Mandibular distraction osteogenesis (MDO) and continuous positive airway pressure (CPAP) may each have a role in effectively treating tongue-based airway obstruction (TBAO) in Robin sequence (RS). This study describes longitudinal outcomes after treatment of TBAO with CPAP and/or MDO.
Design
Retrospective cohort study.
Setting
Tertiary Pediatric Hospital.
Patients
A total of 129 patients with RS treated with CPAP and/or MDO from 2009 to 2019 were reviewed. Subjects receiving baseline and at least one follow-up polysomnogram were included. 55 who underwent MDO ± CPAP and 9 who received CPAP-only treatment were included.
Main Outcome Measures
Patient characteristics, feeding, and polysomnographic data were compared and generalized linear mixed modeling performed.
Results
Baseline obstructive apnea–hypopnea index (OAHI) was greater in the MDO-treated group (median x˜ = 33.7 [interquartile range: 26.5-54.5] than the CPAP-treated group (x˜ = 20.3[13.3-36.7], P ≤ .033). There was significant reduction in OAHI following treatment with CPAP and MDO modalities, P ≤ .001. SpO2 nadir after MDO was lower in syndromic (x˜ = 85.0[81.0-87.9] compared to nonsyndromic patients (x˜ = 88.4[86.8-90.5], P ≤ .005.) CPAP was utilized following MDO in 2/24 (8.3%) of nonsyndromic and 16/31 (51.6%) of syndromic subjects (P ≤ .001,) for a median duration of 414 days. Three patients (5%) underwent tracheostomy, all had MDO. Nasogastric tube feeding at hospital discharge was more common following MDO (44, 80%) than CPAP-only (4, 44.4%, P ≤ .036), but did not differ at 6-month follow-up (P ≥ .376).
Conclusions
CPAP appears to effectively reduce obstructive apnea in patients with RS and moderate TBAO and be a useful adjunct in syndromic patients following MDO with improved but persistent obstruction.
RobinP. A fall of the base of the tongue considered as a new cause of nasopharyngeal respiratory impairment: Pierre Robin sequence, a translation. 1923. Plast Reconstr Surg. 1994;93(6):1301‐1303.
2.
DonnellyLFStrifeJLMyerCM, III. Glossoptosis (posterior displacement of the tongue) during sleep: a frequent cause of sleep apnea in pediatric patients referred for dynamic sleep fluoroscopy. Am J Roentgenol. 2000;175(6):1557‐1560. doi:10.2214/ajr.175.6.1751557
3.
GangopadhyayNMendoncaDAWooAS. Pierre Robin sequence. Semin Plast Surg. 2012;26(2):76‐82. doi:10.1055/s-0032-1320065
4.
MackayDR. Controversies in the diagnosis and management of the Robin sequence. J Craniofac Surg. 2011;22(2):415‐420. doi:10.1097/SCS.0b013e3182074799
5.
AndersonICSedaghatARMcGinleyBMRedettRJBossEFIshmanSL. Prevalence and severity of obstructive sleep apnea and snoring in infants with Pierre Robin sequence. Cleft Palate Craniofac J. 2011;48(5):614‐618. doi:10.1597/10-100
6.
GilhoolyJTSmithJDHowellLLDeschaineBLRicheySL. Bedside polysomnography as an adjunct in the management of infants with robin sequence. Plast Reconstr Surg. 1993;92(1):23‐27.
7.
BreugemCCEvansKNPoetsCFMehendaleFVSaalHMBasartHMurthyJ. Best practices for the diagnosis and evaluation of infants with robin sequence: a clinical consensus report. JAMA Pediatr. 2016;170(9):894‐902. doi:10.1001/jamapediatrics.2016.0796
8.
ReddyVS. Evaluation of upper airway obstruction in infants with Pierre Robin sequence and the role of polysomnography—review of current evidence. Paediatr Respir Rev. 2016;17:80‐87. doi:10.1016/j.prrv.2015.10.001
9.
MarcellusL. The infant with Pierre Robin sequence: review and implications for nursing practice. J Pediatr Nurs. 2001;16(1):23‐34. doi:10.1053/jpdn.2001.20550
10.
PurnellCAJanesLEKlosowiakJLGosainAK. Mandibular catch-up growth in Pierre Robin sequence: a systematic review. Cleft Palate Craniofac J. 2019;56(2):168‐176. doi:10.1177/1055665618774025
11.
AlbinoFPWoodBCHanKDYiSSeruyaMRogersGFOhAK. Clinical factors associated with the non-operative airway management of patients with robin sequence. Arch Plast Surg. 2016;43(6):506‐511. doi:10.5999/aps.2016.43.6.506
12.
AmaddeoAAbadieVChalouhiC, Kadlub N, Frapin A, Lapillonne A, Leboulanger N, Garabédian EN, Picard A, Fauroux B.Continuous positive airway pressure for upper airway obstruction in infants with Pierre Robin sequence. Plast Reconstr Surg. 2016;137(2):609‐612. doi:10.1097/01.prs.0000475799.07597.23
13.
DouglasB. The treatment of micrognathia associated with obstruction by a plastic procedure. Plast Reconstr Surg (1946). 1946;1(3):300‐308. doi:10.1097/00006534-194611000-00007
14.
GoldsteinJAChungCPaligaJT, Cielo C, Marcus CL, Lioy J, Bartlett SP, Taylor JA.Mandibular distraction osteogenesis for the treatment of neonatal tongue-based airway obstruction. J Craniofac Surg. 2015;26(3):634‐641. doi:10.1097/SCS.0000000000001416
15.
McCarthyJGSchreiberJKarpNThorneCHGraysonBH. Lengthening the human mandible by gradual distraction. Plast Reconstr Surg. 1992;89(1):1‐8. discussion 9–10.
16.
ZhangRSHoppeICTaylorJABartlettSP. Surgical management and outcomes of Pierre Robin sequence: a comparison of mandibular distraction osteogenesis and tongue-Lip adhesion. Plast Reconstr Surg. 2018;142(2):480‐509. doi:10.1097/PRS.0000000000004581
17.
BerryRBQuanSFAbreuARHardingSMMarcusCVaughnBV. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications. Darien, IL: American Academy of Sleep Medicine; 2020.
18.
KadakiaSHelmanSNBadheyAKSamanMDucicY. Treacher Collins syndrome: the genetics of a craniofacial disease. Int J Pediatr Otorhinolaryngol. 2014;78(6):893‐898. doi:10.1016/j.ijporl.2014.03.006
19.
KlazenYPCaronCSchaalSC, Borghi A, Van der Schroeff MP, Dunaway DJ, Padwa BL, Koudstaal MJ.What are the characteristics of the upper airway in patients with craniofacial microsomia?J Oral Maxillofac Surg. 2019;77(9):1869‐1881. doi:10.1016/j.joms.2019.03.017
20.
PosnickJCRuizRL. Treacher Collins syndrome: current evaluation, treatment, and future directions. Cleft Palate Craniofac J. 2000;37(5):434. doi:10.1597/1545-1569(2000)037<0434:TCSCET>2.0.CO;2
ChungCUYuJWBastidasNBartlettSPTaylorJA. Utility of the ultrasonic scalpel in mandibular distraction osteogenesis. J Craniofac Surg. 2012;23(5):1279‐1282. doi:10.1097/SCS.0b013e31825196b4
23.
FloresRLGreathouseSTCostaMTahiriYSoleimaniTTholpadySS. Defining failure and its predictors in mandibular distraction for Robin sequence. J Craniomaxillofac Surg. 2015;43(8):1614‐1619. doi:10.1016/j.jcms.2015.06.039
24.
RogersGFMurthyASLaBrieRAMullikenJB. The GILLS score: part I. Patient selection for tongue-lip adhesion in Robin sequence. Plast Reconstr Surg. 2011;128(1):243‐251. doi:10.1097/PRS.0b013e318217420d
25.
BellRBTurveyTA. Skeletal advancement for the treatment of obstructive sleep apnea in children. Cleft Palate Craniofac J. 2001;38(2):147‐154. doi:10.1597/1545-1569_2001_038_0147_saftto_2.0.co_2
26.
CohenSRSimmsCBursteinFD. Mandibular distraction osteogenesis in the treatment of upper airway obstruction in children with craniofacial deformities. Plast Reconstr Surg. 1998;101(2):312‐318. doi:10.1097/00006534-199802000-00008
27.
CohenSRSimmsCBursteinFDThomsenJ. Alternatives to tracheostomy in infants and children with obstructive sleep apnea. J Pediatr Surg1999;34(1):182‐186; discussion 87 doi:10.1016/s0022-3468(99)90253-1.
28.
MonasterioFODruckerMMolinaFYsunzaA. Distraction osteogenesis in Pierre Robin sequence and related respiratory problems in children. J Craniofac Surg2002;13(1):79‐83; discussion 84 doi:10.1097/00001665-200201000-00018.
29.
MorovicCGMonasterioL. Distraction osteogenesis for obstructive apneas in patients with congenital craniofacial malformations. Plast Reconstr Surg. 2000;105(7):2324‐2330. doi:10.1097/00006534-200006000-00003
30.
SidmanJDSampsonDTempletonB. Distraction osteogenesis of the mandible for airway obstruction in children. Laryngoscope. 2001;111(7):1137‐1146. doi:10.1097/00005537-200107000-00004
31.
FloresRLTholpadySSSatiS, Fairbanks G, Socas J, Choi M, Havlik RJ.The surgical correction of Pierre Robin sequence: mandibular distraction osteogenesis versus tongue-lip adhesion. Plast Reconstr Surg. 2014;133(6):1433‐1439. doi:10.1097/PRS.0000000000000225
32.
MurageKPTholpadySSFrielMHavlikRJFloresRL. Outcomes analysis of mandibular distraction osteogenesis for the treatment of Pierre Robin sequence. Plast Reconstr Surg. 2013;132(2):419‐421. doi:10.1097/PRS.0b013e3182958a54
33.
EhsanZWeaverKNPanBSHuangGHossainMMSimakajornboonN. Sleep outcomes in neonates with Pierre Robin sequence undergoing external mandibular distraction: a longitudinal analysis. Plast Reconstr Surg. 2020;146(5):1103‐1115. doi:10.1097/PRS.0000000000007289
34.
GenecovDGBarceloCRSteinbergDTroneTSperryE. Clinical experience with the application of distraction osteogenesis for airway obstruction. J Craniofac Surg2009;20(Suppl 2):1817‐1821 doi:10.1097/SCS.0b013e3181b6c1b0.
35.
LoobyJFSchendelSALorenzHPHopkinsEMAizenbudD. Airway analysis: with bilateral distraction of the infant mandible. J Craniofac Surg. 2009;20(5):1341‐1346. doi:10.1097/SCS.0b013e3181ae4139
36.
ZhangRSLinLOHoppeICBartlettSPTaylorJASwansonJW. Risk factors for perioperative respiratory failure following mandibular distraction osteogenesis for micrognathia: a retrospective cohort study. Plast Reconstr Surg. 2019;143(6):1725‐1736. doi:10.1097/PRS.0000000000005651
37.
CoteAFanousAAlmajedALacroixY. Pierre Robin sequence: review of diagnostic and treatment challenges. Int J Pediatr Otorhinolaryngol. 2015;79(4):451‐464. doi:10.1016/j.ijporl.2015.01.035
38.
KhansaIHallCMadhounLL, Splaingard M, Baylis A, Kirschner RE, Pearson GD.Airway and feeding outcomes of mandibular distraction, tongue-lip adhesion, and conservative management in Pierre Robin sequence: a prospective study. Plast Reconstr Surg. 2017;139(4):975e‐983e. doi:10.1097/PRS.0000000000003167
39.
CieloCMHernandezPCiampagliaAMXanthopoulosMSBeckSETapiaIE. Positive airway pressure for the treatment of OSA in infants. Chest. 2021;159(2):810‐817. doi:10.1016/j.chest.2020.08.020
40.
AbbasDBLavinCFahyEJ, Choo H, Truong MT, Bruckman KC, Khosla RK, Lorenz HP, Momeni A, Wan DC.A systematic review of mandibular distraction osteogenesis versus orthodontic airway plate for airway obstruction treatment in Pierre Robin sequence. Cleft Palate Craniofac J. June 2021. doi:10.1177/10556656211011886
41.
BlancFHarrewijnIDuflosCMaggiulliFCaptierG. Nasopharyngeal tube and functional treatment in Pierre Robin sequence: a tertiary clinical experience from 150 cases. Cleft Palate Craniofac J. July 2021. doi:10.1177/10556656211031105
42.
PangSLHoCHALawCMLYangYLeungYY. Pre-epiglottic baton plate in the management of upper airway obstruction in an infant with femoral facial syndrome: a case report. Cleft Palate Craniofac J. 2021. doi:10.1177/10556656211059705
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