Alternative
Treatment
to Tracheostomy
in
Obstructive
Sleep
Apnea
Syndrome:
Nasal
Continuous
Positive
Airway
Pressure
in
Young
Children
Christian
Guilleminault,
MD, German
Nino-Murcia,
MD,
Gregory
Heldt,
MD, Roger
Baldwin,
MSc,
and David Hutchinson,
MSc
From the Sleep Disorders Clinic, Stanford University School of Medicine, Palo Alto, California, and Department of Pediatrics, University of California at San Francisco
ABSTRACT. Two groups of prepubertal children with
severe obstructive sleep apnea syndrome received
contin-uous positive airway pressure (CPAP) treatment using a
nasal mask. The five children in group A had nasal CPAP only in a hospital setting; the five children in group B received the treatment at home as well. Four of these five children, who have now been followed for as long as 10 months, have adapted well to the treatment at home. The problems encountered with nasal CPAP as a home treat-ment, such as poor cooperation from parents, allergic
rashes, eye irritation resulting from air leaks, are
dis-cussed. Despite the problems, nasal CPAP is a valid alternative to tracheostomy in children as young as 3
years of age. Pediatrics 1986;78:797-802; Continuous
pos-itive airway pressure, obstructive sleep apnea syndrome, tracheostomy, snoring.
adenoids, we found, in 1981, that 20 children of a
population of 50 were not helped by tonsillectomy
and adenoidectomy.5 For children with Treacher
Collins syndrome, Pierre Robin syndrome, or
Chiani malformations type I and II, we have had to
resort to tracheostomy.6 To avoid tracheostomy in
prepubertal children with obstructive sleep apnea
syndrome, we have experimented with CPAP. Here,
we report results, defects, and complications of
nasal CPAP in ten children between 3 and 11 years
of age, who were monitored during the treatment.
Five patients had CPAP treatment in a hospital
setting only; five also had home treatment.
PATIENT POPULATION
In 1981, Sullivan et aP adapted continuous
posi-tive airway pressure (CPAP) so that adults with
obstructive sleep apnea syndrome could use it at
home. Several articles have since reviewed the
re-sults of this approach.24 In 1985, the Food and
Drug Administration approved the first
commer-cially available nasal continuous positive airway
pressure kit for treating adults with obstructive
sleep apnea syndrome (Respironics), but little has
been published on CPAP treatment for children.
Although obstructive sleep apnea syndrome in
chil-dren is often associated with enlarged tonsils and
Received for publication Aug 9, 1985; accepted May 28, 1986. Reprint requests to (C.G.) Stanford Sleep Disorders Clinic and Research Center, Stanford University School of Medicine, Stan-ford, CA 94305.
PEDIATRICS (ISSN 0031 4005). Copyright © 1986 by the American Academy of Pediatrics.
A total of ten children (Table 1), patients 1
through 5 in group A and patients 6 through 10 in
group B, were investigated. The two girls (patients
1 and 2) and eight boys were between 3 and 11
years of age. Group A, studied between 1981 and
1983, received CPAP in a hospital setting only. The
children in group B, who were studied at a later
date and benefited from technical improvements,
received CPAP treatment at home. Patients 1, 3,
and 6 had Down syndrome, patient 2 had
fronto-metaphyseal dysplasia, and patient 4 had Treacher
Collins syndrome. Patient 7 was an achondroplastic
dwarf. The other children had a combination of
problems. Patient 5 was obese and mentally
re-tarded, the result of neonatal asphyxia, and had a
type II malocclusion. Patient 8 had congenital
men-tal retardation of unknown etiology and
retrogna-thia; patient 9 had moderate mental retardation
resulting from neonatal asphyxia, nocturnal grand
Diagnosis Age
(yr)
Sex Tonsillectomy and/or
Adenoidectomy
Tonsils and Adenoids
Enlarged*
Heavy Night
Snoringt Performed
Group A
1 Down syndrome 3 F No 2+ 4+
2 Frontometaphyseal dysplasia 8 F Yes + 3+
3 Down syndrome 9 M No 2+ 4+
4 Treacher Collins syndrome 7 M No + 3+
5 Mental retardation, malocclusion type II, obesity 7 M Yes + 4+
Group B
6 Down syndrome 3 M No 2+ 4+
7 Achondroplastic dwarf 11 M Yes + 4+
8 Mental retardation, retrognathia 6 M No + 4+
9 Mental retardation, grand mal seizures 7 M No + 4+
10 Numerous facial and maxillary congenital anom-alies, mental retardation, storage disease of unidentified type
4 M No + 4+
* Size of tonsils, from 1+ = no tonsillar enlargement, to 4+ = enlarged tonsils completely obstructing airway.
t Scale of severity of snoring, from 0 = no snoring to 4+ = loud, disruptive snoring.
TABLE 1. Patient Population
Group and Patient No.
patient 10 had a storage disease ofunidentified type
with hepatomegaly, multiple congenital anomalies
of the head and neck with a cleft lip, cleft palate,
macrocephaly, agenesis of the corpus callosum,
mental retardation, and cardiomegaly of unknown
etiology. In summary, each patient had some type
of lingual or maxillofacial problem, frequently in
combination with mental retardation. All children
were pnepubertal (Tanner stage i). Patients 2, 5,
and 7 had a tonsillectomy and/on adenoidectomy
before coming to the clinic. None had particularly
enlarged lymphoid tissues (maximum 2+ on a scale
of 1+ to 4+).
HISTORY
RELATED
TO OBSTRUCTIVE
SLEEP
APNEA
All children were initially referred for symptoms
suggesting obstructive sleep apnea syndrome. All
were said to be heavy, loud snorers; most had begun
snoring by 12 months of age. All were said to sweat
during sleep, although some more heavily than
oth-ens, and all were said to move an abnormal amount
during sleep. Patients 5, 7, and 9 were reported to
have had episodes of nocturnal wandering with
confusion and disorientation. All were reported to
have had “breathholding” (apneic) episodes during
sleep. All were said to exhibit excessively sleepy
behavior during the day, falling asleep while playing
on in school. Patients 5, 9, and 10 had been
hospi-talized in intensive cane units for cardiopulmonary
failure. The nocturnal grand mal seizures of patient
9 had increased in frequency, and patient 10 had
hypertension (state BP) of unknown etiology.
OBJECTIVE POLYGRAPHIC MONITORINGS
In-hospital monitonings during periods of sleep
always included EEG, electrooculognaphic (EOG),
chin electromyographic (EMG), and modified V2
ECG leads. Respiration was monitored by thoracic
and abdominal strain gauges in patients 1 and 2,
inductive respiratory plethysmography
(Respi-trace) in patients 3 through 10, and nasal and oral
thermistors in all ten. Respitrace was calibrated
only in patients 4, 7, 9, and 10, who were able to
collaborate and who could perform the respiratory
maneuvers required to calibrate the equipment; it
was used only as a strain gauge in patients 3, 5, 6,
and 8. Oxygen tension was measured with a
trans-cutaneous Po2 electrode; in patients 1, 2, and 3,
oxygen saturation was monitored with a Nelcor
oximeten; in patients 4, 5, and 6, it was monitored
with a Biox oximeten. Patients 7, 9, and 10 were
monitored at home using Vitalog equipment.
The Vitalog is a nine-channel, multipurpose
mi-cnopnbcesson, the size of a cassette tape recorder,
which interfaces with the Respitrace (respiratory
inductive plethysmography) and an ear oximeter
(Biox for Vitalog). Activity is measured with a
movement sensor placed on the nondominant arm.
Before onset of sleep, respiratory sensors are
cali-bnated with a microcomputer (Apple lie or IBM
XT), using commercially available software
(Vi-talog). A tight jersey band that fits over the chest
and abdomen keeps the sensors in place. Apneic
episodes, decreased tidal volume (hypopneic
epi-sodes), paradoxical movements of chest and
abdo-men, oxygen saturation, heart rate, and activity/
inactivity can all be detected with the software
program. Malfunction of the home monitoring
equipment was the result of (1) displaced
respina-tory sensors; (2) displaced oximeter, which was
attached either to a finger or ear; on (3)
microproc-fattyinftration of neck and moderate retroposition of lower mandible associated with dental malocclusion, all factors in his obstructive sleep apnea syndrome. Nasal CPAP mask and tubing were devised from commercial CPAP kit (Respironic); engine was not (see text). Child can sleep on either side with this mask.
essor. When there was an absence or reduction in
recorded signals on the computer printout, we knew
that there had been a malfunction, and we recorded
the patient again within the week.
Each child had at least two in-hospital baseline
nocturnal monitonings and two in-hospital
moni-torings while on nasal CPAP. Patients in group A
usually had four to five successive nights of
moni-toring while on CPAP. In addition, patients in
group B had two nights of in-hospital monitoring
during which air pressure needed for effective
CPAP was determined and one night’s follow-up
monitoring with the Vitalog during the first month
of CPAP treatment and with Vitalog or polygraphy
at
irregular intervals thereafter, either in thehos-pital or at home.
Sleep was scored in 30-second observational
pe-nods according to the criteria of Rechtschaffen and
Kales8; these can be applied after 6 months of age.9
Because the international criteria do not take into
consideration brief (1 to 10 seconds) arousals that
can severely disrupt sleep, a special count was made
of these. Anousals were identified using EEG, EOG,
and EMG criteria, ie, appearance of a-rhythm or a
burst of 0 activity with increased EMG discharge,
regardless of the presence of slow eye movements.
STATISTICAL ANALYSIS
A two-tailed t test for pained observations was
performed on results of nocturnal recordings before
CPAP treatment and during CPAP administration
in the hospital.
CPAP EQUIPMENT
Only patients, 7, 8, and 10 have been fitted with
the commercially available CPAP kit (Sleep-EZ
and CPAP System, Respironics, Monroeville, PA).
Patient 9 uses a custom-made mask derived from
commercially available equipment (courtesy of
Res-pinonics) (Figure). The other six children were
fit-ted with homemade masks derived from the 1981
information of Sullivan et al.’ Patients 6 and 9 were
sent home with a different CPAP motor (Spencer
Vorter Blower VBOO1S, Thomas Air Supply, S San
Francisco, CA). Both motors provide air pressure
at a flow rate of 100 L/min. The homemade nasal
masks were made from an imprint obtained from
the child in a supine position. Initially, they were
made from a special plastic (sheet resin). As our
techniques improved, the best masks were made
using a dental alginate as a mold for a negative
imprint of the nose, which was encased with dental
plaster. A positive mold was then made using dental
stone. From this positive mold, a nasal CPAP mask
was built, using clean plastic (sheet resin, clean type,
for vacuum forming, Derby Co from Dental Supply,
Inc, Rockville Center, NY) and a STA-VAC
vac-uum machine (Statelite Dental Mfg Co, Inc,
Brook-lyn, NY). Masks made at the sleep center were
fitted tightly to the head using a strap, dental
cement, or Sialastic with catalyst (Dow-Corning),
which produced the best results. Pressures needed
to control the obstructive sleep apnea syndrome
were calculated using the procedure customary in
adults: while the patient slept, a valve was gradually
opened so that the air pressure delivered through
the nostrils progressively increased. The pressure
was measured using a Statham or a Grass pressure
transducer and recorded on a Grass polygraph
si-multaneously with the other sleep and respiratory
parameters. Pressures were increased until apneas
disappeared, and oxygen saturation values were
normal. Fine adjustments were made during the
early morning sleep cycles. The pressure used for
night 2 was the lowest that controlled the apneas
and provided normal oxygen saturation on night 1.
Nasal CPAP was administered before the lights
were turned out to prepare the child for the amount
of air pressure at sleep onset.
RESULTS
Group A (In-Hospital Treatment Only)
Although CPAP controlled the obstructive sleep
apnea syndrome, frequent air leakages during the
trial nights made it unfeasible as a continuous home
treatment. Results of baseline (night 2) and of the
are given in Table 2. The best night was the night
that had the fewest problems with leaks in the
mask and the least amount of sleep disturbance
caused by equipment and the research team’s
ma-nipulations. The sleep disturbances related to
tech-nical problems were so major in group A that it was
difficult to preselect a CPAP trial night to compare
with baseline results. The comparisons were made,
therefore, between baseline (the second control
night) and the night when the research team caused
the least sleep disturbance, resulting in the longest
sleep time.
Except for patient 2, who had the fewest
prob-lems, research personnel had to place the mask
back on each child’s face when problems with the
mask’s fitting permitted air leakage. Patient 2, our
oldest patient, had the most moderate syndrome
and almost normal sleep during two of four trial
nights.
Although we had difficulties ensuring a tight
mask and undisturbed sleep, a number of sleep
parameters improved when results from the best
night were compared with baseline: apnea and
hy-popnea (P < .005), number of episodes of
desatu-nation below 90% (P < .01), respiratory disturbance
index defined as the number of episodes of apnea
and hypopnea divided by total sleep time and
mul-tiplied by 60 (P < .005), total sleep time (P < .005),
percentage of REM sleep (P < .01), and number of
arousals (P < .001). Although we selected the night with the fewest technical difficulties, the percentage
of stage 1 NREM sleep was still abnormally high
because of difficulties with the CPAP equipment.
Despite the limitations of the equipment in these
earlier trials, the results from patients in group A
demonstrated that CPAP with a nasal mask could
be effective in treating obstructive sleep apnea
syn-drome in children. The pressure required to control
the obstruction was between 5 and 12 cm H2O,
similar to that required in adults. There was no
correlation between age and amount of pressure
needed. It was the severity of the syndrome and
amount of pressure that correlated. Patient 2, who
was 9 years of age, needed only 5 cm H2O, but
patient 5, who was 6 years of age, needed 12 H2O.
Two children had skin allergies to the dental
ce-ment that sealed the mask onto the face. The
technical problems encountered indicated the
masks and head gear had to be improved before
new trials were pursued.
Group B (In-Hospital and Home Trials)
There was an 18-month lapse between the
mon-itoning of the last patient in group A and the first
patient in group B. This group benefited from
im-provements in CPAP equipment for adults and
knowledge we gained from working with the
chil-dren in group A and adult patients. Because most
technical problems with the nasal mask had been
resolved, we were able to compare consistently
re-sults from baseline monitoring with results from
TABLE 2. Polygraphic Monitoring Bef
Airway Pressure Administration*
ore and Dur ing In- Hospital Co ntinuou s Positive
Patient No.
1 2 3 4 5
Baseline recording
Total No. apnea and hypopnea 250 78 257 297 318
No. of desaturations <90% 117 61 231 271 299
No. of desaturations <80% 58 0 21 49 33
RDI 35 12 41 48 47
TST (mm) 431 386 377 369 381
% REM sleep 12 17 15 6 12
% stage 1 NREM 22 9 29 17 28
CPAP
night (best of 5)Positive airway pressure (cm H2O) 10 5 7 10 12
Total No. apnea and hypopnea 10 0 38 41 32
No. of desaturations <90% 4 0 12 10 12
RDI 1.4 0 5.2 6 4
Time spent refitting mask 33 0 55 20 15
(mm)
TST (mm) 455 462 440 433 461
% REM sleep 19 22 26 17 16
% stage 1 NREM 17 12 8 12 15
* Abbreviations and definitions used are: CPAP, continuous positive airway pressure;
desaturation, patients 1 and 3 were monitored with transcutaneous Po2 and results are
the second night of CPAP administration. The
polygraphic results are presented in Table 3.
A two-tailed t test, performed on data obtained
on the last night before CPAP administration and
on the second in-hospital night, showed significant
improvement in the following: number of episodes
of apnea and hypopnea (P < .001), number of
desaturations below 90% (P < .001), respiratory
disturbance index (P < .001), percentage of REM
sleep (P < .025), and number of arousals from sleep
(P < .001). Total sleep time did not improve
signif-icantly by two-tailed t test, probably because the
number of subjects was too small, but percentage
of time spent in stage I NREM sleep (ie, very light
sleep) decreased significantly (P < .05) and
simul-taneously the percentage of time spent in 5 sleep
(stage III-IV NREM sleep) increased markedly (P
< .001), indicating that sleep structure was normal
in opposition to what was monitored on baseline
nights. In all of the in-hospital recordings of group
B patients using nasal CPAP, the number of apneic
episodes was similar to that reported in normal
children.’#{176} None of the children’s masks leaked.
Two children had to have their hands restrained
because they tried to pull their masks off at onset
of sleep. Parents’ intervention avoided this problem
in the other children. A skin rash developed in
patient 6 after night 2 and a mild nose bleed
oc-curred the following morning. We abandoned
CPAP treatment in this patient after 15 days
be-cause his parents did not cooperate in reassuring
the child and maintaining nasal CPAP all night
long. Consequently, the child often pulled off his
mask and a skin rash developed. The other four
patients have received CPAP at home on a nightly
basis for as many as 10 months.
Long-Term Follow-up: Some Technical Problems
Of the four children who continued CPAP
treat-ment, daytime napping has disappeared completely
and daytime activities have increased. None has
been rehospitalized for cardiorespiratory failure or
breathing-related problems. All have had good
health since chronic CPAP administration.
Be-cause no neuropsychometric testing or multiple
sleep latency tests have been performed, judgments
on degree of alertness and activity are based on
subjective evaluations made by parents and
teach-ers. Patient 9, who was obese, lost 4 kg and grew 8
cm in a 6-month period, probably the result of
greater activity and decreased somnolence. The
other three children have not exhibited such
appar-ent changes in height and weight.
There have been technical problems related to
the mask. Patient 10 has had problems with air
leakage. Because there is no alarm system to alert
parents to an equipment failure, they check him at
regular intervals throughout the night. This
tech-nical
deficiency
is being remedied. After a night ofair leakage, the eyes of patient 9 were very irritated.
During REM sleep, the facial muscles of patient 7
became atonic, and air leaked through the mouth.
This problem has been remedied by attaching a
TABLE 3. Polygraphic Monitoring Bef
Airway Pressure Administration*
ore and Dur ing In -Hospital Co ntinu ous Positive
Patient No.
6 7 8 9 10
Baseline recording
Total No. apnea and hypopnea 310 364 307 378 325
No. of desaturations <90% 276 358 279 371 304
No. of desaturations <80% 23 281 95 197 92
RDI 47.5 58 54 59 65
TST (mm) 391 375 341 382 299
% REM sleep 17 1 5 4 19
% stage 1 NREM 18 11 22 23 31
No. of short arousals 326 378 329 391 337
CPAP night
Positive airway pressure (cm H2O) 10 10 7 8 10
Total No. apnea and hypopnea 4 14 9 14 9
No. of desaturations <90% 0 0 0 2 4
RDI 0.5 3 1.4 2 1.5
TST (minutes) 461 341 380 401 362
% REM sleep 28 20 22 21 10
% stage 1 NREM 5 11 9 12 19
No. of short arousals 23 35 21 28 20
* Abbreviations and definitions used are: CPAP, continuous positive airway pressure; RDI,
respiratory disturbance index (number of episodes of apnea/hypopnea divided by total sleep time and multiplied by 60); TST, total sleep time; short arousal, an EEG arousal
loose chin strap to the mask. Another problem is
that cleaning the commercially available mask
re-petitively makes it brittle, and it must be replaced
after several months. Despite these problems, four
children, including the 3-year-old, have adapted to
nightly CPAP treatment with little trouble. CPAP
has remained consistently effective over time, and
we have not yet had to change the amount of air
pressure for any of the children.
COMMENTS
Obstructive sleep apnea syndrome often occurs
in children who are handicapped by both a
maxil-lofacial malformation and mental retardation.
Gen-erally, caretakers accept tracheostomy reluctantly
because it is a traumatic solution and also has its
share of complications. In young children, nasal
CPAP can be a valid therapeutic solution.
Unfor-tunately, there is no commercially available nasal
CPAP mask to fit over the noses of the children
younger than 7 years of age. The deformities of
skull and face in older children often require that
commercially available masks be adapted to the
child’s features. We had to make our own masks or
modify commercially available ones to some extent
for all of our patients. Follow-up respiratory
mon-itoning with ambulatory equipment provided useful
information on the type and frequency of problems
that occur with nasal CPAP treatment at home. All
problems resulted from a broken nasal seal. Similar
to findings in adults, rash and allergic skin
reac-tions occurred with dental cement and Sialastic;
eyes were irritated by leaks, regardless of the type
of mask. A humidifier can be attached to the CPAP
circuit but our patients never required it, although
it may have prevented the nose bleed of patient 6.
Parents’ cooperation is absolutely essential. Until
an alarm system is incorporated into the CPAP
circuit, there is the risk of an undetected leak
leading to apnea. Young children may have
diffi-culty wearing the nose mask and having air pushed
under pressure through the nose, particularly at
onset of sleep. Our patients had no problems with
this, but they tended to remove the mask
inadvert-ently if they woke during the night. The possibility
of mandibular relaxation during REM sleep-related
atonia can be remedied by fixing a loose chin strap
to
the harness on the mask. No respiratorycompli-cations related to nasal CPAP were noted in our
patient population. Although nasal CPAP may not
be successful in all young children, it may avoid a
number of tracheostomies, particularly in mentally
retarded children who are often more difficult to
treat than other children with obstructive sleep
apnea syndrome.
ACKNOWLEDGMENTS
We thank Cohn Sullivan, MD, and Kathy Phillips who
helped us develop better masks and techniques.
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