during overnight polysom-nography. A Petco2 >45 mm Hg was considered abnormal as it is two standard deviations from the normal.1’ Other studies have shown normal sleep-ing Petco2 in infants to be in the range of 34 to 37

mm Hg.27 None of our control subjects had Petco2 >45 mm Hg during overnight polysomnography. These parameters for desaturation and hypoventi-lation are the standards used in other centers to evaluate OSAS in children.28

Children with Down syndrome have many

ana-tomic predisposing factors for OSAS. These include midlacial and mandibular hypoplasia,3 glossoptosis, and an abnormally small upper airway with super-ficially positioned tonsils and relative tonsillar and adenoidal encroachment.4’5 In addition, these chil-dren are usually hypotonic, have difficulty clearing secretions, and are frequently obese. Determining which of these factors is the major determinant of OSAS in this group of children is beyond the scope of this paper. However, we have shown that obesity

alone is not a major component. In otherwise

healthy children, large tonsils and adenoids are the

major cause of OSAS,’ which can usually be

re-solved by tonsillectomy and adenoidectomy.29 To date, only a small proportion of our subjects with abnormal polysomnograms have undergone

tonsil-lectomy and adenoidectomy. It appears that, in

some subjects, there may be a substantial improve-ment following surgery. It is too early to determine

whether this improvement will be permanent. In

Levine and Simpser’s7 series of four patients, three of the four children with Down syndrome and

Se-vere OSAS who underwent tonsillectomy and

ad-enoidectomy eventually required tracheostomy for permanent relief of their symptoms. In most of our patients, polysomnograms did not completely nor-malize following surgery. In addition, three subjects who were only studied following surgery were re-ferred to us because of suspected, persistent OSAS

and had markedly abnormal polysomnograms.

Thus, some Down syndrome patients may require either more extensive surgery, such as uvulopala-topharyngoplasty or tracheostomy,5 to relieve their obstruction or alternative modes of treatment such as nasal continuous positive airway pressure. A full clinical evaluation would be required to outline the best treatment modality for each individual patient.

Obstructive sleep apnea syndrome in patients with Down syndrome often remains undiagnosed by both physicians and parents. One reason for this may be that many of the sequelae of OSAS, such as failure to thrive, pulmonary hypertension, and behavioral problems, are also disorders commonly associated with Down syndrome. Thus, the pres-ence of these symptoms does not prompt an inves-tigation for their etiology, as it would in otherwise normal children. Parental history alone does not appear to be a sensitive screening test for OSAS in this population. Although polysomnograms were abnormal in all symptomatic children, they were also abnormal in 60% ofthe asymptomatic children. On the basis of our study, we would recommend that physicians have a high index of suspicion for

OSAS when evaluating children with Down

syn-drome and that polysomnography be performed in

any child with a history or physical examination suggestive of OSAS or its complications.

Obstructive sleep apnea syndrome can cause pul-monary hypertension resulting in cor pulmonale”3#{176} and congestive heart failure.3’ This is secondary to pulmonary vasoconstriction caused by chronic, in-termittent hypoxemia and respiratory acidosis dur-ing sleep.3#{176}Many children with Down syndrome have pulmonary hypertension unassociated with, or out of proportion to, congenital heart disease.32’33 Controversy exists as to the etiology of this asso-ciation. Proposed mechanisms include abnormal capillary morphology32 and pulmonary hypopla-sia.34 It is possible that OSAS in some children may be a significant contributor. The pulmonary hyper-tension may be reversible, at least in part, by re-lieving the airway obstruction.3#{176} This was demon-strated by both Loughlin et a18 and Kasian et al,’#{176} who relieved upper airway obstruction in patients

with Down syndrome by intubating them during

the upper airway obstruction have been reported by Rowland et a!9 and Levine and Simpser.7 In those

children with Down syndrome and pulmonary

hy-pertension not totally explained by intrinsic cardiac disease, evaluation for the presence of OSAS should be considered.

OSAS may adversely affect behavior, growth, and neurodevelopment in otherwise normal children.”25 It is possible that in some children with Down syndrome, underlying developmental and behav-ioral abnormalities may be exacerbated by the hy-poxemia and sleep disruption that accompanies OSAS.

Infants and children with Down syndrome are

now surviving into adulthood, although they still have a markedly reduced life expectancy. A recent study reported a 71% survival to age 30 years, vs

97% of the general population.35 Whereas previ-ously these children were institutionalized or re-ceived supportive care only, it is now recognized that they can be functional members of society, and efforts are being made not only to prolong their lives but also to improve their quality of life. Repair of congenital cardiac lesions is now a standard of care, and it has been suggested that these children not be deprived of other complex medical treat-ments such as bone marrow transplants.36 Identi-fication and treatment of common problems such as OSAS prior to the development of serious com-plications is thus reasonable and desirable.

ACKNOWLEDGMENTS

This research was supported, in part, by grants from

the Greater Los Angeles and Washington State Chapters of the National Sudden Infant Death Foundation; the Los Angeles County, Orange County, Inland Empire, and Kern County Chapters of the Guild for Infant Survival; the Junior Women’s Club of Orange; and the Ruth and Vernon Taylor Foundation.

We thank the members of the Down Syndrome

Par-ents’ Group for their enthusiastic support of this project. We thank Michael Stabile, MS, RPFT, Amma Amihyia, MS, Tony Hawksworth, RPFT, Adriana Rachal, RPFT, and Kate Tannenbaum for their technical assistance. We thank the physicians who referred patients to us, espe-cially Drs Richard Koch, Mimi Tutihasi, and Dennis Crockett.

REFERENCES

1. Brouillette RT, Fernbach 5K, Hunt CE. Obstructive sleep

apnea in infants and children. J Pediatr. 1982;100:31-40

2. Bradley TD, Phillipson EA. Pathogenesis and

pathophysi-ology of the obstructive sleep apnea syndrome. Med Clin

North Am. 1985;69:1169-1185

3. Fink GB, Madaus WK, Walker GF. A quantitative study of

the face in Down’s syndrome. Am J Orthod.

1975;67:540-553

4. Southall DP, Stebbens VA, Mirza R, Lang MH, Croft CB,

Shinebourne EA. Upper airway obstruction with

hypoxae-mia and sleep disruption in Down syndrome. Dev Med Child NeuroL 1987;29:734-742

5. Strome M. Obstructive sleep apnea in Down syndrome

chil-dren: a surgical approach. Laryngoscope. 1986;96:1340-1342

6. Clark RW, Schmidt HS, Schuller DE. Sleep-induced

venti-latory dysfunction in Down’s syndrome. Arch Intern Med.

1980;140:45-50

7. Levine OR, Simpser M. Alveolar hypoventilation and cor

puimonale associated with chronic airway obstruction in

infants with Down syndrome. Clin Pediatr (Phila). 1982;

21:25-29

8. Loughlin GM, Wynne JW, Victoria BE. Sleep apnea as a possible cause of pulmonary hypertension in Down

syn-drome. J Pediatr. 1981;98:435-437

9. Rowland TW, Nordstrom LG, Bean MS, Burkhardt H.

Chronic upper airway obstruction and pulmonary

hyperten-sion in Down’s syndrome. AJDC. 1981;135:1050-1052

10. Kasian GF, Duncan WJ, Tyrrell MJ, Oman-Ganes LA.

Elective oro-tracheal intubation to diagnose sleep apnea

syndrome in children with Down’s syndrome and ventricular

septal defect. Can J Cardiol. 1987;3:2-5

11. Gaultier C, Praud JP, Canet E, Delaperche MF, D’Allest

AM, Nedelco H. Respiratory adaption during sleep in

healthy infants. Clin Respir Physiol. 1986;22:54S. Abstract 12. Davidson Ward SL, Scheutz 5, Krishna V, et al. Abnormal

sleeping ventilatory pattern in infants of substance-abusing mothers. AJDC. 140:1015-1020, 1986

13. Shapiro BA, Harrison RA, Walton JR. Clinical

Interpreta-tion of Blood Bases. 3rd ed. Chicago, IL: Year Book Medical Publishers; 1982:122

14. Chipps BE, Mak H, Schuberth KC, Talamo JH, Menkes

HA, Scherr MS. Nocturnal oxygen saturation in normal and

asthmatic children. Pediatrics. 1980;65:1 157-1160

15. Tabachnik E, Muller NL, Bryan AC, Levison H. Changes

in ventilation and chest wall mechanics during sleep in

normal adolescents. J AppI Physiol. 1981;51:557-564

16. Kleinman RE. Obesity. In: Rudolph A, ed. Pediatrics.

Nor-walk, CT: Appleton & Lange; 1987:206

17. Stebbens VA, Denis J, Croft CB, Southall DP. Incidence of sleep related upper airway obstruction in Down syndrome.

Pediatr Pulmonol. 1989;7:281. Abstract

18. American Thoracic Society. Indications and standards for

cardiopulmonary sleep studies. Am Rev Respir Dis. 1989;

139:559-568

19. Lees MH, Newcomb JD, Sunderland CO, Nichols GM.

Chloral hydrate and the CO2 chemoreceptor response of the

infant. Pediatr Res. 1980;14:646. Abstract

20. Hunt CE, Hazinski TA, Gora P. Experimental effects of

chioral hydrate on ventilatory response to hypoxia and

hypercarbia. Pediatr Res. 1982;16:79-81

21. Hershenson M, Brouillette RT, Olsen E, Hunt CE. The

effect of chloral hydrate on genioglossus and diaphragmatic

activity. Pedwtr Res. 1984;18:516-519

22. Hoppenbrouwers T, Hodgman JE, Arakawa K, Harper R,

Sternman MB. Respiration during the first six months of

life in normal infants. Pediatr Res. 1980;14:1230-1233

23. Silvestri R, Guilleminault C, Coleman R, Roth T, Dement

WC. Nocturnal sleep versus daytime nap findings in patients

with breathing abnormalities during sleep. Sleep Res.

1982;1 1:174

24. Series F, Cormier Y, La Forge J. Validity of diurnal sleep

recording in the diagnosis of sleep apnea syndrome (SAS).

Am Rev Respir Dis. 1990;141:A859

25. Guilleminault C. Obstructive sleep apnea and its treatment

in children: areas of agreement and controversy. Pediatr

Pulmonol. 1987;3:429-436

26. Carskadon MA, Harvey AB, Dement WC, Guilleminault C, Simmons FB, Anders TF. Respiration during sleep in

chil-dren. WestJMed. 1978;128:477-481

27. Gaultier C. Respiratory adaption during sleep from the

neonatal period to adolescence. In: Guilleminault C, ed.

Sleep and Its Disorders in Children. New York, NY: Raven

Press; 1987:67-97

28. Brouillette RT, Weese-Mayer DE, Hunt CE. Disorders of

breathing during sleep in the pediatric population. Semin

29. Strading JR, Thomas G, Warley ARH, Williams P, Freeland

A. Effect of adenotonsillectomy on nocturnal hypoxaemia,

sleep disturbance, and symptoms in snoring children. Lan-cet. 1990;335:249-253

30. Perkin RM, Anas NG. Pulmonary hypertension in pediatric patients. J Pediatr. 1984;105:511-522

31. Guilleminault C. Obstructive sleep apnea syndrome in

chil-dren. In: Guilleminault C, ed. Sleep and Its Disorders in

Children. New York, NY: Raven Press; 1987:213-224

32. Chi TL, Krovetz U. The pulmonary vascular bed in children

with Down syndrome. J Pediatr. 1975;86:533-538

33. Yamaki 5, Horiuchi T, Sekino Y. Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with

the Down syndrome. Am J Cardiol. 1983;51:1502-1506

34. Cooney TP, Thurlbeck WM. Pulmonary hypoplasia in Down syndrome. NEnglJMed. 1982;307:1170-1173

35. Baird PA, Sadovnick AD. Life expectancy in Down

syn-drome. J Pediatr. 1987;110:849-854

36. Churchill LR. Bone marrow transplantation, physician bias,

and Down syndrome: ethical reflections. J Pediatr. 1989;

114:87-88

ON THE EVILS OF MODERN MUSIC TEACHING-AS VIEWED IN 1842

Readers of the Boston Medical and Surgical Journal in 1842 received the following warning about vocal instruction in the case of young women.’

A gentleman who lost his only daughter a few days since, by a rapid pulmonary consumption, and who has ascertained that other young ladies are suffering from the incipient symptoms like those he has so painfully witnessed in a member of his own bereaved family, suggests that lesions or some other equally injurious effects, are produced

in the lungs by the modern mode of vocal instruction. The instructor begins with the

pupil by causing a full inspiration to be made-the lungs being distended to their utmost capacity. When in that condition, a horrible noise called a sound is to follow, by allowing the slow escape of the unnatural volume of air, pressed upon by an equally unnatural effort of the external respiratory muscles. The object is said to be the strengthening of

the lungs which is absolutely ridiculous, and no more philosophical than holding one’s feet in a tub of cold water to produce a better base. Now it is true that music-masters actually begin their lessons by overstraining the delicate tissues of the air-cells, they are sowing the seeds of a wide-spread desolation that requires an immediate and careful investigation. Our young ladies are swept off with a melancholy rapidity throughout New England, almost before they have begun to live, through the combined agency of a variable climate, the vices of civilization, and dress; and if there is to be another power brought into requisition, under the specious character of a vocal education, some counteracting influences should at once be devised to undeceive parents, and to develop a less objectionable system, that does not bring disease and premature death with the

first songs of youthful vivacity.

REFERENCE

1. Medical Intelligence-Evils of modern musical teaching. Boston Med Surg J. 1842;26:146

1991;88;132

Pediatrics

L. Davidson Ward

Carole L. Marcus, Thomas G. Keens, Daisy B. Bautista, Walter S. von Pechmann and Sally

Obstructive Sleep Apnea in Children With Down Syndrome

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L. Davidson Ward

Carole L. Marcus, Thomas G. Keens, Daisy B. Bautista, Walter S. von Pechmann and Sally

Obstructive Sleep Apnea in Children With Down Syndrome

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