17.- sleep disorders in children

Sleep Disorders inChildren

Timothy F. Hoban, MD

ABSTRACTPurpose of Review: The purpose of this review is to examine how sleep disorders inchildren are affected by age and comorbid medical influences, and to discuss currentunderstanding of how the clinical manifestations, pathophysiology, and treatment ofcommon childhood sleep disorders differ from those of the adult population.Recent Findings: Recently established age-specific norms are required for accurateinterpretation of polysomnograms and multiple sleep latency tests in children.Summary: Sleep disorders such as insomnia, obstructive sleep apnea, and excessivedaytime somnolence are common in both children and adults, but the clinical mani-festations and underlying pathophysiology of these disorders vary substantially withage. For example, the bedtime struggles of a temperamental toddler are associatedwith different symptoms and causative factors compared to psychophysiologicinsomnia affecting a middle-aged person. Similarly, a 6-year-old child with obstructivesleep apnea is more likely to exhibit daytime inattention and hyperactivity as a referabledaytime symptom than the clear-cut lethargy or sleepiness that most affected adultsexperience. This review will examine how insomnia, excessive sleepiness, and ob-structive sleep apnea differ in children compared to adults.

Continuum (Minneap Minn) 2013;19(1):185–198.

SLEEPLESSNESS IN INFANTSAND YOUNGER CHILDRENDetermining whether night waking inan infant or young child represents aclinically significant problem sometimesrequires careful comparison of the child’ssleep patterns and sleep duration tohighly variable and age-dependent nor-mative data (Figure 10-1).1 Most new-borns and younger infants initially sleepin brief 2- to 4-hour periods equally dis-tributed across the daytime and night-time hours and affected by the need forfrequent feedings during early infancy.As a result, frequent night waking isconsidered entirely normal for youngerinfants.

As circadian regulatory mechanismsgrow stronger and become entrained torecurring behavioral and environmentalcues, nighttime sleep gradually be-

comes longer and more continuousand daytime napping diminishes via aprocess called settling. Although mostinfants settle during the first year oflife, about 10% do not achieve con-solidated nighttime sleep by the age of12 months.2 Brief waking, up to severaltimes nightly, persists as a normalphenomenon for many toddlers with-out clinical sleep problems, usually at alevel not noticed by parents and care-givers.3 Night waking is consideredabnormal when it is disproportionatelyfrequent, prolonged, or disruptive forthe person’s age. Problematic night wak-ing is estimated to affect 20% of infantsand toddlers.4

Night waking in infants and youngerchildren is frequently associated withbedtime struggles, which may take theform of crying in infants, tantrums or

Address correspondence toDr Timothy F. Hoban, L3221Women’s Hospital, 1500 EastMedical Center Dr, SPC 5203,Ann Arbor, MI 48109-5203,[email protected].

Relationship Disclosure:Dr Hoban has performedmedicolegal review ofpediatric neurology-relatedcases.

Unlabeled Use ofProducts/Investigational

Use Disclosure: Dr Hobandiscusses the use oftreatments for sleep apnea inchildren, including nasalsteroids and maxillaryexpansion devices,which are unlabeled.

* 2013, American Academyof Neurology.

Supplemental digital content:Videos accompanying this ar-ticle are cited in the text asSupplemental Digital Content.Videos may be accessed byclicking on links provided inthe HTML, PDF, and iPadversions of this article; theURLs are provided in the printversion. Video legends beginon page 196.

185Continuum (Minneap Minn) 2013;19(1):185–198 www.aan.com/continuum

Review Article

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

‘‘curtain calls’’ in toddlers, and otherforms of bedtime resistance that varywith age (Case 10-1). Problematicbehaviors at bedtime may reflect avariety of underlying influences,

including anxiety, lack of sleepiness, adesire to engage the parent, or delib-erate effort to forestall sleep onset.Although bedtime struggles may occuron an occasional basis in healthy

KEY POINTS

h The symptoms,pathophysiology, andtreatments for somesleep disorders aresubstantially differentfor children comparedto adults.

h Ten percent of childrendo not achieveconsolidated nighttimesleep by 1 year of age.

Case 10-1A 12-month-old infant was referred by his pediatrician for evaluation oflong-standing difficulties settling to sleep at bedtime, associated withfrequent night waking. The child’s mother reported that he had alwayshad difficulty settling to sleep unless she was with him. On most nights, hewould fall asleep near 9:00 PM while being held by his mother, who wasusually watching television. Although he would initially remain asleepupon being transferred to his crib, on most nights he would awaken2 hours after sleep onset, crying, fully awake, and difficult to console. Hewould return to sleep after 30 to 60 minutes only if held by his motheruntil asleep. Night waking of similar duration and character would recurduring the remainder of the night at intervals of about 90 minutes untilmorning waking at or before 7:00 AM. The mother reported similardifficulties and circumstances settling her son for his 90-minute middaynap. The family’s attempts to let the child settle to sleep alone wereunsuccessful, as he would cry unabated until picked up and held, typicallyafter 20 to 40 minutes.

No obstructive symptoms or restlessness was reported during nighttimesleep, but daytime irritability was consistently observed when night wakingwas worse. The child was developmentally normal and otherwise healthyapart fromallergic rhinitis. Physical examination results were notable formildnasal congestion and 2+ tonsillar size (Figure 2-4), but otherwise normal.

Comment. This case illustrates a typical example of behavioral insomniaof childhood, a disorder in which a child’s difficulty falling asleep orstaying asleep is secondary to inappropriate sleep-onset associationsVinthis case a habitual need to have his mother present while fallingasleepVor inconsistent limit setting.5

FIGURE 10-1 Percentiles for total nighttime (A) and daytime (B) sleep duration during childhood.

Reprinted with permission from Iglowstein I, et al, Pediatrics.1 B 2003, by the AAP. pediatrics.aappublications.org/content/111/2/302.abstract?sid=8cea3a74-5f1c-4356-9d03-57d1cf604c90.

186 www.aan.com/continuum February 2013

Sleep Disorders in Children


pediatrics.aappublications.org/content/111/2/302.abstract?sid=8cea3a74-5f1c-4356-9d03-57d1cf604c90

pediatrics.aappublications.org/content/111/2/302.abstract?sid=8cea3a74-5f1c-4356-9d03-57d1cf604c90

younger children, more substantialbedtime resistance is estimated toaffect 10% to 20% of toddlers andpreschool-aged children.6

In the sleep-onset association typeof behavioral insomnia, a child habitu-ally settles to sleep via circumstancesthat cannot be independently sus-tained, such as being rocked or havinga parent present. Affected childrenbecome so dependent on these rou-tines for transition to sleep that theyare unable to settle to sleep withoutthem. Attempts to do so result in in-somnia or distress at bedtime, andinability to return to sleep followingthe physiologic awakenings normallyoccurs during the middle and latterportions of the nighttime sleep period.

The limit-setting type of behavioralinsomnia is characterized by inconsis-tent limit setting by parents or care-givers in response to a child’s bedtimestruggles or night waking. Whereasconsistent limit-setting reduces thefrequency and severity of problematicbehaviors at these times, inconsistentor suboptimal responses to a child’sinappropriate behavior tend to perpet-uate the sleep problem.

Treatment of insomnia and nightwaking for infants and young childrenbegins with optimization of sleephygiene, practices that promote opti-mal nighttime sleep. Structured bed-time routines appropriate for age helpmany younger children transition froma higher level of daytime activity to aquieter and more relaxed state thatfacilitates sleep onset. Routines foryounger children often include bathing,tooth-brushing, changing clothes, read-ing stories, and being ‘‘tucked in.’’Potentially stimulating activities suchas vigorous play or watching televisionare best avoided in children who havedifficulty with settling at bedtime.

Establishing an optimal sleep envi-ronment is also important for younger

children with sleep problems. To theextent possible, the child should beprovided with an age-appropriate cribor bed in a quiet and comfortable en-vironment. Exposure to ambient lightat bedtime and during the night shouldbe minimized apart from judicious useof small night lights for children whoare afraid of the dark. Maintaining aregular sleep schedule can promotecircadian entrainment, which oftenfacilitates sleep onset close to the ha-bitual bedtime.

Structured behavioral interventionsare often additionally necessary foreffective treatment of behavioral in-somnia of childhood. Well-establishedmethods include extinction (systematicignoring of crying or other behaviorsuntil the child eventually falls asleep)and graduated extinction (a variationin which extinction techniques areapplied for progressively increasingintervals before parents are allowed tobriefly resettle their child).7,8 Bedtimefading involves transiently moving achild’s bedtime later, ie, closer to thetime of actual sleep onset, with even-tual return to an earlier, age-appropri-ate bedtime after the child is fallingasleep more quickly.9 Consistent andsustained application of these interven-tions is usually necessary to achievesustained clinical improvement in chil-dren with more severe forms of bed-time struggles and night waking.

Drug treatment of insomnia in in-fants and younger children has receivedscant formal study and is not routinelyrecommended.

INSOMNIA IN OLDER CHILDRENAND ADOLESCENTSInsomnia remains common as childrengrow older, affecting at least 10% ofadolescents,10 but underlying causeschange substantially with advancingage. Older children and adolescentshave increasing autonomy with respect

KEY POINTS

h Sleep-onset associationdisorder is one of themost common underlyingor contributing causesfor insomnia and nightwaking in infants andyounger children.

h Many families tryextinction-basedinterventions for only afew nights, encounter an‘‘extinction burst’’ oftemporarily worsesymptoms, and abandonthe technique before ithas had time to beeffective.



to bedtime and sleep schedule as theygrow older, often resulting in bedtimesthat are too late to permit habituallysufficient nighttime sleep. Evening-time use of electronic devices such ascomputers, smart phones, televisions,or video games can delay bedtime orbe sufficiently stimulating to delaysleep onset even after cessation ofthe activity.11 Consumption of caffei-nated beverages, particularly late in theday, may also be associated withinsomnia and sleep problems in thisage group.12

One of the most important influen-ces that affect insomnia in older chil-dren and adolescents is the well-recognized tendency for many childrenin this age range to become ‘‘nightowls’’ who gravitate toward later bed-times and waking times.13 Circadianrhythm disorder, delayed sleep-phasetype, can be diagnosed when a ten-dency toward delayed sleep phase isassociated with habitual inability to fallasleep and/or wake up at desired orsocially acceptable times. Some pa-tients with this disorder compensatefor insufficient nighttime sleep withdaytime naps, which may lead tofurther difficulties falling asleep at aconventional bedtime. The commonadolescent practice of habitually laterbedtime and waking time on nonYschool nights can lead to worseningof the sleep-phase delay and result inadditional irregularity of sleep sched-ule compared to school nights.14

Other forms of insomnia, such aspsychophysiologic insomnia and in-somnia due to drug or substance, canalso affect older children and teenagers,with symptoms and contributory influ-ences similar to those of affected adults.Insomnia may occur secondary tounderlying medical conditions such asattention deficit hyperactivity disorderor developmental disabilities. Insomniacan also represent an associated feature

of other sleep disorders such as restlesslegs syndrome.

Treatment of insomnia for olderchildren and adolescents is seldom suc-cessful unless all pertinent influencesare addressed and the child is moti-vated enough to make the lifestyle andsleep schedule changes that are usuallynecessary to correct the problem.

Sleep hygiene should be examinedand optimized before more specificand labor-intensive treatments are im-plemented. Any excessive consumptionof caffeinated beverages should benormalized on a gradual basis to avoidwithdrawal symptoms and any late-dayintake should be eliminated. Potentiallystimulating activities, including home-work, vigorous exercise, or use of elec-tronic devices, should be moved toalternative times and optimally replacedwith a structured prebedtime routineincorporating less stimulating activities.

A target weekday sleep scheduleshould be identified based on thechild’s age, school schedule, individualsleep needs, and family needs. Effortsshould be made to keep bedtime andwaking time on nonYschool days con-sistent with those on school days toeliminate irregularity of sleep schedule.Any daytime napping should be elimi-nated. Sleep schedule goals should benegotiated with the family in a prag-matic fashion, which sometimes re-quires that initial targets be achievable,although perhaps not ideal.

When insomnia related primarily todelayed sleep phase is mild, consis-tent implementation of the measuresdescribed above are sufficient to alle-viate insomnia and permit adequatenighttime sleep duration. Gradual butconsistent advancement of the child’sbedtime and waking time toward theearlier times of the target schedulemay be additionally necessary.

Chronotherapy is a useful methodfor treating severe or resistant forms

KEY POINTS

h Delayed sleep phase isan extremely commoncause or contributingfactor to insomnia inadolescent or olderpreadolescent children.When present,medication treatmentalone is unlikely to beeffective.

h ‘‘Sleeping in late’’ onweekend andnonYschool days canreduce the effectivenessof other interventionsfor delayed sleep phase.




of insomnia related to delayed sleepphase. This treatment entails pro-gressive delays of the child’s bedtimeand waking time by 2 to 3 hours dailyuntil the target sleep scheduleis attained, typically within 5 to 7days.15,16 The technique is rapid, safe,and effective, but benefits are onlysustained when the target sleepschedule is rigorously maintained fol-lowing the initial treatment. Lighttherapy using carefully timed expo-sure to high-intensity full-spectrumsources following morning waking isalso sometimes used for treatment ofdelayed sleep phase in children andadolescents.16

Drug treatment of insomnia in olderchildren and adolescents has not beenrigorously studied despite seeminglywidespread use of homeopathic, non-prescription, and off-label prescriptionagents in this population.17,18 Severalstudies have reported melatonin to beeffective in treating insomnia forschool-aged children.19,20

OBSTRUCTIVE SLEEP APNEAIN CHILDRENObstructive sleep apnea (OSA) isestimated to affect about 2% of chil-dren, with peak incidence thought tooccur between 3 and 8 years of age.21

Clinical features of OSA in childrencompared to adults are summarizedin Table 10-1.22

The nighttime symptoms exhibitedby affected children are similar to, butoften milder than, those exhibited byadults. Most children with OSA havesome degree of snoring or noisy respi-ration during sleep, but thismay bemildor intermittent. Other common butvariable nighttime symptoms includemouth breathing, restlessness, and dia-phoresis. Dramatic pauses in respirationduring sleep are observed by parentsand caregivers only when OSA is severe,consistent with current understanding

that childhood OSA is more commonlycharacterized by intermittent or sus-tained partial obstruction during sleepas opposed to recurrent episodes ofcomplete obstruction (SupplementalDigital Content 10-1, links.lww.com/CONT/A28).

Daytime symptoms of OSA in chil-dren may be more subtle or variablecompared to adults. Children with OSAare seldom obviously sleepy during theday unless nighttime airway obstructionis severe. Daytime sleepiness in af-fected children is more commonly in-termittent or mild, sometimes evidentonly during sustained sedentary activ-ities such as riding in an automobile.Disturbances in attention, behavior,and academic performance representmore common and prominent daytimesymptoms in children with OSA, butthese are frequently misattributed toalternative causes, such as attentiondeficit hyperactivity disorder (ADHD).

A small proportion of children withOSA may present with isolated systemicor constitutional symptoms such asfailure to thrive or unexplained hyper-tension.23 A variety of medical condi-tions may be associated with increasedrisk for childhood OSA (Table 10-2),24

including Down syndrome, Prader-Willisyndrome, cleft palate repair, and cra-niofacial disorders characterized bymicrognathia, such as Pierre Robinsyndrome (Figure 10-2).25,26 Obesityrepresents an increasingly prevalentrisk factor for OSA in children, partic-ularly during adolescence.27

Although the physical examinationfindings of children with OSA are fre-quently normal, some children exhibitcharacteristic findings associated withincreased risk for upper airway obstruc-tion during sleep, including mouthbreathing, tonsillar hypertrophy, nar-row upper palate, and maxillary or man-dibular hypoplasia. Although tonsillarhypertrophy is common among children

KEY POINTS

h Obstructive sleep apneais underdiagnosed inchildren in part becausethe lack of observedrespiratory pauses andobvious daytimesleepiness in mostaffected children limitparent and medicalpractitioner recognitionthat the condition mightbe present.

h Although obesityrepresents a risk factorfor childhoodobstructive sleep apnea,children with low ornormal body weight canhave substantialobstructive sleep apnea,particularly whenunderlying adenotonsillarhypertrophy is present.



links.lww.com/CONT/A28


with OSA (Figure 2-4 in ‘‘Approach toand Evaluation of Sleep Disorders’’),its presence is neither necessary norsufficient for the diagnosis of child-hood OSA.

Current criteria for the diagnosis ofOSA in childrenwere established in 2005by the International Classification ofSleep Disorders, Second Edition: Diag-nostic and Coding Manual.1 Definitivediagnosis of the disorder requires acombination of clinical criteria asso-

ciated with symptomatic OSA (eg, snor-ing, diaphoresis, daytime hyperactivity,or behavior problems) and polysomno-graphic confirmation that airway ob-struction associated with disturbedgas exchange or sleep architecture ispresent. Whereas the diagnostic criteriafor adult OSA require that at least fiverespiratory disturbances per hour bepresent during polysomnography(PSG), pediatric criteria require a mini-mum of only one disturbance per hour,

TABLE 10-1 Comparative Features of Obstructive Sleep Apnea inChildren Compared to Adultsa

Features Children Adults

Physical characteristicsGender Younger children: sexes

equally affectedPrimarily males,postmenopausal females

Adolescents: males9females

Peak age 2Y8 Years Middle aged and older

Body weight Usually normal, occasionallyoverweight

Most often obese

Upper airway Adenotonsillar enlargementfrequent

Adenotonsillarenlargement occasional

Redundant soft tissueoccasional

Redundant soft tissuefrequent

Symptoms during sleepSnoring Frequent, continuous,

or intermittentFrequent, ofteninterrupted by pauses

Witnessed apnea Occasional Frequent

Polysomnographic characteristicsObstruction Prolonged partial

obstruction9intermittentCyclical intermittentobstruction

Sleep architecture Normal9fragmented Frequent arousals withsleep fragmentation

Secondary symptomsDaytime sleepiness Most often absent or

intermittentFrequent, usuallyprominent

Neurobehavioral Inattention, hyperkinesis,disturbed behavior

Cognitive slowing,increased accident risk

Cardiovascular Hypertension, cor pulmonale Hypertension, heartdisease, stroke

a Reprinted with permission from Hoban T, Ann N Y Acad Sci.22 onlinelibrary.wiley.com/doi/10.1111/j.1749-6632.2009.05112.x/abstract.




onlinelibrary.wiley.com/doi/10.1111/j.1749-6632.2009.05112.x/abstract

onlinelibrary.wiley.com/doi/10.1111/j.1749-6632.2009.05112.x/abstractlinks.lww.com/CONT/A29

reflecting the increased vulnerability ofchildren to the effects of mild noc-turnal airway obstruction compared toadults.28

Adenotonsillectomy represents themost commonly used treatment forOSA in children. Although this proce-dure had historically been considered tobe highly effective in treating childhoodOSA,29 recent studies using contempo-rary diagnostic criteria and PSG havesuggested that respiratory parametersduring sleep fully normalize in only25% to 50% of children with OSA whoundergo adenotonsillectomy.30,31 Al-though these studies should be inter-preted with due recognition that manyof the children whose OSA was notcompletely cured by adenotonsillec-

tomy nevertheless experienced signifi-cant partial improvement of their OSA,these and other reports suggest that

KEY POINT

h Of children undergoingadenotonsillectomy fortreatment of obstructivesleep apnea, 50% to75% may still havesome degree ofobstructive sleep apneapostoperatively (usuallymilder).

TABLE 10-2 Medical Conditions Associated With Increased Risk forChildhood Obstructive Sleep Apneaa

b Craniofacial Syndromes Featuring ProminentMaxillary orMandibularHypoplasiaApert syndrome

Crouzon syndrome

Pierre Robin syndrome

Saethre-Chotzen syndrome

Treacher Collins syndrome

b Other Skeletal and Craniofacial DisordersAchondroplasia

Choanal atresia

Cleft palate, especially following surgical repair

Velocardiofacial syndrome

b Systemic Genetic and Metabolic DisordersDown syndrome

Hypothyroidism

Mucopolysaccharide storage disorders (eg, Hunter syndrome, Hurler syndrome)

Prader-Willi syndrome

b Other Medical and Neurologic ConditionsBrainstem and cranial nerve disorders (eg, syringobulbia, Chiari malformation)

Chronic nasal obstruction (eg, septal deviation, allergic rhinitis, polyp, infections)

Obesity

Sickle cell disease

a Reprinted from Hoban TF, Chervin RD, Sleep Med Clin.24 B 2007, with permission from Elsevier.www.sciencedirect.com/science/article/pii/S1556407X07000550.

FIGURE 10-2 Child with Pierre Robin syndrome andmicrognathia.

Reprinted from Tewfik TL, Der Kaloustian VM, Oxford University Press.25

By permission of Oxford University Press, USA.



www.sciencedirect.com/science/article/pii/S1556407X07000550

clinicians must remain vigilant for re-sidual or recurrent OSA following ade-notonsillectomy in children.32,33

Most childrenwithOSAwho undergoadenotonsillectomy exhibit tangibleimprovements in obstructive symptomsduring sleep and in overall sleep quality.The impact on daytime symptoms maybe variable, especially when comorbidnon-OSA influences are present, butmultiple studies have reported im-proved academic performance or ADHDsymptoms in children whose OSA wastreated via adenotonsillectomy.34,35

Continuous positive airway pressure(CPAP) is also considered a first-linetreatment for childhood OSA. Althoughused less frequently than adenotonsil-lectomy for the pediatric population,CPAP is particularly useful in childrenwhose OSA persists despite adenoton-sillectomy and in children who are notappropriate candidates for adenotonsil-lectomy (eg, morbid obesity, craniofa-cial disorders, and other conditions inwhich airway obstruction is not primar-ily related to adenotonsillar obstruc-tion) (Case 10-2).

KEY POINT

h Continuous positiveairway pressure isconsidered a first-linetreatment for obstructivesleep apnea in children.

Case 10-2An 8-year-old boy with a history of autoimmune hepatitis and recentliver transplantation was referred for evaluation of snoring and daytimesleepiness. Snoring had become severe since liver transplantation and wasassociated with mouth breathing and significant restlessness during sleep.The family also reported substantial daytime tiredness for at least 1 yeardespite 10 hours of sleep per night on average. The child typically nappedafter school 2 to 3 days weekly and would fall asleep quickly duringautomobile rides. His teachers at school reported substantial problemswith drowsiness, inattention, oppositional behavior, and poor academicperformance. Initial examination was notable for obesity (body mass indexof 34 kg/m2) and noisy mouth breathing. Examination of the oropharynxwas significant for a Mallampati class IV airway, large scalloped tongue,mildly high-arched palate, and 2+ tonsillar size.

A baseline polysomnogram confirmed the presence of severe obstructivesleep apnea (OSA), characterized by frequent obstructive apneas andhypopneas associated with marked sleep fragmentation and prominentdesaturation of SpO2. The apnea-hypopnea index was 86.7 events/h and9.7% of total sleep time spent with SpO2 levels below 90.0%. End-tidalcarbon dioxide monitoring demonstrated no sleep-related hypoventilation.

The patient underwent adenotonsillectomy and was admitted overnightfor postoperative respiratory monitoring (as is standard practice for childrenwith severe sleep apnea undergoing this procedure). Following the child’srecovery from surgery, his family reported that his snoring and restlessnessduring sleep had improved substantially and become mild in severity.Daytime somnolence and napping had nearly resolved. The child’s teachersreported little improvement with respect to inattention and distractibility atschool, however, and told the child’s mother that he was at risk of not beingpromoted to the next grade.

A postoperative polysomnogram demonstrated mild OSA, characterizedby hypopneas associated with mild desaturation of SpO2 and slightfragmentation of sleep architecture. The apnea-hypopnea index was

Continued on page 193




CPAP is generally considered to beboth safe and effective for the treatmentof childhood OSA, although acquiredmaxillary hypoplasia has been reportedas a rare complication of long-termtreatment.36 Limitations of this treat-ment in children relate primarily tothe fact that not all children success-fully acclimate to use of the device orachieve long-term compliance with itsuse. Use of CPAP may be especially chal-lenging in young or developmentallydisabled children, although structureddesensitization techniques are some-times effective in helping these patientsachieve successful long-term use.

Alternative treatments for childhoodOSA are considered when the first-linetreatments are either ineffective or notfeasible. Several studies have suggestedthat use of nasal steroids or maxillaryexpansion devices may help alleviateupper airway obstruction for some chil-dren with OSA, but the overall effec-tiveness and safety of these treatmentshave not been well established.37,38 Useof supplemental oxygen or positionaltherapy during sleep may providesome (often partial) benefit for chil-dren with OSA. Children with severeand otherwise refractory OSA some-

times benefit from alternative surgicalinterventions, such as uvulopalato-pharyngoplasty, maxillary or mandibularadvancement, or even tracheostomy,although pediatric data regarding theseprocedures are extremely limited.

EXCESSIVE DAYTIMESOMNOLENCE IN CHILDRENHealthy children and adolescentswho receive sufficient nighttime sleepnormally exhibit excellent levels ofdaytime alertness and seldom nap dur-ing the day. The presence of persis-tently excessive sleepiness in a childgenerally signifies one of three majorpossibilities:

1. That the child is not receivingsufficient nighttime sleep

2. That an underlying sleep disorderhas severely disrupted nighttimesleep

3. That a sleep or medical disorderthat may cause sleepiness even inthe absence of disrupted nighttimesleep (eg, narcolepsy, anemia,hypothyroidism) is present

Determining whether a youngpatient is receiving habitually sufficientnighttime sleep requires careful review

KEY POINTS

h Nasal steroids and rapidmaxillary expansionrepresent promisingalternative techniquesfor treatment ofchildhood obstructivesleep apnea.

h Some pediatric studiessuggest that mildobstructive sleep apnea ismore likely to beassociated with symptomssuggestive of attentiondeficit hyperactivitydisorder than more severeforms are.

h The presence ofsignificant sleepiness ina child with snoring isconcerning for thepresence of severeunderlying obstructivesleep apnea.

1.9 events/h, rising to 6.2 during REM sleep. A subsequent continuouspositive airway pressure trial demonstrated that pressure settings of 7 cmand 9 cm of water provided good control of obstructive respiratorydisturbances during sleep.

The child was highly compliant with home CPAP use, and the familyreported that snoring and restlessness during sleep resolved completelywhen it was initiated. Inattention and distractibility at school improvedmoderately after CPAP was started, and the family reported no substantialacademic concerns at their last appointment.

Comment. This case illustrates several important clinical aspects ofchildhood OSA, including the facts that severe OSA frequently persists in amilder form following adenotonsillectomy, that mild OSA without daytimesomnolencemay still be associatedwith neurobehavioral sequelae that benefitfrom treatment, and that CPAP can successfully be implemented as long-termtreatment for OSA in a relatively young child with complex medical needs.

Continued from page 192



of the child’s sleep schedule and com-parison to age-appropriate norms(Table 10-1).1 If daytime sleepinessresolves after nighttime sleep is length-ened, additional investigation andtreatment are often unnecessary.

When excessive sleepiness persistsdespite adequate or lengthened night-time sleep, other potential causes must

be explored. A detailed medical historyoften identifies symptoms and riskfactors that guide further investigation,such as symptoms of nocturnal sleepdisruption (eg, snoring, restlessness), ahistory of medical disorders that can beassociated with fatigue and sleepiness(eg, depression, Epstein-Barr virus in-fection), or use of potentially sedating

KEY POINT

h Chronically insufficientnighttime sleeprepresents a verycommon cause ofsleepiness and pooracademic performancein children.

TABLE 10-3 Ontogeny of Sleep EEG Background and SleepArchitecture During Infancy and Childhood

b Primary EEG Background Activity During Sleep in Premature Infants26Y30 Weeks postconceptional age: trace discontinue

28Y30 Weeks postconceptional age: delta brushes (disappear by term)

32Y36 Weeks postconceptional age: trace alternant (disappears by 12weeks postterm)

b Ontogeny of Key Sleep-Staging Landmarks30 Weeks postconceptional age: REMs apparent

2 Months postterm: sleep spindles apparent

4Y6 Months: K complexes and well-differentiated non-REM stages

b Development of the Waking Posterior Dominant Rhythm2 Months: Q4 Hz

6 Months: Q6 Hz

3 Years: Q8 Hz

9 Years: Q9 Hz

b Ontogeny of Sleep ArchitectureTerm neonates: 50% REM; 50% non-REM (sleep-onset REM common)

6Y12 Months: 30%Y35% REM; 65%Y70% non-REM

3Y5 Years: 25% REM; 75% non-REM

TABLE 10-4 Pediatric Norms for Multiple Sleep Latency Testinga,b

Tanner Stage Mean Sleep Latency (Minutes)c SD

Stage 1 19.0 1.6

Stage 2 18.5 1.9

Stage 3 16.1 3.8

Stage 4 15.8 3.4

Stage 5 16.6 2.1

Older adolescents 15.7 3.4

SD = standard deviations.a Adapted from Carskadon MA, Addison-Wesley.43b Reprinted from Hoban TF, Chervin RD, Semin Pediatr Neurol.44 B 2001, with permission from Elsevier.c Data averaged from tests performed on 3 successive days of recording.




medications. Physical examinationsometimes identifies additional clues,such as anatomic features associatedwith increased risk for OSA or physicalfindings suggestive of underlying ane-mia or hypothyroidism.

Although sleepiness in children ismost often secondary to insufficientnighttime sleep or another underlyingsleep disorder, such symptoms areoccasionally the result of hypersom-nias of central origin, such as recurrenthypersomnia (Kleine-Levin syndrome)or narcolepsy.

Kleine-Levin syndrome is a rare con-dition characterized by recurrent epi-sodes of sleepiness, encephalopathy, andaltered appetite that last days toweeks.5,39 Behavioral disturbances dur-ing episodes may include aggression orinappropriate sexual behaviors that re-quire additional treatment and safety pre-cautions beyond symptomatic treatmentof the hypersomnia. This disorder mostoften affects adolescent boys and some-times remits spontaneously with time.

Narcolepsy in children is character-ized by the same clinical manifestations

Case 10-3An 8-year-old girl was referred by another neurologist for further evaluation of pervasive daytimesleepiness and intermittent weakness sometimes associated with loss of posture. The family reportedthat her pervasive sleepiness had been noted during the prior school year, when the child hadoccasionally fallen asleep in school. Sleepiness at the time of initial evaluation was described assubstantial, with the child sleeping almost constantly on automobile rides and napping for up to 90minutes daily despite 10 to 11 hours of sleep during most nights.

Episodic weakness was first noted at 2 years of age and became gradually more prominent withadvancing age. Events were reported to occur almost exclusively during giggling or laughter, persistingfor several seconds to several minutes before subsiding. Milder episodes were characterized onlyby brief, subtle dipping of the head, whereas more severe events were characterized by diffuse weaknessand loss of postural tone. (The accompanying video [Supplemental Digital Content 10-2, links.lww.com/CONT/A29] depicts a different patient with a similar condition.) Several spells had been associated withfalls resulting in broken bones.

The family reported no snoring or significant nighttime sleep problems apart from brief partialawakenings and mild restlessness during sleep. They observed no hallucinatory events or sleepparalysis near sleep onset or offset. Episodes of weakness and loss of posture were not associated withany impairment of consciousness, and an extensive prior workup for seizures had been negative. Theinitial examination was remarkable only for mouth breathing, a mildly narrow upper palate, andequivocal limitation of upward gaze.

A nocturnal PSG was essentially normal, recording 458.5 minutes of sleep and no significantrespiratory disturbances. A multiple sleep latency test performed the next day recorded sleep duringall five nap opportunities, with mean sleep-onset latency of 0.7 minutes and four sleep-onset REMperiods. Findings were interpreted as supporting the presence of severe sleepiness and compatiblewith the diagnosis of narcolepsy.

Daytime sleepiness and cataplectic episodes improved considerably following initiation of treatmentwith modafinil and tricyclic agents. School performance also improved, but declined several months laterdespite continued good control of sleep-related symptoms. This prompted further diagnosticinvestigation. A skin biopsy and cholesterol esterification studies demonstrated findings consistent withNiemann-Pick disease type C.

Comment. This case illustrates several important clinical aspects of childhood narcolepsy, includingmultiple sleep latency test findings that were strongly indicative of childhood narcolepsy, the fact thatnarcolepsy often respondswell to off-label treatment usingmodafinil, and that childrenwho presentwithnarcolepsy at a younger-than-typical age or experience progressive symptoms despite treatment mayrequire screening for other underlying disorders.





experienced by adults: daytime somno-lence, sleep paralysis, hypnagogic andhypnopompic hallucinations, and some-times cataplexy. Onset of the conditionis occasionally abrupt, but symptomsmore commonly evolve in an insidiousfashion over time. Clinicalmanifestationsmost commonly begin during the sec-ond decade of life, and onset at youngerages is sometimes associated with thepresence of additional underlying con-ditions such as Niemann-Pick diseasetype C or Prader-Willi syndrome.40,41

Sleep laboratory assessment of exces-sive sleepiness in children often requiresboth PSG and multiple sleep latencytesting (MSLT). Investigation commen-ces with nocturnal PSG, which docu-ments the duration of nighttime sleepand screens for OSA and other potentialdisruptors of nighttime sleep. The noc-turnal PSG should be interpreted usingpediatric norms42 (Table 10-3), whichdiffer substantially for children com-pared to adults. MSLT is performedthe next day to objectively assess theseverity of daytime somnolence andscreen for sleep-onset REM periods andother findings that help differentiatebetween narcolepsy and other potentialcauses of excessive sleepiness. Becausemean sleep latency scores for healthychildren undergoing MSLT are signifi-cantly longer than those for adults, itis essential that the nap study alsobe interpreted using pediatric norms(Table 10-4).43,44 Most children withnarcolepsy demonstrate multiple sleep-onset REM periods and mean sleeplatency of less than 5 minutes on MSLT.

Treatment of excessive daytimesleepiness in children is customizedbased on the severity of somnolenceand the nature of the associated clinicalcircumstances. Some sleepy childrenrequire nothing more than lengtheningnighttime sleep or treatment of anassociated medical or sleep disorderthat is causing sleepiness on a secon-

dary basis. Children with hypersomniasof central origin such as narcolepsy,however, usually benefit from treat-ment with wake-promoting medica-tions, such as modafinil, stimulants, orsodium oxybate, which are used cau-tiously on an off-label basis in thispopulation. Off-label use of selectiveserotonin reuptake inhibitors or tricy-clic agents is sometimes effective incontrolling cataplexy for children withnarcolepsy (Case 10-3).

VIDEO LEGENDSSupplemental Digital Content 10-1

Sleep apnea syndrome in a child. Video dem-onstrates sleep apnea syndrome in a child. Thischild is overweight and has grade 4+ tonsils,which has led to complete cyclical airway oc-clusion and resuscitative arousals. Obstructivesleep apnea in childrenmay have different causesand consequences than in adults. Children withuntreated obstructive sleep apnea may experi-ence neurocognitive decline, hyperactivity,hypersomnolence, and decline in IQ scores.


B 2013 Sona Nevsımalova, MD, DSc. Used withpermission.

Supplemental Digital Content 10-2

Child cataplexy. Video demonstrates cataplexyin a child, depicting loss of muscle tone triggeredby laughter.


B 2013 Sona Nevsımalova, MD, DSc. Used withpermission.

REFERENCES1. Iglowstein I, Jenni OG, Molinari L, Largo RH.

Sleep duration from infancy to adolescence:reference values and generational trends.Pediatrics 2003;111(2):302Y307.

2. Guilleminault C, Anders TF. Thepathophysiology of sleep disorders inpediatrics. Part II. Sleep disorders in children.Adv Pediatr 1976;22:151Y174.

3. Sadeh A, Lavie P, Scher A, et al. Actigraphichome monitoring of sleep-disturbed andcontrol infants and young children: anew method for pediatric assessment ofsleep-wake patterns. Pediatrics1991;87(4):494Y499.

4. Sheldon SH. Disorders of initiating andmaintaining sleep. In: Sheldon SH, Ferber R,Kryger MH, eds. Principles and practice of






pediatric sleep medicine. Philadelphia, PA:Saunders, 2005.

5. Anonymous. The international classificationof sleep disorders. 2nd ed. Westchester, NY:American Academy of Sleep Medicine, 2005.

6. Ramchandani P, Wiggs L, Webb V, et al. Asystematic review of treatments for settlingproblems and night waking in youngchildren. BMJ 2000;320(7229):209Y213.

7. Mindell JA, Kuhn B, Lewin DS, et al.Behavioral treatment of bedtime problemsand night wakings in infants and youngchildren [published erratum in Sleep.2006;29(11):1380]. Sleep 2006;29(10):1263Y1276.

8. Morgenthaler TI, Owens J, Alessi C, et al.Practice parameters for behavioraltreatment of bedtime problems and nightwakings in infants and young children.Sleep 2006;29(10):1277Y1281.

9. Taylor DJ, Roane BM. Treatment ofinsomnia in adults and children: apractice-friendly review of research. J ClinPsychol 2010;66(11):1137Y1147.

10. Roberts RE, Roberts CR, Chan W. Persistenceand change in symptoms of insomnia amongadolescents. Sleep 2008;31(2):177Y184.

11. Cain N, Gradisar M. Electronic media useand sleep in school-aged children andadolescents: a review. Sleep Med 2010;11(8):735Y742.

12. Bryant Ludden A, Wolfson AR.Understanding adolescent caffeine use:connecting use patterns with expectancies,reasons, and sleep. Health Educ Behav2010;37(3):330Y342.

13. Carskadon MA. The second decade. In:Guilleminault C, ed. Sleeping and wakingdisorders: indications and techniques.Menlo Park, CA: Addison Wesley, 1982:99Y125.

14. Crowley SJ, Acebo C, Carskadon MA.Sleep, circadian rhythms, and delayed phasein adolescence. Sleep Med 2007;8(6):602Y612.

15. Czeisler C, Richardson G, Coleman R, et al.Chronotherapy: resetting the circadianclocks of patients with delayed sleep phaseinsomnia. Sleep 1981;4(1):1Y21.

16. Okawa M, Uchiyama M, Ozaki S, et al.Circadian rhythm sleep disorders inadolescents: clinical trials of combinedtreatments based on chronobiology.Psychiatry Clin Neurosci 1998;52(5):483Y490.

17. Pelayo R, Dubik M. Pediatric sleeppharmacology. Semin Pediatr Neurol2008;15(2):79Y90.

18. Owens JA, Rosen CL, Mindell JA. Medicationuse in the treatment of pediatric insomnia:results of a survey of community-basedpediatricians. Pediatrics 2003;111(5 pt 1):e628Ye635.

19. Smits M, Van Stel H, Van der Heijen K, et al.Melatonin improves health status andsleep in children with idiopathic chronicsleep-onset insomnia: a randomized,placebo-controlled trial. J Am Acad ChildAdolesc Psychiatry 2003;42(11):1286Y1293.

20. van Geijlswijk IM, Mol RH, Egberts TCG,Smits MG. Evaluation of sleep, puberty andmental health in children with long-termmelatonin treatment for chronic idiopathicchildhood sleep onset insomnia.Psychopharmacology (Berl) 2011;216(1):111Y120.

21. Marcus CL. Sleep-disordered breathing inchildren. Am J Respir Critical Care Med2001;164(1):16Y30.

22. Hoban TF. Sleep disorders in children. Ann NY Acad Sci 2010;1184:1Y14.

23. Li AM, Au CT, Sung RY, et al. Ambulatoryblood pressure in children with obstructivesleep apnoea: a community based study.Thorax 2008;63(9):803Y809.x

24. Hoban TF, Chervin RD. Sleep-relatedbreathing disorders of childhood:description and clinical picture, diagnosis,and treatment approaches. Sleep Med Clin2007;2:445Y462.

25. Tewfik TL, Der Kaloustian VM. Congenitalanomalies of the ear, nose, and throat. NewYork, NY: Oxford University Press, 1997:517.

26. Shott SR, Amin R, Chini B, et al. Obstructivesleep apnea: should all children with Downsyndrome be tested? Arch Otolaryngol HeadNeck Surg 2006;132(4):432Y436.

27. Kalra M, Inge T, Garcia V, et al. Obstructivesleep apnea in extremely overweightadolescents undergoing bariatric surgery.Obes Res 2005;13(7):1175Y1179.

28. Rosen CL, D’Andrea L, Haddad GG. Adultcriteria for obstructive sleep apnea do notidentify children with serious obstruction.Am Rev Respir Dis 1992;146(5 pt 1):1231Y1234.

29. Brietzke SE, Gallagher D, Brietzke SE, et al.The effectiveness of tonsillectomy andadenoidectomy in the treatment ofpediatric obstructive sleep apnea/hypopneasyndrome: a meta-analysis. OtolaryngolHead Neck Surg 2006;134(6):979Y984.

30. Tauman R, Gulliver TE, Krishna J, et al.Persistence of obstructive sleep apnea



syndrome in children after adenotonsillectomy.J Pediatr 2006;149(6):803Y808.

31. Guilleminault C, Huang YS, Glamann C,et al. Adenotonsillectomy and obstructivesleep apnea in children: a prospective survey.Otolaryngol Head Neck Surg 2007;136(2):169Y175.

32. Amin R, Anthony L, Somers V, et al.Growth velocity predicts recurrence ofsleep-disordered breathing 1 year afteradenotonsillectomy. Am J Respir Crit CareMed 2008;177(6):654Y659.

33. Mitchell RB. Adenotonsillectomy forobstructive sleep apnea in children:outcome evaluated by pre- andpostoperative polysomnography.Laryngoscope 2007;117(10):1844Y1854.

34. Chervin RD, Ruzicka DL, Giordani BJ, et al.Sleep-disordered breathing, behavior, andcognition in children before and afteradenotonsillectomy. Pediatrics2006;117(4):e769Ye778.

35. Gozal D. Sleep-disordered breathing andschool performance in children. Pediatrics1998;102(3 pt 1):616Y620.

36. Li KK, Riley RW, Guilleminault C. Anunreported risk in the use of home nasalcontinuous positive airway pressure andhome nasal ventilation in children: mid-facehypoplasia. Chest 2000;117(3):916Y918.

37. Kheirandish-Gozal L, Gozal D. Intranasalbudesonide treatment for children withmild obstructive sleep apnea syndrome.Pediatrics 2008;122(1):e149Ye155.

38. Villa MP, Malagola C, Pagani J, et al. Rapidmaxillary expansion in children withobstructive sleep apnea syndrome: 12-monthfollow-up. Sleep Med 2007;8(2):128Y134.

39. Arnulf I, Lin L, Gadoth N, et al. Kleine-Levinsyndrome: a systematic study of 108patients. Ann Neurol 2008;63(4):482Y493.

40. Kotagal S. Narcolepsy in children. SeminPediatr Neurol 1996;3(1):36Y43.

41. Smit LS, Lammers GJ, Catsman-BerrevoetsCE. Cataplexy leading to the diagnosis ofNiemann-Pick disease type C. Pediatr Neurol2006;35(1):82Y84.

42. Iber C, Ancoli-Israel S, Chesson A, Quan SF.The AASM manual for the scoring ofsleep and associated events. Westchester,NY: American Academy of Sleep Medicine,2007.

43. Carskadon MA. The second decade. In:Guilleminault C, ed. Sleeping and wakingdisorders: indications and techniques. MenloPark, CA: Addison-Wesley, 1982.

44. Hoban TF, Chervin RD. Assessment ofsleepiness in children. Semin Pediatr Neurol2001;8(4):216Y228.




17.- sleep disorders in children

Documents