SS Relief of Upper Airway Obstruction With Mandibular Distraction Surgery Long-term Quantitative Results in Young Children Sandra ¥. Lin, MD; Ann C. Halbower, MD; David E, Tunkel, MD; Craig Vanderkolk, MD Objective: To evaluate the long-term benefits of man- dibular distraction on sleep-related upper airway obstruc- tion in young children with mandibular hypoplasia, Results: Of the 5 children, 3 were cured of upper air- way obstruction as documented by polysomnography with an apnea-hypopnea index of less than 1.5 and no snoring, The fourth child had primary snoring without apnea, The filth child had severe obstruc- live sleep apnea, with an apnea-hypopnea index of Design: Cross-sectional study. Subjects were exam- ined for sleep-disordered breathing using medical his- tory, physical examination results, and a written ques- Uonnaire, Subjects underwent standard overnight polysomnography, during which measures of sleep- disordered breathing were collected. Conelusions: Most children who undergo mandibular distraction for upper airway obstruction associated with mandibular hypoplasia demonstrate significant clinical improvement of obstructive sleep apnea. How- ever, those children who continue to have symptoms: of sleep-disordered breathing after surgery should undergo polysomnography for evaluation of persistent obstructive sleep apnea. Tertiary care hospital Patients: Five children with upper airway obstruction from craniofacial anomalies treated with mandibular dis- traction, with a minimum follow-up of 12 months. Main Outcome Measures: Apnes-bypopnes index, oxygen saturation nadir, and peak end-tidal carbon di- oxide value. Arch Otolaryngol Head Neck Surg, 2006;132:437-#41 Author Affiliations: Departments of tolaryngology-Head & Neck surgery (Drs Lin and Tunkel) and Plastic Surgery (r Vanderkolk) and Eudowood Division of Petrie Respiratory Sciences (DrHalbowe), Hopkins School of Medicine Ballimore, Md ANDIBULAR DISTRAC- tion has been used in children with upper airway obstruction from mandibular hy- poplasia since the first reported series, ‘was published in 1992,” Most children treated with this technique have either unilateral or bilateral mandibular hypo- plasia due to hemifacial microsomia, other syndromic disorders, or the Pierre Robin syndrome. Mandibular distrac- tion may relieve sleep-related upper air- way obstruction and allow some chil dren to avoid tracheotomy. It can also be used (o facilitate earlier decannula- tion in those children who have already undergone tracheotomy children with craniofacial abnormalities are at high risk for persistent obstruc- tive sleep apnea (OSA). Symptoms of (OSA include snoring, gasping, respira- tory pauses, and restless sl cal examination is sensitive but not spe- These same The Johns cific for OSA Polysomnography is the gold standard for the evaluation and diagnosis of OSA, Published series'* have demon- strated the short-term efficacy of man- dibular distraction in the treatment of OSA in patients with eraniofaeial disor- ders. However, the postoperative sleep study data in these series were typically obtained shortly alter surgery. Other studies' show improvement of airway obstruction with this surgery without complete cure of OSA, There is a pau city of literature regarding the long- term effectiveness of mandibular dis traction in the prevention and treatment of OSA in children with craniofacial anomalies. Our objective was to exam- {ne the patients with mandibular hypo- plasia who have had mandibular dis- long-term benefit in relieving upper air- way obstruction and OSA, fon 10 assess the (©2006 American Medical Association. AI rights reserved, ‘Downloaded From: http:/jjamanetwork.com/pdfaccess ashx turl-/dataljournals/otol/11913 by elena prunici on 02/27/20171. yeh we ang 2 ted tops rating eras paige 4. ed gp wie sbi {yeh hts ising 5. yeh satis ge 6 yeh as teqayen pg 1 t a 1 hd wt we spon ‘yh ean i mt hl sing ssi stan posters 1 Whi shaped ing te 4h bas tog is at sou ea 12. Myehldhs roy Arar bi un archi al ad da prs wit ati Cl oe ngs Amos Samana Myst Samatinas ae ngs Amos Samana ae Myst Samatinas ae ngs Amos Samana ae Myst Samatinas ae ngs Amos Samana ae Myst Samatinas ae ngs Amos Samana ae Myst Samatinas ae ngs Amos Samana ae Mya Hm Samatinas ae Figur. Pediatric obstructive Es }- After obtaining approval from The Johns Hopkins Schoo! of Medicine insitional review board, lige patients were Wen tiled by the cranofacil surgeon (CV) from his cline pa tient databace, Patents were recruited Grom the pede cra ofacal clini and otolaryngology clinic. Subject eligible for thisstady included children aged» to 14 years with crantola cial anomalies who had undergone mandibular distraction for sirvay obstruction, and had undergone surgery a minimum of 12 months before recruitment, Exclusion criteria inched non English-speaking patients and patient who are tracheotomy dependent The study subjects wee interviewed and examined by the investigators or signs and symptoms of sleep disordered breath ing, by medical history. a physial examination, anda weiten tucslonnaire. History of pharyngeal surgery or other teat thent for OSA was recorded. A physical examination was Per formed with particular attention t the upper airway charac teristics, including mandibular postion and size and tosilar size, The pallens parent or carclaker completa wri ques Uionnaie (the pediatric OSA symptom questionnale) regard ing symptoms of OSA. The questionnaite addressed 12 poten talsymptoms of OSA and thefrequency of occurence (Higure). The quesionnare was numerically scored a follows O nde cates never, sometimes; 2, almost always; and 3, always. The possible otal score range on the questionnaire was from Oto Bo. While this questionnaire has not been formally validated itwas used to Taclitate a standard History and review of signs and symptoms Palen then underwent standard overnight polysomnog- raphy ina pediatric facility accredited by the American Board of Sleep Medicine. The study was performed by 4 trained pediatic polysomnographic technician, sing san- ded techniques. A parent remained inthe same rot asthe child throughout testing. During the slep study, surface electrodes and monitoring devices (acquired digitally by ‘lice 3 and Alice 4 Allain, Ga) measured signals fom the central clectroencephalogeam, the right and lft eleciro- oclograms, the surface cectromyelogram, and an elecio- Cardiogramn; chest and abdominal wall motion; pulse oxim- tity; and end-tidal Peo, (Novamettix, Wallinglord, Cont). Aiflow was measured by oronasl thermistor in ll children. in addon, siflow was monitored using nasal pressure to abs a uanitative signal (Protech, Mukilteo, Wash). All studies were monitored wih real-time videotape for motion pre snptom questo analysis and snoring recording, The medical history was btained and physical examination, questionnalre, and poy somnography were performed in these patients for research Purposes ony ‘Although no standard pediatric criteria exist forthe diag- nosis of pediatric OSA, pele erteria as suggested bythe ‘American Thoracic Solty." were used for evaluating sleep SHrictue and respiratory patierns observed in the slep stu ies Sleep architecture was recorded according to standard criteria.” Respiratory analysts consisted of defining apness 4 4 cessation of allow for 2 or more breath cycles Hypop- nea was defined asa visual reduction in allow over 2 or tmore respiratory cycles, accompanied by a 3% or greater decreae in oxygen saturation ot terminated by an arousal.” Hypopneas were measured preferentially with the nasl pres- sure monitor 2 quantitative measure of respiratory low that. tnore reliably demonsratesarlow Uinitation compared with the thermistor" Obstructive apneas are indicated by the presence of paradoxical breathing movements and/or snor- Ing n assoclation with decreased sirflow signals for 2 or amore respiratory cycles, Central apneas are without evidence of respiratory effort, indicated by paradoxical thoracoab- dominal movements, and are not fecorded in the apnea hypopnes index (AHI). Mixed apneas are a combination of central and obstructive events The AHI is defined a the fhumber of obstructive spneas, mixed apnea, and hypopness bccurring per hour of sleep. although standard pediatric sleep scoring criteria have not been established, to our knowledge, Healthy nonsnoring children without craniofa- cial anomalies are described as having an AHI mich less than [event per hour" Patients were considered to have tno OSA ifthe AH was less than 13 and there was no snor- ing primary snoring ithe AHI was less than 15 and snor- ing was present, mild OSA, ifthe AHI was 1.510 50, and signlicant OSA, i the AHI was greater than 59. ’A pulse oximeter (asim Corporation, tvine, Calf) was sed, which has ashort averaging time and, therefore, detects txyhemoglobin changes sensttively with less movement art fact Oxygen saturation measurements obscured by artilact(de- tected by lack of synchrony between the pulse wave and lec- trocardiogram) were deleted from analysis. The oxy} ssluration nadir isthe lowest saturation tabulated, Studie of normal polyzomnographi respiratory values in children in- dicate that onjgem satraton rarely decreases oles than 95% except during cental apnea. (©2006 American Medical Association. AI rights reserved, ‘Downloaded From: http:/jjamanetwork.com/pdfaccess ashx turl-/dataljournals/otol/11913 by elena prunici on 02/27/2017Tale, Demographi an Cnia Chracerste o dal Paents conte a fesasugeme 7 oF = 2 05 fone a 8 & & a attain es " " " te Cana de Prt ER seh sana Ae Ae ov on f . e : : tae see a a a a Sean 2 ‘ i 2 a ma 2 a 8 2 -bbrvition: A, pneshypopnen index GED, gaetronsophagea refx daze, P RESULTS | saturation nadir of 8%. After full away endoscopy and, ‘adenotonsillectomy, the patient was monitored in the hos- pital with her tracheotomy tube plugged and had no signs of sleep-disordered breathing. She subsequently under went decannulation and remained symptom free for more than 1 year. Approximately 6 to 12 months before our study, the patient began developing symptoms of noc- tural airway obstruction. The results of the polysom- rogram, done as part ofthis study, were communicated to this family and to the treating physicians: her condi- tion is successfully maintained using bilevel (biphasic) positive airway pressure The median oxygen saturation for the sleep studies was 87%, The median peak end-tidal carbon dioxide level was 54mm Hg. Eight patients identified by the craniofacial surgeon (C.V.) {rom his clinical database were eligible to partiipate in the study. Examination of the clinical database also revealed that no patients who had undergone mandibular distrac- tion longer than 12 months ago weresill tracheotomy de- pendent, Five patients agreed to participate and com- pleted the study (Falole). One patient who declined the study cited inconvenience; the other 2 agreed to the study but did not complete their sleep studies despite several al- tempts at scheduling. The median age atthe time of study \was 49 months, Four patients were born with Pierre Robin syndrome, and the other was born with severe microgna- thia without cleft palate, All patients underwent mandibu- lar distraction at our institution, performed by one of us (EX) watheindcatonoltespeatoy dst tem op. JSST per airway obstruction. The median follow-up from sue- gery was 47.5 months. One patient had undergone rache- Children with craniofacial anomalies and mandibular hy- tomy, but then underwent decannulation 34 months alter _poplasia are at high risk for upper airway obstruction in mandibular distraction, Two patients clinically continued the neonatal period. Symptoms range in severity [rom in- to have severe retrognathia alter distraction, and the re- creased work of breathing and OSA to continuous up- maining 3 had mild retrognathia. One patient underwent per airway obstruction, Before mandibular distraction, apriortonsillectomy, and the other patients had +1 or+2 children with craniofacial anomalies and severe airway tonsils. Dental occlusion was abnormal in 3 patients, and obstruction required endotracheal intubation, long- 4 patients reported abnormal dentition. No patients com- term nasal alrways, craniofacial surgery, and often tra- plained of lip numbness. The median size of averbite was cheotomy’ 44mm (range, 3-12 mm). None of the children were obese In 1902, MeCarthy et al! described the first series of or had neurologic problems, patients to undergo mandibular distraction, which ean The median score on the pediatric OSA symptomques-_help avoid tracheotomy in some children with mandibu- Jonnaire was 19 (range, 7-24) of a maximum possible lar hypoplasia. Mandibular distraction involves the sur- score of 36, gical creation of osteotomies in the mandible. The man- The patients underwent standard overnight polysom- _ dible is then serially distracted in small amounts with a nography. One patient had no snoring or OSA (AHI<1.5 rigid fixator device. Bone forms between the separated and no snoring), and 3 patients had primary snoring (AHI bone surfaces, with distraction rates of approximately 1 1.3 with snoring). mun/d, The lengthening of the manclible corrects the hy- ‘One patient had severe OSA, with an AHI of20.2. The _poplasia of the mandible, with improved supraglotticand patient had undergone tracheotomy and had the high- pharyngeal airway support. The original article by est score (score, 24) on the OSA symptom question- McCarthy etal described the use of mandibular distrac- naire. The eategiver or this patient answered always for on to improve aesthetic appearance in children with era- items regarding snoring, gasping, stopping breathing, rest-__niofscial anomalies, Several subsequent articles have doct- less sleep, and mouth breathing on the questionnaire. This mented the use of mandibular distraction to improve the child had undergone polysomnography with her trache- airway in children with mandibular hypoplasia. tomy tube plugged 2 yeats before our study and had « Carls and Sailer described a series of 7 children with respiratory disturbance index of 3.7 with an oxygen de- severely hypoplastic mandibles and associated respira- (©2006 American Medical Association. AI rights reserved, ‘Downloaded From: http:/jjamanetwork.com/pdfaccess ashx turl-/dataljournals/otol/11913 by elena prunici on 02/27/2017tory distress who underwent distraction. All 7 had relief oftheir alway symptoms after surgery. The distraction allowed for the decannulation of the 2 paticnts in this series who underwent prior tracheotomy. Sidiman etal! described a series of L1 micrognathic children with tra- cheotomnics who underwent successful dccannilation a- ter mandibular distraction ln their report, patients were considered to have astccessful outcome fthey were able toundergo decannilation, but polysomnography was not routinely done postoperatively The successful use of mandibular distraction to treat OSA in children with craniofacial anomalies has been documented in the literature.°"” Morovie and Monaste- rio® described 7 children younger than 18 months who were treated with mandibular distraction. Postdistrac- tion polysomnograms showed an improvement in the ap- nea indexes forall children, but these children contin- ued to have obstructive events, ranging from Sto 18 events perhour, indicating persistent OSA alter surgery. Bell and Turvey* described 8 patients with severe migrognathia who were treated with distraction. Pour were success- fully treated, as 2 of 3 patients with tracheotomies un- derwent decannulation and another 2 children were ‘weaned from supplemental oxygen. However, no sleep study data were reported for this study. Monasterio et al’ described 15 patients with severe OSA with the Pierre Robin syndrome who had improvement of their apnea as documented by polysomnography that was per- formed a mean of 3.2 months alter surgery. Cohen etal” described a series of 20 patients, 12 who were studied with preoperative and postoperative polysomnograms. They reported an improvement in the mean AHI from, 25.24 to 1.72; their postoperative sleep studies were per- formed before discharge from the hospital. None of these studies report long-term results of mandibular distrac- tion as documented by polysomnography. To our knowledge, the present stud isthe first report oflong-term polysomnographic data after mandibular dis- Uiaction, ALour institution, neonates presenting with man- dlibular hypoplasia and respiratory distress without appar ent neuromotor disease of complex craniofacial anomalies are candidates for mandibular distraction, All patients who have undergone distraction at ou institution have either avoided tracheotomy or, ifthey did require tracheotomy’ subsequently underwent decannulation. We have not done routine postoperative polysomnography, but have used clinical relief fairway obstruction, extubation, and close intensive care monitoring with normal oxygen satura- dons as primary outcomes for stich surgery. We have per- formed sleep stuclies for symptomatic patients alter sur- gery. For the 5 patients in our study, 2 underwent sleep studies before their participation in our study, 1 was being, observed at another institution, 1 had been scheduled tovice for a sleep study but did not follow through be- fore he was recruited for our study, and 1 had no docu- mented airway or sleep complaints in the medical rec- ord. We routinely admit patients for monitored observation with the iricheotomy tube corked hefore de- cannulation, and we also obtain sleep study results with the tracheotomy tube capped. ‘Our study has demonstrated the long-term improve- ment of OSA in 4 of 5 children with « median follow-up of 47.5 months. OF these 5 patients, 1 had severe OSA. documented, with an AHI of 20.2. This patient was the only patient who completed the study who underwent a previous tracheotomy; this patient also had the highest score on the pediatric OSA symplom questionnaire. In four simall sample of patients who underwent mandibu- lar distraction, itis not possible to draw conclusions on the sensitivity and specificity of either clinical assess- ment or polysomnography for the diagnosis of OSA af- ter this procedure. However, previous studies" of chil dren who have not undergone mandibular distraction have shown that medical history and physical examination alone are not reliable in assessing for OSA. Polysomnog- raphy is the gold standard for evaluation of OSA and, therefore, should be olfered to children with obstruc- tive symptoms alter distraction, ‘Our study is limited by the small sample size. Addi- ional long-term prospective evaluation of more chil- dren undergoing mandibular distraction is necessary 10 fully understand the benefits and limitations of this surgery In conclusion, most young children with upper airway obstruction from mandibular hypoplasia have long-term reliefof OSA after mandibular distraction, In children with continued or recurrent symptoms of sleep-disordered breathing, clinical evaluation and polysomnography are recommended, Submitted for Publication: July 18, 2005; final revision received October 31, 2005; accepted November 28, 2005. Correspondence: Sandra Y. Lin, MD, Department of Oto- laryngology-Head & Neck Surgery. The Johns Hopkins School of Medicine, 601 N Caroline St, Room 6254, Bal- timore, MD 21287 (slin30@jhmi-edu). Author Contributions: Dr Lin had full access to all the data in the study and takes responsibility for the integ- rity of the data and the accuracy of the data analysis. Financial Disclosure: None. Punding/Support: This study was supported by grant ‘MO1-RR00052 from the National Center of Research Re- sources, National Institutes of Health (General Clinical Research Center, The Johns Hopkins School of Medi cine), Role of the Sponsor: The funding body had no role in data extraction and analyses, in the writing of the manu- script, oF in the decision to submit the manuscript for publication. Acknowledgment: We thank Kathryn A, Carson, SeM, senior biostatistician, Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, Bal- Limore, for her assistance on this project. —_ EES} 1. MeCany J, Seer J Karp et. Lengthening ha hanan man by radial can Pst acon Surg. 10928010, 2 hie SE, az 5, Roberson. Cn istry ar physical xanga re 1 agnose pda cbs sip grehypopnen snore ays ‘eres of haat. Olrngot ad Mek Surg 200,31: 87-82 4 Cars Saler Seven yes cnc xperine wah macular tation inchlen. J Granimantote Sur. 186 26197 208 4 SidmanJ0, SangsonD Tampon Disraconastcgonei othe mantle faraway abstr in eran Layegoscope 201 1T-157-146 (©2006 American Medical Association. AI rights reserved, ‘Downloaded From: http:/jjamanetwork.com/pdfaccess ashx turl-/dataljournals/otol/11913 by elena prunici on 02/27/2017aro 05, Monasrio _Disbacon osteogenesis for obstrcv apes in Fates th congenital roti malate. last cont Surg 200 fasaaee ran, all, Tr TA Shktal adancarnt ote estmentfbetuctve ley ‘pean chr. et Pale Cite 200 38 47-15 anasto, Orca Mk Mina F Ysa, Osacionastogees ine tin saqunce andre spt problemsin chien. JCranotc Sug aan 137083, Villars Mau, rayon BH, Longer MT Meaty 4, Ely cau Tan whist manda acon fo eacheeony spend pte Pst const Sr, 1295 103:8-59 Cahn oss DA Burstein FD, eal State expansion contin wit so {eau edton a ewe of abst slap apea in in ys loge. Crtyga Aad eck Su. 1B TEAR. aban $8, Simms © Bust FD Manus ditacon etoganesi inh uarar of upa away obucon ncn wih ital tries PhseRecansr Surg. 1086101312318. Cah SR, Sens Busan FD, Thomsen J. Aanabes to achestory in infants anderen wih cbsrucive sep apne. Pea Sug. 109838: nee? Jame, Lin ML Mandible consti in chien it bettie lap spat ronan, Plt ont Su, 187100131113. Arian Thi Sooaty Sada andideatons or carepdonryskep Studs in chile AJ Resp it CaM 10065158 266578. -acischatn les A nl of Stored Terni, Techigse and Seong System or Sep Sages of Human Sublets Los Angie Cali UCL ran norman Saran Reser etn: 1064 EEG arousal: ering les and als: rainy repr rom he Stop Driers tas TasFre fh Anetean Sap sores Asotin. Sop. sanass73teu, Eptin MD, Cie SA Hanuma RC, Otto ot pps stance same using a nasal canlressure wanda. Chest 2000117107. a7. ie 5, Tauran 8, rene tt Norma psonnuraphi spt a: es mchen and ales. Chest 204 25872 378 ‘Mares CL, OK Basi, toma pelysagaphie values for halen ad adlscans. An Ry esis 1015 1235-129. Broil RT, Mail, Lamas, Mtr KR, Lacan R, Ducharme FAL Noctua ple vir an abr ering modal for peda ob suc sleep ape, Pedr, 200010505412 Trial Registration Required In concert withthe Intemational Commie of Medial Journal Editors (ICME), Archives of Otolaryngology died & Neck Surgery willrequteastconditonof cone sideration for publication, registration of all ral in @ public ral rity Gah ae hp/Ciieal Teas po ot Bup/feontoledirals com) Tess mnt be registered at or before te ose of patent enrollment This plicyap- plies to any clinical al stating enrollment str uly 1/2003. Fortra that began envaliment before his date registration wl be required by September 13,2003. be- fore considering the al for pubiation. 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