Therapies for Children withObstructive Sleep Apnea

Christopher Cielo, DOa, Lee J. Brooks, MDb,*

KEYWORDS

� Children � Obstructive sleep apnea � Adenotonsillectomy � Positive airway pressure� Polysomnography

KEY POINTS

� The mainstays of therapy for obstructive sleep apnea in children are adenotonsillectomy and pos-itive airway pressure.

� Nonsurgical therapies, including anti-inflammatory medications, dental devices, and weight loss,may be useful in specific circumstances.

� Airway surgery has been shown to be effective in children with craniofacial abnormalities, but not inthe general pediatric population.

� Children with Down syndrome, craniofacial abnormalities, obesity, or Prader-Willi syndrome are atincreased risk for obstructive sleep apnea syndrome and may require more frequent polysomnog-raphy and specific therapies.

DIAGNOSIS OF OBSTRUCTIVE SLEEP APNEASYNDROME IN CHILDREN

Obstructive sleep apnea syndrome (OSAS) inchildren is a “disorder of breathing during sleepcharacterized by prolonged partial upper airwayobstruction and/or intermittent complete obstruc-tion (obstructive apnea) that disrupts normal venti-lation during sleep and normal sleep patterns.”1

Symptoms vary by age in children with OSAS,and may include neurobehavioral problems inyounger children, daytime sleepiness or head-aches in older children, and habitual snoring withor without observed apnea at any age. The se-quelae of untreated OSAS in children includesexcessive daytime sleepiness,2 neurocognitive im-pairment and behavioral problems,3,4 blood pres-sure dysregulation,5 diastolic hypertension, andless commonly pulmonary hypertension and fail-ure to thrive.6 Risk factors for OSAS in children

Disclosure: Dr Brooks has stock holdings in various prelevant to the topic of this article.a Division of Pulmonary Medicine, The Children’s HospitCivic Center Boulevard, Philadelphia, PA 19104, USA; b

Children’s Hospital of Philadelphia, University of PennCivic Center Boulevard, Philadelphia, PA 19104, USA* Corresponding author.E-mail address: brooksl@email.chop.edu

Sleep Med Clin 8 (2013) 483–493http://dx.doi.org/10.1016/j.jsmc.2013.07.0101556-407X/13/$ – see front matter � 2013 Elsevier Inc. Al

include adenotonsillar hypertrophy,7 neuromus-cular disorders,8 craniofacial abnormalities,9 andobesity.10

History and physical examination alone areunreliable for predicting the presence andseverity of OSAS in children.11,12 The gold stan-dard for evaluating sleep apnea in the pediatricpopulation is overnight, attended, in-laboratorypolysomnography. Children who snore on aregular basis and for whom parents reportobserved apnea, sleep enuresis, sleeping whilesitting or with the neck in a hyperextended posi-tion, cyanosis, headaches on awakening, daytimesleepiness, attention-deficit/hyperactivity disor-der, or learning problems should be referred toa sleep specialist or for polysomnography. Chil-dren who habitually snore and have physicalfindings including underweight or overweight,tonsillar hypertrophy, adenoidal facies, microgna-thia or retrognathia, high-arched palate, failure to

ublicly-traded, health-related companies. None are

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Cielo & Brooks484

thrive, or hypertension should also be referred forpolysomnography.13 Evidence-based guidelinesdefining OSAS severity in children do not exist,and the polysomnogram must be interpreted inthe clinical context of each individual case. Thedecision to treat OSAS in children involves con-sideration of both the severity of clinical signsand symptoms and the polysomnographic find-ings. Many therapies, both surgical (Table 1)and nonsurgical (Table 2), are available for selectgroups of children.

SPECIFIC THERAPIESAdenotonsillectomy

Tonsillectomy is a surgical procedure in which theperitonsillar space between the tonsil capsule andmuscular wall is dissected, completely removingthe tonsil. When performed in conjunction withsurgical removal of the adenoids, this procedureis referred to as adenotonsillectomy. If a childhas OSAS and has adenotonsillar hypertrophy,adenotonsillectomy is usually recommended asthe first-line treatment.13

In most children, adenotonsillectomy is effectivein treating OSAS and improving quality of life.Relative contraindications include very smalltonsils/adenoids, morbid obesity with small ton-sils/adenoids, bleeding disorders refractory to

Table 1Surgical therapies for OSAS in children

Therapy Population B

Adenotonsillectomy Children withenlargedtonsils and/oradenoids

HW

Partial tonsillectomy Children withenlarged tonsilswith or withoutadenoids

Sh

Tracheostomy Children with severeOSAS and no othertherapeutic option

H

Bariatric surgery Select obeseteenagers for whomother therapieshave failed

Sm

Craniofacial surgery(mandibular distractionosteogenesis,lip-tongue adhesion)

Children withmicrognathia,craniosynostosis,other craniofacialconditions

H

treatment, andmedical instability. The rate of com-plete resolution of OSAS after adenotonsillectomydepends on many factors. Retrospective studieshave found that adenotonsillectomy is highly effec-tive in reducing the apnea-hypopnea index (AHI)and 25% to 71% of children have a postoperativeAHI of less than 1 event per hour.14–16 Older age,obesity, and higher preoperative AHI are negativepredictors of successful resolution of OSAS afteradenotonsillectomy.11,14,16

Treatment of obstructive sleep apnea (OSA) iscost-effective. Adenotonsillectomy has been ass-ociated with a reduction in health care costs byone-third, a 39% reduction in emergency depart-ment visits, and 60% fewer hospital admissions.17

The efficacy of treating OSAS with adenotonsillec-tomy has not been compared with tonsillectomy oradenoidectomy alone, but in children withoutenlarged tonsils, particularly those younger than1 year, adenoidectomy is often performed withouttonsillectomy.Adenotonsillectomy is a relatively safe proce-

dure, and as many as 93% of patients have nointraoperative or postoperative problems.18 Themost common complications include pain andpoor oral intake, with more severe complicationsincluding hemorrhage, dehydration, infection, res-piratory complications, and atlantoaxial subluxa-tion. The rate of major complications, including

enefits Risks/Challenges

ighly effectiveell-tolerated inmost children

Common adverse effectsinclude pain, decreasedoral tolerance, rarehemorrhaging,respiratory complications

orter recoverytime and fewerpostoperativecomplications thanadenotonsillectomy

Efficacy in treatingOSAS less-established

Effect of tonsillarregrowth on OSASunknown

ighly effective Requires increased level ofsupport at home

Increased risk of significantcomplications

all studies showhigh short-termsuccess rate inselect populations

Significant complicationsNo long-term efficacy dataSuccess varies by center/type of surgery

ighly effective inselect population

Minimal long-termfollow-up data

Success varies by center/type of surgery

Significant complications

Table 2Nonsurgical therapies for OSAS in children

Therapy Population Benefits Risks/Challenges

Positive airway pressure(PAP)

Any child Strong evidence forefficacy, even if OSASsevere

Some will have troubletolerating

Few mask options forsome children

Nasal steroids &leukotriene receptorantagonists

Children with mild-to-moderate OSAS

Minimally invasive Overall weak evidenceLength of therapy

needed is unknownUnclear which children

will benefit

Rapid maxillaryexpansion

Children with narrowmaxilla or constrictedmaxillary arch,nonobese, withoutadenotonsillarenlargement

Therapy is short-term,minimally invasive

Few studies showingefficacy

Unclear which childrenwill benefit most

Oral appliances Unclear In some children, maybe better toleratedthan PAP

Dry mouth and dentaldiscomfort seen

Few studies showingefficacy

Can be expensive

Weight loss Older, obese children Noninvasive, good foroverall health

Can be performed inconjunction with PAP

DifficultNo evidence for

sustained resolutionof OSAS

Supplemental oxygen UnclearPossibly infants or

those with no othertherapeutic options

May prevent hypoxemia Does not treat airwayobstruction

May cause hypercapnia

Therapies for Children 485

hemorrhage and dehydration, ranges from 0.7% to3.1% depending on the surgical technique used.19

A study of 475 consecutive adenotonsillectomiesfound that 11.2% of children had some respiratorycomplication postoperatively, most commonlymultiple episodes of desaturation requiring anoropharyngeal or nasopharyngeal airway. Risk fac-tors for complications included young age, obesity,and high preoperative AHI.20 Both the AmericanAcademy of Otolaryngology-Head and Neck Sur-gery and the American Academy of Pediatricsrecommend that children at increased risk for res-piratory complications be observed overnight in afacility with experience in caring for children withairway problems.13,21 Children considered highrisk include those younger than 3 years and thosewith severe OSA, cardiac complications of OSAS,failure to thrive, obesity, craniofacial abnormalities,neuromuscular disorders, or current respiratoryinfections.

Although most otolaryngologists rely on clinicaljudgment to determine which patients requireadenotonsillectomy, and only 10% of children

undergoing adenotonsillectomy have a polysom-nography before surgery,22 several studies haveshown that history and physical examination aloneare unreliable in predicting the presence andseverity of OSAS in children.11,12,23 The AmericanAcademy of Otolaryngology-Head and Neck Sur-gery suggests that polysomnography should beperformed before adenotonsillectomy in childrenwith obesity, Down syndrome, craniofacial abnor-malities, neuromuscular disorders, sickle cell dis-ease, or mucopolysaccharidoses. Additionally,clinicians should advocate for polysomnographybefore adenotonsillectomy when the benefit ofsurgery is unclear or when tonsil size on examina-tion seems small in relation to the symptoms ofsleep-disordered breathing.21

Understanding the severity of OSAS before ad-enotonsillectomy is useful for several reasons.First, it allows anesthesia personnel and the sur-geon to better evaluate risk for perioperative com-plications. Second, it permits clinicians to plan forpostoperative management, which may includehospital admission or observation in an intensive

Cielo & Brooks486

care unit. Finally, it helps determine which patientsshould be reevaluated for residual OSAS aftersurgery.

Partial Tonsillectomy

In an attempt to decrease postoperative complica-tions related to adenotonsillectomy and reduce re-covery time, significant interest has been shown indeveloping less-invasive surgical techniques. Avariety of partial tonsillectomy procedures exist,including powered intracapsular tonsillectomyand radiofrequency tonsillectomy. Powered intra-capsular tonsillectomy uses a microdebrider andspares the tonsillar capsule. A variety of radiofre-quency techniques use heat to perform a similarfunction.Limited data are available comparing polysom-

nography outcomes using these newer tech-niques. For intracapsular tonsillectomy, onestudy of 14 children with moderate OSA foundthat 93% had an AHI of less than 1 event perhour at 4 to 8 weeks postoperatively.24 A smallretrospective study with several limitations com-paring intracapsular tonsillectomy with total tonsil-lectomy found no differences in the rate of residualAHI of greater than 5 events per hour.25 For radio-frequency tonsillectomy, one group reportedsimilar rates of snoring at follow-up comparedwith total tonsillectomy, but no follow-up poly-somnography was performed.26 Randomizedstudies comparing partial tonsillectomy surgerieswith traditional adenotonsillectomy, includingfollow-up polysomnography, are needed to eval-uate efficacy of these newer techniques as treat-ments for OSAS.Advantages to partial tonsillectomy include

shorter recovery times and fewer complicationsthan traditional adenotonsillectomy. Children areable to return to normal activity earlier, are lesslikely to require pain medication postoperatively,are able to return to full diet sooner, or have hadless postoperative bleeding than those who un-dergo traditional adentonsillectomy.27–30

Unlike traditional tonsillectomy, however, partialtonsillectomy is associated with a risk of tonsillarregrowth. Studies of children who underwent par-tial tonsillectomy have shown tonsillar regrowthrates between 7.2% and 16.6%, but have variedin the follow-up duration and type of proce-dure.31,32 The implications of this regrowth onsleep-disordered breathing are unknown.

Positive Airway Pressure

If OSAS persists after adenotonsillectomy or if ad-enotonsillectomy is not performed, positive airwaypressure (PAP) is often the next line of therapy.13

Therapy with PAP involves air that is pressurizedby an electronic device and delivered during sleepvia a nasal mask, acting as a pneumatic stent ofthe airway. PAP can be delivered as continuouspositive airway pressure (CPAP) or as bilevel pres-sure (BPAP). PAP is effective in eliminating OSASin children, even those younger than 2 years ofage, but adherence can be challenging even withclose follow-up, and parental report of PAP usecan be unreliable.33–35 PAP is titrated in the sleeplaboratory to determine the pressures required tonormalize breathing. Limited evidence is availableto guide clinicians in titrating PAP in youngchildren.36

Autotitration of PAP is not recommended forchildren, because the lower pressures theygenerate may be insufficient to properly triggerthe machine. Unlike adults, children may notneed PAP for life.37 Periodic retitration may berequired to assess the continuing need and effi-cacy of PAP, particularly if significant growth orweight gain or loss has occurred, surgical proce-dures have been performed, or in the presenceof other factors that may affect upper airway anat-omy or physiology.The challenges in treating children with OSAS

using PAP include finding the appropriate equip-ment and achieving tolerance of that equipment.Some evidence shows that behavioral therapycan improve adherence to PAP in children whoare willing to participate in this type of therapy.38,39

The availability of appropriate equipment for chil-dren with OSAS has also been a challenge, partic-ularly in younger patients. Machines that generatepressure for CPAP and BPAP are designedfor adults, and algorithms used in these machinesto adequately ventilate young children requiringmore advanced settings have not been well stud-ied. Smaller childrenmay not be able to adequatelytrigger a BPAP machine because of smaller pres-sures and tidal volumes. Furthermore, few nasalmasks are available that fit the faces of childrenwith OSAS, particularly those younger than 2 yearsand those with craniofacial abnormalities.Although most children who are able to effec-

tively use PAP do so without side effects, thetechnique has several potential complications.Children may experience skin erythema or break-down from poorly fitting masks or those appliedtoo tightly. Global facial flattening and midfacehypoplasia has also been observed.40 Nasal sym-ptoms, including congestion or dry nose, are rela-tively common and can often be treated byadjusting the machine’s humidification. Epistaxisis seen occasionally. Other potential complica-tions include headache, irritation of the eyes fromair leak, and aerophagia.

Therapies for Children 487

Pharmacologic Therapies

Nasal steroidsEnlarged tonsils and adenoids consist of hypertro-phied lymphoid tissue, and topical steroids may becapable of reducing this inflammation. In the pres-ence of residual lymphoid tissue from adenoidalregrowth after adenotonsillectomy or when adeno-tonsillectomy cannot performed, intranasal ste-roids have been considered to treat OSA. Onesmall study of children younger than 10 yearsfound that those randomized to nasal fluticasonepropionate had an AHI that decreased from 10.7� 2.6 to 5.8 � 2.2 in the treatment group, butincreased in the control group.41 A slightly largersimilar study of intranasal budesonide also dem-onstrated short-term polysomnographic improve-ment but not overall resolution of OSAS aftersteroid administration.42 Side effects include nasalirritation and epistaxis, and a potential risk existsfor adrenal and growth suppression if used long-term. The long-term efficacy of nasal steroids forOSAS and the length of therapy required remainunknown.

Leukotriene receptor antagonistsLeukotrienes regulate inflammation in the respira-tory system, and mediators of this pathway, suchas human cysteinyl leukotriene receptor 1, havebeen shown to be overexpressed in tonsillar tissueof childrenwithOSAS. Leukotriene receptor antag-onists such asmontelukast have been proposed totreat OSAS through their anti-inflammatory actionon this pathway. Two small trials (by the samegroup) of montelukast in children with mild OSASshowed modest improvement in AHI after 12 to16 weeks of therapy without significant sideeffects.43,44 Larger studies are needed to knowwhether these findings can be generalized to chil-dren with more severe OSAS or those who arenot surgical candidates.

Combination therapy with montelukast andintranasal steroids has also been studied. In astudy of children who had residual mild OSAS afteradenotonsillectomy, 22 patients who receiveddaily montelukast and intranasal budesonide hadreductions in AHI and improvement in severalother sleep parameters.45

Other Nonsurgical Therapies

Rapid maxillary expansionRapid maxillary expansion (RME) involves placinga dental appliance in the child’s mouth, which isworn during sleep. The device is connected tothe posterior teeth and provides lateral pressureon the maxillary surface of the mouth. This re-opens the midpalatal suture to increase the

transverse diameter of the hard palate over aperiod of several months. Over 2 to 3 weeks, thedevice is expanded through daily turning ofscrews, followed by a fixed retention phase, inwhich the device remains in place for an additional6 to 13 months.

RME has been proposed to treat OSAS in spe-cific pediatric populations, including those withconstricted maxillary arches. In a study of 31 non-obese children with upper jaw constriction butwithout adenotonsillar hypertrophy who had amean AHI of 12 events per hour, maxillary expan-sion for 10 to 20 days resulted in resolution ofOSAS, with all subjects having an AHI of lessthan 1 event per hour at follow-up.46 A smallerstudy of school-aged children with dental maloc-clusion showed more modest improvements inmore mild OSAS at a 24-month follow-up.47 RMEhas also been considered as a second-line therapywhen OSAS persists after adenotonsillectomy.One study of 31 prepubertal children with OSAwho had both tonsillar enlargement and a narrowmaxilla were sequentially treated with eitheradenotonsillectomy or RME, and then the otherintervention. Although 30 patients had persistentOSAS after one intervention, all but 2 had com-plete resolution of OSAS after both interventions,regardless of the order of intervention.48 Larger,more inclusive studies are needed to better definewhich pediatric populations will benefit from RMEand whether the benefit is sustainable.

Oral appliancesOral appliances are another dental option availablefor the treatment of OSAS. These devices, wornduring sleep, advance the mandible or tongue,increasing the size of the upper airway. ACochranereview found that in adults, oral appliances cancause a decrease in AHI, but are not curative inthose with more severe OSAS.49 A single smallstudy of children with mostly mild-to-moderateOSAS showed general improvement in AHI anddaytime symptoms but not resolution of OSASafter 6 months of orthodontic therapy.50 Reportedside effects of oral appliances include dentaldiscomfort and dry mouth.

Positional therapyAmong adults, 50% with mild and 19% with mod-erate OSAS can normalize their sleep-disorderedbreathing through sleeping in a nonsupine position.Devices are available that have been successful intreating adults with positional obstructive apneathrough preventing them from rolling on their backsduring sleep.51 Less evidence is available inchildren that positional OSAS exists, with somestudies actually showing more apnea in the lateral

Cielo & Brooks488

than supine position, and others not findingany correlation between sleep position andOSAS.52,53 No studies have evaluated the role ofpositional therapy in treating pediatric OSAS.

Weight lossWeight loss has been shown to be effective inimproving OSAS in adults, but not curing it.54 Ina study of 49 obese children aged 10 to 18 withOSAS, weight loss was associated with resolutionof OSAS in 71%.55 A previous study by the samegroup also found that weight loss improvedOSAS in obese teens.56 However, sustainedweight loss is difficult without continued interven-tion. The limited data available suggest that weightloss could be considered adjunctive therapy forolder children and teenagers with OSAS, espe-cially considering that adenotonsillectomy is lesslikely to be successful in this group. CPAP couldbe used simultaneously until OSAS is shown tohave resolved. If significant weight gain occurs,patients should be reevaluated for OSAS.

Supplemental oxygenAlthough it does not reduce airway obstruction,supplemental oxygen has been used to normalizeoxyhemoglobin saturation during sleep in childrenwith OSAS. In one small study of young childrenwith OSAS caused by adenotonsillar hypertrophy,supplemental oxygen was effective in increasingmean oxyhemoglobin saturation, reducing scora-ble obstructive apneas, and increasing rapid eyemovement (REM) sleep, but did not worsen alve-olar ventilation.57 Another small study of childrenaround the same age found no change in the num-ber of obstructive events, but 2 children had signif-icant elevation in end-tidal carbon dioxide with theaddition of supplemental oxygen.58 With thepaucity of data available, supplemental oxygenshould be used with caution in treating OSASbecause of concerns for hypercapnia, and itsinability to actually treat upper airway obstruction.Especially in young children who are normoxemicexcept for brief intermittent obstructive events,prolonged supplemental oxygen exposure canbe associated with toxicity, and the titration ofoxygen can be problematic.

Other Surgical Therapies

UvulopalatopharyngoplastyAlthough uvulopalatopharyngoplasty is commonlyperformed in adults with OSAS, no studies inchildren evaluating its efficacy include polysomno-grams. Case series of children who have under-gone uvulopalatopharyngoplasty for OSAS arelimited to patients with neurologic impairment,

and use parental report of symptoms as a metricof successful resolution of OSAS.59,60

TracheostomyTracheostomy is a highly effective means of treat-ing upper airway obstruction.61 Through simplybypassing the site of obstruction with an artificialairway in the trachea, this therapy is virtually100% effective in eliminating OSAS. Tracheos-tomy allows patients to receive PAP without theneed for a mask. However, the burden of a trache-ostomy in children is great, requiring increasedcare and having increased risk for complications,including tracheitis and death. Because of theinvasive nature of this procedure and alternativetherapies such as PAP, tracheostomy is usuallyreserved for children with refractory OSAS andno available alternative.

Bariatric surgeryA variety of surgeries exist to restrict stomach sizeor impair micronutrient absorption to aid in weightloss. Weight loss surgery can be effective in treat-ing OSAS in children, with success rates of 77% to100% in resolving OSAS, depending on AHIcriteria used.62 Limitations in these studies includeminimal polysomnographic follow-up and lackof long-term polysomnographic follow-up. TheRoux-en-Y gastric bypass, which can be per-formed laparoscopically, creates a small gastricpouch and bypasses the duodenum and proximaljejunum. Laparoscopic adjustable gastric bandsurgery places a restrictive band around the prox-imal stomach, but has not been approved for use inchildren in the United States. Bariatric surgery canbe effective in sustainedweight loss, but complica-tions can be serious, including wound infection,stricture, dehydration, micronutrient deficiency,peripheral neuropathy, beriberi, and death.63

SPECIAL POPULATIONSInfants

Infants are particularly susceptible to OSASbecause of the laryngeal chemoreflex and a REM-predominant sleep state distribution. Diagnosisand treatment of OSAS in infants are challengingfor several reasons. First, no well-establishednormative polysomnographic respiratory data existfor infants, making the diagnosis of OSAS some-what problematic. With regard to management,adenotonsillectomy has been shown to be lesseffective in resolving OSAS in young children. Onestudyof 73 children younger than24months under-going adenotonsillectomy for OSAS found that66% had moderate to severe residual OSAS post-operatively.64 Younger children and infants arealso more likely to have respiratory complications

Therapies for Children 489

postoperatively, and should be observed in-hospital after adenotonsillectomy. CPAP can beused to effectively treat OSAS in infants, althoughthe lack of appropriately sized interfaces and head-gear available for conventional CPAP units canmake this particularly challenging. Some centerschoose to initiate CPAP in a hospital setting to facil-itate acceptanceof amask, whereas othersmay beable to do this at home with appropriate parentaland medical support.

Children with Craniofacial Abnormalities

Children with craniofacial abnormalities, such asPierre Robin sequence, craniosynostosis, andconditions causing midface hypoplasia, are atincreased risk for OSA because of isolated or mul-tiple sites of upper airway narrowing.65 Even non-syndromic isolated cleft palate significantlyincreases the risk of OSAS in young children.66 Inchildren with micrognathia, OSAS can be seenbeginning in infancy because of tongue-basedairway obstruction. Conservative therapies in-clude prone positioning, nasopharyngeal tubeplacement, and CPAP, but all can be difficult forparents to initiate and may be ineffective in elimi-nating OSAS. Surgical therapies include lip-tongue adhesion, in which the mucosa andmuscles of the lip and tongue are surgically fixed,and mandibular distraction osteogenesis (MDO),which involves osteotomies of the mandibularrami and the placement of a distraction apparatusthat is extended gradually over several weeks.Case series of these procedures suggest thatMDO may be more successful at resolvingOSAS,67 but this surgery carries the risk of severalcomplications, including nerve damage and infec-tion. For children with midface hypoplasia causedby craniosynostosis, gradual frontofacial distrac-tion or single-stage midface advancement areavailable surgical therapies, but data regardingimprovement in OSAS are limited and variable.68

Adenotonsillectomy should be considered in olderchildren with craniofacial abnormalities and OSAS,although some populations have a high risk for re-sidual OSAS. Tracheostomy should be consideredin patients in whom surgical correction is notpossible, who have multiple sites of obstruction,and/or for whom CPAP trials have failed.

Obese Children

As in adults, obesity is an independent risk factorfor OSAS in children, and the combination ofOSAS and obesity can exacerbate the risks asso-ciated with both disorders.69 Although not ascommon as in adults with severe obesity, the prev-alence of OSAS in adolescents with body mass

index greater than 40 kg/m2 has been shown tobe approximately 50%, with boys at greater riskthan girls.62 A weight loss plan may be used inconjunction with medical or surgical therapydirected at OSAS. Adenotonsillectomy should stillbe considered first-line therapy in obese childrenwith enlarged tonsils or adenoids, particularly inyounger children. If significant weight loss or gainis seen, repeat polysomnogram or PAP titrationshould be performed to reevaluate the need forCPAP and the appropriate pressure required.

Down Syndrome

Children with Down syndrome are at increased riskfor OSAS because of midface hypoplasia, reducedupper airway volume, glossoptosis, decreased up-per airway tone, and increased rates of obesitycompared with children without Down syndrome.Similar to what has been published in studies ofotherwise healthy children with adenotonsillar hy-pertrophy, children with Down syndrome usuallyhave improvement of OSAS after adenotonsillec-tomy, but only between 27% and 54% havecomplete resolution according to polysomnogra-phy.70–72 Children with Down syndrome are alsoat higher risk for complications after adenotonsil-lectomy, and postoperative in-hospital monitoringand follow-up polysomnography should bestrongly considered.73,74 In children with Downsyndrome who are unable to undergo adenotonsil-lectomy or who have persistent OSAS postopera-tively, CPAP treatment should be considered,although adherence can be challenging.75 Othersurgical procedures that have been reported inchildren with Down syndrome include MDO, mid-face advancement, genioglossus advancement,tongue reduction surgery, or tracheostomy.

Prader-Willi Syndrome

In Prader-Willi syndrome (PWS), a period of failureto thrive and hypotonia is seen during infancy, fol-lowed by hyperphagia, obesity, and short staturein older children. Although growth hormones canbe an effective and important part of therapy forthese children, patients with PWS should be moni-tored carefully, particularly those who are sick withrespiratory illnesses. Respiratory disorders are aleading cause of death in infants with PWS, andcase reports exist of infants who have died fromrespiratory illnesses soon after starting growthhormone.76 Studies evaluating the effect of growthhormone on sleep-disordered breathing in chil-dren with PWS show mixed results, dependingon the age of children studied and the length oftime patients are followed.77,78 One small caseseries of children with PWS with OSAS who

Cielo & Brooks490

underwent adenotonsillectomy showed that mostof those with mild OSAS had normalization ofAHI postoperatively, but a few patients with moresevere OSAS had no improvement or worseningafter surgery.79 This finding suggests that childrenwith PWS and OSAS who have adenotonsillarenlargement may be good candidates for adeno-tonsillectomy, but that they should have a repeatpolysomnography postoperatively to reevaluatefor residual OSAS.

SUMMARY

The mainstays of therapy for OSA in children areadenotonsillectomy and PAP. Nonsurgical thera-pies, including anti-inflammatory medications,dental devices, and weight loss, may be useful inspecific circumstances. Airway surgery has beenshown to be effective in children with craniofacialabnormalities, but not in the general pediatricpopulation. Children with Down syndrome, cranio-facial abnormalities, obesity, or PWS are atincreased risk for OSAS and may require morefrequent polysomnography and specific therapies.

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