To describe the initial care practices for children with Pierre Robin sequence (PRS) and analyze the factors predicting the severity of the obstruction breathing disorders.
Design:
A retrospective single-center study of 150 children with PRS.
Setting:
Single tertiary care center, Regional Competence Center for the diagnosis and treatment of PRS.
Patients:
A total of 150 children with PRS consecutively followed between 1986 and 2017. Group 1 comprises children without specific respiratory management; group 2, children requiring prone positioning to alleviate their respiratory distress symptoms; and group 3, children requiring nasopharyngeal airway tube (NT) or nonconservative surgical treatment.
Main outcome measures:
Evolution and results of the initial treatment of PRS.
Results:
Forty-two percent (n = 63) were attributed to group 1, 39% (n = 50) to group 2, and 19% (n = 29) to group 3. Preterm birth, birth weight, or associated congenital malformations were not significantly different between the groups. However, the age of exclusive oral feeding was significantly different: 1 day (quartiles: 0-3) for group 1; 11 days (quartiles: 1-28) for group 2; 39 days (quartiles: 19-111) for group 3 (P < .0001). Considering the NT, its use relieves the upper airway obstruction, assessed by a respiratory polygraphy, in 14 children.
Conclusions:
Nasopharyngeal airway tube has become our major first-line treatment, avoiding more complex procedures in most of the cases. The achievement of exclusive oral feeding seems to be a good predictor of the severity of respiratory symptoms in PRS.
AbadieVMorisseau-DurandMPBeylerCManachYCoulyG. Brainstem dysfunction: a possible neuroembryological pathogenesis of isolated Pierre robin sequence. Eur J Pediatr. 2002;161(5):275–280.
2.
American Academy of Pediatrics Committee on Fetus and Newborn, American College of Obstetricians and Gynecologists Committee on Obstetric Practice. The Apgar score. Pediatrics. 2015;136(4):819–822.
3.
AndersonICWSedaghatARMcGinleyBMRedettRJBossEFIshmanSL. Prevalence and severity of obstructive sleep apnea and snoring in infants with Pierre robin sequence. Cleft Palate-Craniofacial J. 2011;48(5):614–618.
4.
AndersonKDColeAChuoCBSlatorR. Home management of upper airway obstruction in Pierre robin sequence using a nasopharyngeal airway. Cleft Palate-Craniofacial J. 2007;44(3):269–273.
5.
AndrewsBTFanKLRoostaeianJFedericoCBradleyJP. Incidence of concomitant airway anomalies when using the university of California, Los Angeles, protocol for neonatal mandibular distraction. Plast Reconstr Surg. 2013;131(5):1116–1123.
6.
BaudonJJRenaultFGoutetJMFlores-GuevaraRSoupreVGoldFVazquezMP. Motor dysfunction of the upper digestive tract in Pierre robin sequence as assessed by sucking-swallowing electromyography and esophageal manometry. J Pediatr. 2002;140(6):719–723.
BerryRBBudhirajaRGottliebDJGozalDIberCKapurVKMarcusCLMehraRParthasarathySQuanSF, et al.Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the sleep apnea definitions task force of the American academy of sleep medicine. J Clin Sleep Med. Med. 2012;8(5):597–619.
9.
BreugemCCEvansKNPoetsCFSuriSPicardAFilipCPaesECMehendaleFVSaalHMBasartH, et al.Best Practices for the diagnosis and evaluation of infants with robin sequence: a clinical consensus report. JAMA Pediatr. 2016;170(9):894–902.
10.
BuchenauWWenzelSBacherMMüller-HagedornSArandJPoetsCF. Functional treatment of airway obstruction and feeding problems in infants with robin sequence. Arch Dis Child Fetal Neonatal Ed. 2017;102(2):F142–F146.
11.
ColeALynchPSlatorR. A new grading of Pierre robin sequence. Cleft Palate-Craniofacial J. 2008;45(6):603–606.
12.
CôtéAFanousAAlmajedALacroixY. Pierre Robin sequence: review of diagnostic and treatment challenges. Int J Pediatr Otorhinolaryngol. 2015;79(4):451–464.
13.
de Buys RoessinghASHerzogGHohlfeldJ. Respiratory distress in Pierre robin: successful use of pharyngeal tube. J Pediatr Surg. 2007;42(9):1495–1499.
14.
DelormeRPLarocqueYCaouette-LabergeL. Innovative surgical approach for the Pierre Robin anomalad: subperiosteal release of the floor of the mouth musculature. Plast Reconstr Surg. 1989;83(6):960–964; discussion 965-966.
15.
Drago Marquezini SalmenICLazarini MarquesI. In situ and home care nasopharyngeal intubation improves respiratory condition and prevents surgical procedures in early infancy of severe cases of robin sequence. BioMed Res Int. 2015;2015(2):1–7.
16.
EssouriSNicotFClementAGarabedianENRogerGLofasoFFaurouxB. Noninvasive positive pressure ventilation in infants with upper airway obstruction: comparison of continuous and bilevel positive pressure. Intensive Care Med. 2005;31(4):574–580.
17.
EvansAKRahbarRRogersGFMullikenJBVolkMS. Robin sequence: a retrospective review of 115 patients. Int J Pediatr Otorhinolaryngol. 2006;70(6):973–980.
18.
GangopadhyayNMendoncaDAWooAS. Pierre Robin sequence. Semin Plast Surg. 2012;26(2):76–82.
19.
GómezOJBarónOIPeñarredondaML. Pierre robin sequence: an evidence-based treatment proposal. J Craniofac Surg. 2018a;29(2):332–338.
20.
GómezOJBarónOIPeñarredondaML. Pierre robin sequence: an evidence-based treatment proposal. J Craniofac Surg. 2018b;29(2):332–338.
21.
HansonJWSmithDW. U-shaped palatal defect in the robin anomalad: developmental and clinical relevance. J Pediatr. 1975;87(1):30–33.
22.
LogjesRJHHaasnootMLemmersPMANicolaijeMFAvan den BoogaardMJHvan der MolenABMBreugemCC. Mortality in Robin sequence: identification of risk factors. Eur J Pediatr. 2018;177(5):781–789.
23.
MaasCPoetsCF. Initial treatment and early weight gain of children with robin sequence in Germany: a prospective epidemiological study. Arch Dis Child - Fetal Neonatal Ed. 2014;99(6):F491–F494.
24.
MoriceASoupreVMitanchezDRenaultFFaurouxBMarlinSLeboulangerNKadlubNVazquezMPPicardA, et al.Severity of retrognathia and glossoptosis does not predict respiratory and feeding disorders in Pierre robin sequence. Front Pediatr. 2018;6:351.
25.
Pierre Robin. Glossoptosis due to atresia and hypotrophy of the mandible. Am J Dis Children. 1934;48(3):541–547.
26.
PoetsCFMaasCBuchenauWArandJVierzigABraumannBMüller-HagedornS. Multicenter study on the effectiveness of the pre-epiglottic baton plate for airway obstruction and feeding problems in Robin sequence. Orphanet J Rare Dis. 2017;12(1):1–6. doi:10.1186/s13023-017-0602-8
27.
RathéMRayyanMSchoenaersJDormaarJTBreulsMVerdonckADevriendtKVander PoortenVHensG. Pierre robin sequence: management of respiratory and feeding complications during the first year of life in a tertiary referral centre. Int J Pediatr Otorhinolaryngol. 2015;79(8):1206–1212.
28.
RobinP. Glossoptosis due to atresia and hypotrophy of the mandible. Arch Pediatr Adolesc Med. 1934;48(3):541.
van LieshoutMJSJoostenKFMHoeveHLJMathijssenIMJKoudstaalMJWolviusEB. Unravelling robin sequence: considerations of diagnosis and treatment. Laryngoscope. 2014;124(5):E203–E209.
31.
van NunenDPFvan den BoogaardMJHBreugemCC. Robin sequence: continuing heterogeneity in nomenclature and diagnosis. J Craniofac Surg. 2018;29(4):985–987.
32.
VatlachSMaasCPoetsCF. Birth prevalence and initial treatment of robin sequence in Germany: a prospective epidemiologic study. Orphanet J Rare Dis. 2014;9(1):1–5.
33.
VipulananthanNCooperTWitmansMEl-HakimH. Primary aerodigestive presentations of Pierre Robin sequence/complex and predictive factors of airway type and management. Int J Pediatr Otorhinolaryngol. 2014;78(10):1726–1730.
34.
WiechersCBuchenauWArandJOertelAFPetersKMüller-HagedornSKoosBPoetsCF. Mandibular growth in infants with robin sequence treated with the Tübingen palatal plate. Head Face Med. 2019;15(1):1–8. doi:10.1186/s13005-019-0200 -1
