S L E E PM E D I C I N E

C L I N I C S

Sleep Med Clin 2 (2007) 445–462

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Sleep-Related Breathing Disordersof Childhood: Descriptionand Clinical Picture, Diagnosis,and Treatment ApproachesTimothy F. Hoban, MDa,*, Ronald D. Chervin, MD, MSb

- Clinical features of sleep-relatedbreathing disorders inchildren

- Physical features associated withchildhood sleep-related breathingdisorders

- Medical conditions associated withincreased risk for childhoodsleep-related breathing disorders

- Clinical and laboratory assessment ofchildren with suspected sleep-relatedbreathing disorders

- Polysomnography in children withsleep-related breathing disorders

- Supplemental respiratory monitoringtechniques

- Diagnostic criteria for childhoodsleep-related breathingdisorders

- Treatment of sleep-related breathingdisorders in children:adenotonsillectomy

- Treatment of sleep-related breathingdisorders in children: continuouspositive airway pressure

- Alternative surgical therapies forchildhood sleep-related breathingdisorders

- Dental and orthodontic therapies forchildhood sleep-related breathingdisorders

- Alternative medical therapies forchildhood sleep-related breathingdisorders

- Present needs and future directions forassessment and treatment of childhoodsleep-related breathing disorders

- References

Children, the victims of nasal and pharyngeal

obstructions, often suffer from headaches. andfrequently evince marked inability to fix their attention

on their lessons or work for any length of time.- William Hill, B.Sc., M.B.Lond., 1889 [1]

Perceptive clinical descriptions of sleep-relatedbreathing disorders (SRBDs) have been identified

1556-407X/07/$ – see front matter ª 2007 Elsevier Inc. All righsleep.theclinics.com

in medical journals and textbooks as early as thelate nineteenth century [2]. Contemporary des-criptions of the condition begin with the report ofGuilleminault and colleagues [3] of eight childrenwith obstructive sleep apnea (OSA) accompaniedby additional symptoms, such as hypertension, hy-peractivity, and daytime somnolence. Subsequent

a The Michael S. Aldrich Sleep Disorders Laboratory, Departments of Pediatrics and Neurology, University ofMichigan, L3227 Women’s Hospital, 1500 East Medical Center Drive, Ann Arbor, MI 48109–0203, USAb The Michael S. Aldrich Sleep Disorders Laboratory, Department of Neurology, University of Michigan, C728Med Inn, Box 0845, 1500 East Medical Center Drive, Ann Arbor, MI 48109–0845, USA* Corresponding author.E-mail address: thoban@umich.edu (T.F. Hoban).

ts reserved. doi:10.1016/j.jsmc.2007.05.006

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reports have contributed to present understandingthat the clinical manifestations and treatments forSRBDs are substantially different for children com-pared with adults. This article addresses clinicalaspects of SRBDs in children, with particular focuson sleep and daytime symptoms, polysomnogra-phy (PSG), and options for surgical and medicaltreatment. OSA is examined in greatest detail, butupper airway resistance syndrome (UARS) andsleep-related hypoventilation are also considered.

Clinical features of sleep-related breathingdisorders in children

Snoring represents one of the cardinal symptomsof obstructive SRBDs in children, and may beobserved in the context of OSA, UARS, and primarysnoring (Table 1). Children with upper airwayobstruction are usually noisy breathers duringsleep, but severity may range from minimallyloud breathing to heroic snoring. Snoring mayvary with body position during sleep and com-monly worsens during respiratory infections or ex-acerbations of allergic rhinitis. It is not unusualfor snoring to be accompanied by mouth breathingor neck hyperextension during sleep, which arethought to represent compensatory mechanismsthat may improve airway patency during periodsof partial obstruction.

The prevalence of habitual snoring has been esti-mated to be 10% to 12% in most studies of youngerschool-age children and was reported to be 6% ina community-based urban cohort of United Statesadolescents [4–8]. Prevalence is highest betweenages 2 and 8 years, coinciding with peak adenoton-sillar hypertrophy relative to upper airway size [9].Obesity, nasal obstruction, craniofacial abnormali-ties, and passive exposure to tobacco smoke mayrepresent independent risk factors for the develop-ment of snoring in childhood [10].

Witnessed apneas during sleep are less frequentlyreported for children than for adults. This is consis-tent with evidence that chronic partial airway ob-struction without arousal is more frequent thanintermittent apneic obstruction for many childrenwith SRBDs [9]. Witnessed apneas were reportedfor 3.4% of 5 year olds in a large, population-basedsurvey of children in eastern Massachusetts, but thedegree to which witnessed apneas are associatedwith clinically significant SRBDs remains uncertain.

In contrast to the noisy respiration exhibited bychildren with OSA and UARS, children with nonob-structive SRBDs tend to be quiet breathers whose re-spiratory symptoms are usually less prominentduring sleep. Children with neuromuscular disor-ders causing hypoventilation without obstructionmay exhibit tachypnea or shallow respiration that

may not be obvious to family members. The lackof easily recognizable respiratory symptoms duringthe sleep of these children may lead to delays indiagnosis and treatment, particularly if daytimesymptoms are mild or misattributed to othercauses.

Children with SRBDs may exhibit a variety ofnonrespiratory symptoms during sleep. Childrenwith OSA exhibit greater degrees of restlessness, per-spiration, and waking during sleep compared withcontrols [11]. Nocturnal enuresis is also overrepre-sented in children with OSA, reported in 42(29%) of 144 children with PSG-confirmed OSAin one case series. Nighttime bedwetting resolvedwithin 1 month for 11 (41%) of the 27 enuretic chil-dren treated with adenotonsillectomy (AT) amongthis group [12]. Parasomnias, such as sleepwalking,sleeptalking, and night terrors, also are more fre-quent among children with SRBDs than amongother children, and may respond to AT [13,14].

Daytime symptoms of childhood SRBDs are var-ied. On awakening, affected children sometimescomplain of transient dry mouth, sore throat, orheadache. Grogginess or tremulousness on morn-ing waking may also be disproportionate relativeto that expected for a child’s age and sleep duration.Mouth breathing during wakefulness is quite com-mon in children with obstructive SRBDs, parti-cularly when adenoidal hypertrophy or othersignificant nasal obstruction is present [11].

Daytime somnolence is seldom the most obvioussymptom for prepubertal children with SRBDs un-less the underlying respiratory disturbance is severeor unless other influences, such as insufficient sleep,are concurrently present. Careful questioning offamilies, however, reveals subjective sleepinessmore than three times more often in childrenwith SRBDs than in control subjects [15]. Sleepi-ness often manifests in a subtle or intermittent fash-ion during sedentary activities, such as automobilerides. Subjective impressions of daytime sleepiness,even if not obvious in many instances, are con-firmed by objective Multiple Sleep Latency Teststhat show modest reductions in mean sleep latencyfor children with OSA [16,17]. These data suggestthat some degree of daytime sleepiness is probablypresent in many children with SRBDs, but that thissleepiness may be expressed in the form of otherneurobehavioral symptoms, and perhaps obscuredby those other more prominent symptoms. As suc-cinctly summarized by Stores [18], ‘‘sleepiness cantake the form of an increase rather than reductionof activity’’ in children.

Neurobehavioral deficits in children with SRBDsmay involve learning, attention, and behavior tovariable degrees [19,20]. Early reports of childhoodOSA, which documented relatively severe cases,

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Table 1: Clinical features of sleep-related breathing disorders across the lifespan

Childhood Adolescence Adulthood

Physical characteristicsGender Male 5 female ? Male > female Male > femalePeak age 2–8 y Not identified 40–60 yearsBody weight Usually normal Obese > normal Obese > normalRisk factors Adenotonsillar

hypertrophyCraniofacialabnormalitiesObesity

ObesityAdenotonsillarhypertrophyCraniofacialabnormalities

ObesityMale sex

Symptoms during sleepBreathing Snoring often

continuousMouth breathing

Snoring withor without pausesMouth breathing

Snoring withwitnessed pauses

Other Unusual sleepingpositionsRestlessnessPerspiration

RestlessnessPerspiration

RestlessnessNocturia

Secondary symptomsSleepiness Frequently present

on careful inquirybut often obscuredby more problematicbehavior

More common Very common

Neurobehavioral HyperactivityInattentionBehavioraldisturbances

InattentionHyperactivityBehavioraldisturbances

Cognitive impairmentIncreased crash risk

Cardiovascular HypertensionRare cor pulmonale

HypertensionRare cor pulmonale

Frequent hypertension[ risk of strokeor heart disease

Pertinent associated findingsMode of obstruction Prolonged >

intermittentProlonged orintermittent

Intermittent >prolonged

Arousal withobstruction

Less frequent Variable More frequent

Sleep architecture Normal > fragmented Normal to fragmented Fragmented > normal

identified high frequencies of associated hyperactiv-ity, school problems, and behavioral disturbances[3,21,22]. More recent data suggest that less severechildhood OSA is also associated with cognitiveand behavioral sequelae, particularly in the do-mains of attention and executive function [23,24].Neurobehavioral problems have also been reportedin children who have primary snoring, withoutOSA [25–27]. Hyperactive behavior may be partic-ularly common when SRBDs affect young children(eg, those 8 years old or younger) [28]. Neurobeha-vioral sequelae of childhood SRBDs are reviewed indetail elsewhere in this issue.

Physical features associated with childhoodsleep-related breathing disorders

Characteristic physical features, such as tonsillarhypertrophy, are frequently but not universally

identified in children with obstructive SRBDs.Other physical findings that may predispose toupper airway obstruction include retrognathia,malocclusion, nasal septal deviation, narrow orhigh-arched palate, and macroglossia. MRI studieshave identified larger soft palate size in childrenwith OSA compared with controls, consistent withexamination findings of long soft palate or highMallampati scores for some affected children[29,30]. Craniofacial anomalies, such as midfaceor mandibular hypoplasia, may be apparent forsome children with SRBDs. Although adenoidalsize can usually not be assessed during examinationwithout endoscopic visualization, some childrenwith adenoidal hypertrophy manifest adenoid fa-cies: mouth breathing, pinched nose, and elongatedfacial appearance.

Early accounts of childhood OSA reported highrates of clinical cor pulmonale, which reflected

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severe cases in an era when awareness and treat-ment of the condition were extremely limited[21,22]. Most children with SRBDs of mild or mod-erate severity have normal cardiovascular examina-tions. Hypertension during wakefulness has beenreported for some children with SRBDs, with lim-ited evidence suggesting that this finding may beinfluenced by severity of sleep-disordered breathing[31,32].

Body weight and body mass index is most oftennormal in school-age children with SRBDs, incontrast to the high prevalence of obesity amongaffected adults. Obesity nevertheless representsa clearly identifiable risk factor in at least somepediatric cases [33], and the number of such casesmay be increasing as obesity reaches endemicproportions in the United States. Risk for SRBDsseems to be highest for children and adolescentswith severe obesity. Among 34 adolescents withmean body mass index of 57 � 10 kg/m2 undergo-ing gastric bypass surgery, 19 (55%) demonstratedan apnea-hypopnea index (AHI) R5 on preopera-tive PSG [34]. Marcus and colleagues [35] reportedabnormal PSGs for 10 (46%) of 22 morbidly obesechildren who presented without sleep or respiratorycomplaints.

Children with SRBDs may occasionally presentwith low body weight, sometimes associated withdecreased somatic growth or frank failure-to-thrive[22,36]. It is postulated that increased work ofbreathing may be one of the mechanisms responsi-ble for this association, because improved growthand weight gain has been demonstrated followingAT even when caloric intake is unchanged [37].

Medical conditions associatedwith increased risk for childhoodsleep-related breathing disorders

A variety of medical, craniofacial, and genetic disor-ders are associated with increased risk for SRBDs(Box 1), but systematic study has been undertakenfor only a few conditions. SRBDs are thought to bevery common in children with Down syndrome,where case series have reported prevalence ratesranging from 31% to 57% [38–40]. SRBDs arethought to be common among children withPrader-Willi syndrome despite widely varied fre-quencies reported by the small case series in existingmedical literature [41]. The degree to which the in-creasing use of human growth hormone in childrenwith Prader-Willi syndrome might impact risk fordevelopment of SRBDs remains uncertain. Among25 children and young adults with Prader-Willi syn-drome who were assessed before and after 6 weeksof growth hormone therapy, AHI improved for 19patients and worsened for 6 patients [42]. Two

adolescents in this series were reassessed following6 months of growth hormone treatment. Both dem-onstrated improvements from pretreatment AHI,but no other outcome data are available on long-term impact of growth hormone use in thispopulation.

Many children with craniofacial disorders alsohave high risk for SRBDs, particularly when man-dibular or maxillary hypoplasia is present. A pro-spective study of 21 infants with Pierre Robinsyndrome identified 18 (86%) with significantOSA or desaturation [43]. Although cleft palate it-self provides a widely patent airway, case serieshave reported SRBDs in children following surgicalrepair of cleft palate [44,45].

Clinical and laboratory assessmentof children with suspected sleep-relatedbreathing disorders

Clinical assessment of the child with a suspectedSRBD should include a detailed history and physi-cal examination, as outlined in Table 2. Inaddition, the clinician should ask about symptomsof other common pediatric conditions that canmimic OSA to some extent, or accompany it (eg, pe-riodic limb movement disorder, gastroesophagealreflux, or nocturnal exacerbation of asthma).

Box 1: Conditions associated with increasedrisk for SRBDs

Craniofacial syndromes featuring prominentmaxillary or mandibular hypoplasiaApert’s syndromeCrouzon’s diseasePierre Robin syndromeSaethre-Chotzen syndromeTreacher Collins syndromeOther skeletal and craniofacial disordersAchondroplasiaChoanal atresiaCleft palate, especially following surgicalrepairVelocardiofacial syndromeSystemic genetic and metabolic disordersDown syndromeHypothyroidismMucopolysaccharide storage disorders (eg,Hunter’s syndrome, Hurler’s syndromes)Prader-Willi syndromeOther medical and neurologic conditionsBrainstem and cranial nerve disorders: syringo-bulbia, Chiari’s malformationChronic nasal obstruction: septal deviation,allergic rhinitis, polyp, infectionsObesitySickle cell disease

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Table 2: Clinical assessment of children with suspected sleep-related breathing disorders

Sleep history Snoring: volume, character, changes over timeMouth breathing, increased work of breathingUnusual sleeping positions (eg, neck hyperextension, propping on pillows)Restlessness, limb movements, excessive sweating, use of a fan at nightSleep schedule and duration, including night wakingEnuresis and parasomniaFamily history of SRBDs or other sleep disorders

Daytime symptoms Mouth breathing during wakefulnessSymptoms on waking: grogginess, headache, sore throat, dry mouthBehavior: irritability, distractibility, hyperkinesis, temperamental behaviorSchool: inattention, poor academic performance, aggression toward peersSleepiness, especially in sedentary situations (eg, automobile rides)

Medical history ENT: adenotonsillar hypertrophy, allergic rhinitis, known anatomicabnormalitiesEndocrine: obesity, delayed growth, thyroid diseaseCardiovascular: hypertension, pulmonary hypertension, congenital heartdiseasePulmonary: asthma, other intrinsic lung diseaseNeurologic: disorders affecting brainstem, cranial nerves, neuromuscularfunction, peripheral nerves, or autonomic functionDevelopment: developmental delay, failure-to-thriveOther: craniofacial disorders, genetic syndromes

Physical examination Vital signs: weight, height, body mass index, blood pressure, percentile ranksOropharynx: tonsillar size, palate, dentition, occlusion, tongue, MallampatiscoreNasopharynx: polyps, septal deviation, airflow, ‘‘pinched-nose’’ appearanceCraniofacial: micrognathia, midface hypoplasia, cleft palate repair orcraniofacial syndromeNeck: thyroid, masses, circumferenceThorax: cardiac auscultation (especially split S2), lung auscultation, evidenceof scoliosisNeurologic: cranial nerve palsies, muscle weakness, peripheral neuropathyBehavior: attention deficit, hyperkinesis, irritability, or sleepinessOther: obligate mouth breathing, noisy breathing, ‘‘adenoid facies’’

Although a good history and physical exam-ination, followed by PSG, represent standarddiagnostic approaches, alternative methods of as-sessment may be less time consuming, less expen-sive, and appropriate in some circumstances.Several questionnaires have been investigated forclinical and research use in children with sus-pected SRBDs. The Pediatric Sleep Questionnairecontains a SRBD scale that is reasonably sensitiveand specific [46]. This instrument is commonlyused in research settings, but does not replicatePSG results closely enough to substitute for labo-ratory testing in clinical settings. Few studies,however, have compared questionnaires and thestandard PSG on the basis of their ability to pre-dict childhood health outcomes. In one study thatdid, the Pediatric Sleep Questionnaire performedas well as or better than a standard PSG in pre-diction of baseline neurobehavioral problemsand their improvement after AT [47]. Otherquestionnaire measures used in the evaluation of

children with SRBDs include the Sleep Dis-turbance Scale for Children, which has a scalefor sleep breathing disorders; the OSA-18, whichassesses quality of life; and a modified EpworthSleepiness Scale [48–50]. Among 108 childrenwith suspected SRBDs, modified Epworth Sleepi-ness Scale scores were higher than for controlsubjects, but the questionnaire did not reliablydistinguish between children with OSA and thosewith primary snoring [49].

Although home audio recordings of a child’ssnoring were previously advocated as a simpleand inexpensive screening measure, this techniquedoes not reliably distinguish SRBDs from primarysnoring [51,52]. Likewise, overnight oximetry haspoor sensitivity as a screening measure for SRBDsin children. Among 210 children with PSG-documented OSA, 120 (57%) were found to havenormal or inconclusive nocturnal oximetry studies,vividly illustrating that normal oximetry does notrule out underlying SRBDs in children [53].

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Polysomnography in childrenwith sleep-related breathing disorders

Laboratory-based PSG remains the gold standardfor the diagnosis and classification of SRBDs inchildren. Advantages of PSG include the fact thatthe test represents a standardized and objectivemeasure of multiple physiologic parameters. Thetest can be adapted for use in children of all ages.The PSG recording montages can be readily custom-ized to include supplemental monitoring of end-tidal carbon dioxide (ETCO2), esophageal pressure,or other physiologic variables when clinical circum-stances warrant. Pediatric PSG also has several lim-itations, including the fact that the test is lengthy,labor-intensive, and potentially stressful for youn-ger or developmentally disabled children. Addition-ally, not all sleep laboratories have sufficient staff ortechnical expertise for studies involving small chil-dren. Finally, standard PSG is not always sensitivefor the detection of the prolonged partial airwayobstruction frequently encountered in childhoodSRBDs unless supplemental monitoring, such asesophageal pressure or pulse transit time, are used(technology that is presently not available inmany sleep laboratories).

A PSG should be considered whenever a child’ssymptoms, physical examination, and risk factorssuggest that a SRBD may be present. Practiceparameters that address guidelines for the use ofPSG in children have been published by both theAmerican Thoracic Society and the American Acad-emy of Pediatrics [54,55]. The issue of whether chil-dren with suspected SRBDs should receive empiricAT without PSG assessment has been vigorouslydebated without resolution [56,57]. Until out-come-based research more clearly defines an opti-mal clinical pathway, the authors’ practice is touse the same standards used for adults: to docu-ment the presence and severity of an SRBD byPSG before decisions are made with respect to treat-ment. In light of recent evidence that SRBDs maypersist after AT more commonly than previouslythought [30,58,59], perhaps because of increasedcontributions from obesity, PSG also can play animportant role after surgery.

Standard PSG in children is performed ina manner analogous to adult studies, using centraland occipital electroencephalogram channels, elec-tro-oculogram, ECG, and electromyogram of thechin and extremities. Frontal electroencephalogramleads are now used in some laboratories for im-proved detection of cortical arousals [60]. Standardrespiratory monitoring includes nasal and oralairflow, thoracic and abdominal movement, andpulse oximetry. Many laboratories routinely addnasal pressure monitoring for improved sensitivity,

in comparison with thermistors or thermocouples,in the detection of flow limitation or subtledecrements in flow [61]. Capnography and time-averaged ETCO2 is often added during studies ofchildren who have neuromuscular disorders orother risk factors for hypoventilation.

Scoring of sleep stages and arousals for pediatricPSG is performed using 30-second epochs andstandard diagnostic criteria in the same manner asadult studies [62,63]. There are presently no univer-sally accepted standards for scoring and interpreta-tion of respiratory events on pediatric studies,although revised scoring rules are expected in2007. Most centers performing pediatric studiesscore obstructive apneas (Fig. 1) when cessationof airflow exceeds the duration of two respiratorycycle lengths. Criteria for scoring hypopneas(Fig. 2) vary considerably among sleep laboratories.Scoring criteria used at the University of Michiganrequire a reduction in airflow or effort of 20% to80% for at least two respiratory cycle lengths fol-lowed by arousal, awakening, or oxygen desatura-tion of at least 4%.

Interpretation of ETCO2 during PSG (Fig. 3) isinfluenced by the fact that abnormal values seldomoccur as discrete, scoreable events and more com-monly present as prolonged and often gradualelevations that cannot be tabulated by the samescoring process used for apneas and hypopneas.Proposed pediatric criteria require that peakETCO2 for the night exceed 53 mm Hg or thatETCO2 exceed 50 mm Hg for over 10% of total sleeptime for a diagnosis of hypoventilation to beestablished [64].

Supplemental respiratory monitoringtechniques

Standard PSG techniques that rely primarily ondetection of oral-nasal flow may not reliably detectperiods of prolonged partial airway obstruction inthe absence of desaturation or discrete apneas andhypopneas. Because prolonged obstruction ismore frequent than intermittent obstruction forsome childhood SRBDs (eg, UARS, obstructivehypoventilation), some laboratories use specializedrecording techniques designed to improve the sen-sitivity of PSG for nonapneic airway obstruction.Esophageal pressure monitoring (Fig. 4) representsthe most well-established of these techniques.Although esophageal pressure is minimally invasive(requiring insertion of a thin catheter through thenasopharynx into the esophagus), the techniquehas minimal impact on children’s sleep quality, isgenerally well tolerated by children in clinical set-tings, and remains the most accurate quantitative

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Fig. 1. (A) Prolonged obstructive apnea (60-second epoch). The apnea is characterized by cessation of airflow onboth oral-nasal and nasal pressure leads and progressive oxygen desaturation to below 70%. The event is accom-panied by paradoxical respiratory effort and development of progressive bradycardia with a nonconducted P waveat the end of the event. (B) Brief obstructive apnea (60-second epoch). The apneic event lasts slightly greater thantwo respiratory cycle lengths in duration and is not accompanied by desaturation or arousal from sleep.

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Fig. 2. Hypopnea (60-second epoch). Hypopnea is characterized by subtle decrement of oral-nasal flow anddevelopment of paradoxical respiratory effort followed by arousal but not desaturation.

method available for assessment of increased upperairway resistance [65,66].

Although some have proposed that nasalpressure can substitute for esophageal pressuremonitoring in adults, few comparative data areavailable for children, and studies of nasal pressurein this age group have shown substantial failurerates during nocturnal PSG [67,68]. Other tech-niques for assessment of nonapneic upper airwayobstruction also remain largely investigational.Pulse transit time, which provides beat-to-beatassessment of changes in blood pressure and vas-cular tone, has been found to correlate well withesophageal pressure fluctuations during obstructiveevents and to represent a sensitive marker forarousal [69,70]. Computer analysis of respiratorycycle-related electroencephalogram changes hasidentified subtle breath-to-breath electroencepha-logram changes during nonapneic sleep of childrenwith SRBDs, which may represent a marker of inspi-ratory microarousal [71]. Data from childrenwith SRBDs and controls suggest that respiratorycycle-related electroencephalogram changes maybe more useful than the AHI in predicting eitherobjective or subjective daytime sleepiness, andshow that respiratory cycle-related electroencepha-logram changes improve after treatment for OSA[15,72]. Another promising approach to improve

understanding of pediatric SRBDs focuses on theratio of respiratory-related arousals to non–respira-tory-related arousals [73]. Other investigationalPSG techniques for detection of upper airwayobstruction and arousal include transcutaneousdiaphragmatic electromyogram analysis, respira-tory inductance plethysmography, and peripheralarterial tonometry [74–76].

Ancillary diagnostic testing beyond PSG is usedselectively in the work-up of children with sus-pected SRBDs. When daytime sleepiness representsa prominent symptom, the Multiple Sleep LatencyTest provides objective and quantitative assessmentwhen interpreted using pediatric norms [77,78].Children presenting with prominent neurobeha-vioral symptoms usually benefit from appropriateclinical evaluation and treatment of these prob-lems. Children suspected to suffer from a severeSRBD sometimes require additional cardiopulmo-nary evaluation including ECG, echocardiogram,and chest radiographs.

Diagnostic criteria for childhoodsleep-related breathing disorders

Criteria for the diagnosis of SRBDs are less well-established for children than for adults. It has longbeen known that more than one obstructive apnea

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Fig. 3. Capnography and end-tidal CO2. This 60-second epoch demonstrates a brief period of obstructivehypoventilation, during which snoring and partial airway obstruction are accompanied by mild hypercapnia.No desaturations, apneas, or hypopneas are evident during the period of hypercapnia.

per hour of sleep is statistically abnormal amonghealthy, asymptomatic children [79]. It is also gen-erally accepted that children with clinically signifi-cant SRBDs are often found on PSG to have AHIsthat are normal by adult standards (ie, less thanfive events per hour). Research studies have oftenchosen an AHI threshold of 1.5 to 5. Existingresearch, however, remains insufficient to specifya confident threshold at which AHI or other PSGparameters become associated with clinically signi-ficant health consequences for children.

In recognition of this, the current InternationalClassification of Sleep Disorders, Second Edition(ICSD-2) guidelines for the diagnosis of pediatricOSA incorporate both clinical and PSG criteria, out-lined in Box 2 [80]. Although these criteria havehelped provide a more formal and cohesive frame-work for the diagnosis of OSA in children, theICSD-2 criteria for SRBDs also have several impor-tant limitations in the pediatric population.

First, ICSD-2 criteria for the diagnosis of pediatricOSA require an AHI of at least one scoreable respi-ratory event per hour. Although this is less than theAHI required for the diagnosis of OSA in adults,ICSD-2 does not specify pediatric criteria forUARS and other SRBDs where the AHI may be

less than 1. Proposed pediatric criteria for UARShave been published elsewhere [81].

Second, ICSD-2 does not specify pediatric criteriafor hypoventilation except for congenital central al-veolar hypoventilation syndrome, a rare geneticallymediated cause of hypoventilation during sleepthat usually presents during infancy [80,82]. Giventhat clinically significant hypoventilation duringsleep is common in children with neuromusculardisorders and other predisposing conditions,many sleep laboratories continue to use previouslyestablished criteria for the diagnosis of hypoventila-tion in children: ETCO2 > 45 mm Hg for more than60% of total sleep time, ETCO2 > 50 mm Hg formore than 10% of total sleep time, or peakETCO2 > 53 mm Hg [64].

Treatment of sleep-related breathingdisorders in children: adenotonsillectomy

AT represents the most frequently used treatmentfor obstructive SRBDs in children. The procedureis thought to be effective in alleviating upper airwayobstruction for many symptomatic children, butclinical research assessing the frequency with whichthe procedure ‘‘cures’’ SRBDs has yielded highly

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Fig. 4. Esophageal pressure monitoring. This 60-second epoch demonstrates increased nonapneic partial airwayobstruction in a child with upper airway resistance syndrome. The esophageal pressure tracing (labeled ExPes)demonstrates excessive peak-to-trough fluctuations ranging from �30 to �50 cm H2O (normal is about 0 – �10).

variable results. Early case series reported high re-sponse rates exceeding 70%, but these studieswere limited by substantial variability in the areasof patient selection, outcome measures, and PSGcriteria used to define the presence or absence ofOSA [59,83,84]. Clinical practice guidelines issuedby the American Academy of Pediatrics in 2002regarding diagnosis and management of OSA esti-mated that AT was associated with PSG resolutionof OSA in 75% to 100% of otherwise healthy chil-dren undergoing the procedure [55]. Similarly,a meta-analysis of published research regardingthe effectiveness of AT in the treatment of pediatricOSA calculated a summary success rate of 82.9%using a random effects model [85].

Several large, recent case series have reportedsomewhat lower success rates for AT. Tauman andcolleagues [58] assessed 100 children with OSA(mean obstructive AHI 5 22.3 � 28.9; 52% obesesubjects) before and after AT. Although the meanpostoperative AHI declined to 5.6 � 9.3, completenormalization of AHI (ie, AHI % 1) was observedin only 25% of subjects. Guilleminault and col-leagues [30] also reported relatively high rates ofpersistent PSG abnormalities in a recent prospectiveseries, where 94 (47%) of 199 subjects

demonstrated abnormal PSGs postoperatively.These and other reports suggest that children whoare obese, have high Mallampati scores, and haveother anatomic abnormalities of the upper airwaymay be at greatest risk for persistent sleep-disor-dered breathing following AT [86].

Despite the variability of these data and uncer-tainty regarding the impact of increasing rates forchildhood obesity, present research suggests thatchildhood SRBDs persist following AT at sufficientfrequency to warrant clinical follow-up at mini-mum, and laboratory-based PSGs with adequatelysensitive recording techniques in a significant pro-portion of cases. Postoperative PSG is always indi-cated for children with residual symptomsfollowing surgery and for those children whose pre-operative disease was severe. Postoperative PSGshould be considered in obese children. Clinicalvigilance as the child matures may also be appropri-ate in light of several reports of late recurrencefor OSA in children treated with AT during earlychildhood [87,88].

Data regarding impact of AT on non-PSG mea-sures are limited. Several case series have reportedpostoperative improvements in measures of atten-tion, behavior, sleepiness, and quality of life [17,89,

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90]. Postoperative improvements have also beenreported for somatic growth parameters, serum in-sulin-like growth factor-1 levels, and nocturnalenuresis [12,37,91,92].

Potential clinical benefits of AT in children withSRBDs should be considered with due attentionto the risks and side effects of the procedure. Post-operative hemorrhage has been reported in up to

Box 2: ICSD-2 criteria for pediatric OSA

1. Parent or caregiver report of snoring,labored breathing, or other obstructivesymptoms during sleep

2. Parent or caregiver report of at least oneassociated symptom

Paradoxical chest wall motion duringinspirationMovement arousalsExcessive perspirationUnusual sleeping positions (eg, neckhyperextension)Daytime symptoms: hyperactivity, aggres-sive behavior, or sleepinessImpaired growthHeadache on wakingSecondary enuresis

3. PSG documents at least one scoreablerespiratory event lasting at least two respi-ratory cycles in duration per hour of sleep(criteria subject to revision as additionaldata become available)

4. PSG demonstrates either

At least one of the following findings:

i. Frequent arousals associated withincreased respiratory effort

ii. Oxygen desaturation with apneicevents

iii. Hypercapnia in sleepiv. Excessively negative esophageal pres-

sure fluctuations

Periods of abnormal gas exchange duringsleep (hypoxemia, hypercapnia, or both)with snoring, paradoxical chest wallmotion, and at least one of the following:

i. Frequent arousals during sleepii. Excessively negative esophageal pres-

sures swings

5. The disorder is not better explained byother sleep disorders, medical or neurologicconditions, or by medication or substanceuse

From American Academy of Sleep Medicine. Theinternational classification of sleep disorders. 2ndedition. Westchester (IL): American Academy ofSleep Medicine; 2005; with permission.

8% of treated children [93]. Postoperative respira-tory complications have been reported in up to36% of routine cases and 60% of urgently per-formed cases [94,95]. Major risk factors for postop-erative respiratory complications in childrenwith SRBDs include young age (<2–3 years); highpreoperative AHI; low nadir oxygen saturation(<70%–80%); and presence of concurrent medicalproblems or craniofacial disorders [94,96–101].Late complications of AT may include nasopharyn-geal stenosis and velopharygeal incompetence [9].

Treatment of sleep-related breathingdisorders in children: continuous positiveairway pressure

Nasal continuous positive airway pressure (CPAP)represents the most common nonsurgical treatmentfor childhood SRBDs. This treatment is used lessfrequently than AT for children with prominent ad-enotonsillar hypertrophy, but is the most commontreatment in children who are not good surgicalcandidates; in children whose SRBD persists despiteAT; and in children with nonobstructive SRBDs,such as hypoventilation. CPAP in children may becustomized in several ways based on the clinicalcircumstances of individual patients. A variety of in-terface options are available, including nasal masks,full face masks, and nasal pillows. Use of bilevelCPAP may improve ventilatory parameters in chil-dren with hypoventilation and in those who donot comfortably tolerate CPAP.

Effective use of CPAP for the treatment ofchildhood OSA has been reported in several largecase series with success rates ranging from 74% to97% [102–107]. Side effects, such as mask leak,skin irritation, and pressure sores, are generallymild, self-limited, and easily addressed by adjustingor refitting the interface. Nasal dryness or conges-tion can be addressed with use of a heated humid-ifier. Serious short-term side effects are uncommonapart from rare reports of pneumothorax in chil-dren with underlying neuromuscular disorders[108,109]. The development of midface hypoplasiafollowing prolonged use of nasal CPAP has beenreported as a rare long-term complication [110].

Data regarding use of CPAP and bilevel CPAP inthe treatment of nonobstructive SRBDs are ex-tremely limited, but successful use of bilevel CPAPhas been reported in small case series of childrenwith sleep-related hypoventilation caused by con-genital central alveolar hypoventilation syndromeand Duchenne’s muscular dystrophy [111–113].

A child’s compliance with long-term CPAP ther-apy may be influenced by several factors. Childrenwho are old enough to perceive improvement of

Hoban & Chervin456

symptoms on treatment or who otherwise recog-nize and accept the importance of therapy oftenachieve consistent long-term use, particularlywhen appropriate parental support is present. In-fants and developmentally disabled children whoare physically unable to remove the CPAP interfacemay also successfully entrain to long-term use onceacclimated. Compliance is often most problematicin younger children and developmentally disabledchildren who do not understand the importanceof treatment and who are able independently to re-move their interface. Sustained parental effort andimplementation of behaviorally based desensitiza-tion programs are sometimes helpful in improvingcompliance for children whose tolerance of CPAP isinitially poor.

Alternative surgical therapies for childhoodsleep-related breathing disorders

Other surgical treatments may be considered forchildren with severe obstructive SRBDs that arerefractory to AT, CPAP, and other conventionaltherapies. Procedures that remove or reduce non-lymphoid tissue of the upper airway are sometimeseffective in alleviating obstruction, but have re-ceived limited study in the pediatric age group.Septoplasty and turbinectomy may improve nasalairflow in older children with significant nonade-noidal obstruction. Uvulopalatopharyngoplasty issometimes performed in children with highMallampati scores or redundant lateral pharyngealtissue. Several small case series have reported clini-cal improvement in children with Down syndromeand other developmental disabilities treated withuvulopalatopharyngoplasty, but surgical outcomein otherwise healthy children has not been assessed[114–117]. Other soft tissue reduction procedures,such as lingual tonsillectomy and radiofrequencyablation of the tongue base or soft palate, are onlyrarely undertaken in children.

For children with craniofacial disorders or non-syndromic hypoplasia of the maxilla or mandible,surgical procedures involving the bony structuresof the upper airway are sometimes undertaken. Ina group of five children whose severe micrognathiahad been previously treated with mandibular dis-traction surgery, significant OSA was identified inonly one case [118]. Additional case reports havedocumented improvement in SRBDs for childrenfollowing treatment with mandibular distrac-tion and with mandibular midline osteotomy[119,120]. Maxillary advancement procedures aresometimes performed in children with significantmaxillary hypoplasia, but impact of the procedureon associated SRBDs has not been rigorouslyassessed.

Tracheostomy is only undertaken when a child’sSRBD is severe and refractory to less drastic formsof therapy. The procedure is highly effective foralleviation of upper airway obstruction, but adverseimpact on quality of life has been reported byseveral investigators [21,121,122]. The procedurealso necessitates ongoing stoma care and risk forbacterial colonization and infection. Because ofthese significant tradeoffs, aggressive use of alterna-tive surgical techniques has been advocated [123].

Dental and orthodontic therapiesfor childhood sleep-related breathingdisorders

Oral appliances have not achieved widespread usein the treatment of childhood SRBDs becauseof concern that this treatment might have thepotential to cause orthodontic problems. This con-cern notwithstanding, two small case series havereported improvements in AHI for children withSRBDs following 6 months of treatment with anoral appliance [124,125]. Rapid maxillary expan-sion is also being investigated as a possible treat-ment for SRBDs in children with narrow upperjaws. The procedure involves a fixed oral appliancethat is gradually expanded to open the midpalatalsuture with the goal widening the maxilla andmaintaining it in this position until the suturereossifies. In a group of 31 children with OSA andnarrow maxillas but no adenoidal hypertrophy,rapid maxillary expansion resulted in a reductionof the mean AHI from 12.2 to 0.4 events per hour,with all treated subjects having a posttreatment AHIof less than 1 [126].

Alternative medical therapies for childhoodsleep-related breathing disorders

Supplemental oxygen is sometimes used in thetreatment of primary sleep apnea of infancy, butis seldom used as sole therapy for older childrenwith SRBDs in the absence of concurrent cardio-pulmonary disease. Marcus and colleagues [127]reported that use of supplemental oxygen at 1 literper minute in 23 children with OSA resulted inmodestly higher mean and nadir oxygen satura-tions with no effect on apnea duration or frequency.Two patients in this double-blinded crossover studyexhibited hypercapnia during treatment with oxy-gen. Although ETCO2 levels remained normal forthe group as a whole during treatment, the authorsrecommended that ETCO2 be monitored forchildren with OSA receiving oxygen therapy.

Several investigators have assessed the impact ofnasal steroids in children with SRBDs. Mansfield

Sleep-Related Breathing Disorders of Childhood 457

and colleagues [128] found that 6 weeks of therapyusing intranasal budesonide resulted in improve-ment of mean AHI from 8.4 to 1.2 among 14 pread-olescent children with allergic rhinitis and SRBD.More modest improvements in AHI (5.2–3.2)were apparent in a separate study of 27 children re-ceiving 4 weeks of budesonide [129]. In a random-ized, placebo-controlled trial, a 6-week trial offlucticasone was associated with reduction of AHIfrom 10.7 to 5.8 for 13 children with OSA com-pared with an increase (10.9–13.1) for affectedchildren who received placebo [130]. Becauseposttreatment AHIs remained abnormal for manyof the children in these studies, and becauselong-term trials assessing safety and effectivenesshave not been performed, nasal steroids cannotyet be recommended as sole treatment for mostchildhood SRBDs.

Other medical treatments for childhood SRBDshave received only limited study. Five days of oralcorticosteroid therapy was found to be ineffectivein treating OSA in seven children with adenotonsil-lar hypertrophy [131]. Weight loss was reported tobe effective in improving nocturnal oxygen satu-ration in a morbidly obese 8 year old placed ona calorie-restricted diet [132]. Positional therapy,in which a patient is prevented from comfor-tably assuming a supine position during sleep byattaching a small ball to the back of the pajamas,has been reported to be helpful in adults withposition-dependent OSA, but has not been studiedin children [133].

Present needs and future directions forassessment and treatment of childhoodsleep-related breathing disorders

Despite considerable advances in the assessmentand treatment of childhood SRBDs, importantquestions and clinical needs must still beaddressed.

� There is little evidence-based consensus onoptimal PSG methods and standards foruse in children. Available data suggest thatstandard PSG measures do not reliablypredict neurocognitive sequelae and thatnew techniques are required for moreaccurate understanding of the relation-ships between subjective symptoms, PSGfindings, and outcomes in children withSRBDs.

� Cost-effective and clinically validated screen-ing measures are required for the assessmentof at-risk children. Despite relatively highprevalence in children, exceeding that ofdiabetes, sickle cell anemia, and other

childhood conditions for which childrenare commonly screened [134], symptoms ofOSA often go unrecognized by parents andprimary care providers. Even when symptomsare recognized, a lack of sleep laboratoriesfamiliar with assessment of children andoften lengthy wait-times before PSG is per-formed contribute to well-recognized delaysin diagnosis and treatment for many childrenwith SRBDs [135]. Development of inexpen-sive and cost-effective screening tools isrequired for identification of children whoneed screening with full PSG and thosewho require more urgent clinical or labora-tory assessment.

� Evidence-based guidelines are needed todetermine which pathways for clinicalassessment and treatment are the most eff-ective, safe, and economical. Although PSGis the only way of accurately determiningthe presence and severity of SRBDs, nomore than 12% of children undergoing ATfor suspected OSA receive preoperativePSG [136]. This results in some childrenwho do not have OSA receiving surgerythat is potentially unnecessary. Legitimatecounterarguments can made that lengthywait-times for laboratory-based PSG canresult in delayed treatment for symptomaticchildren and that presence or absence ofOSA as determined by standard PSG doesnot always predict which children willexhibit neurobehavioral improvements fol-lowing surgery [17]. Much additional workis needed to establish efficient and appro-priate clinical pathways acceptable for usein children.

� Randomized trials are required to definemore accurately the extent to which PSGmeasures, neurobehavioral outcomes, andother SRBD-related morbidities improvefollowing AT or with use of CPAP. In addi-tion, data are needed to determine betterwhen treatment should be implemented (ie,do SRBDs need to be identified and treatedearly to avoid long-term developmentalconsequences?).

� Improved nonsurgical treatment options areneeded for children. Despite increasing useof CPAP in the pediatric population,availability of child-sized interfaces remainsextremely limited. Additional research is re-quired to determine whether newer medicaltherapies for SRBDs, such as nasal steroidsand rapid maxillary expansion, are safe andeffective enough for routine clinical use inchildren.

Hoban & Chervin458

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