Abstract
The human mandible is a unique structure that undergoes considerable anatomic changes during development. Hypoplasia of the mandible can lead to a sequence of events resulting in severe respiratory compromise in neonates. This chapter explores the background, epidemiology, history and classification, diagnosis, and management of tongue-based airway obstruction due to micrognathia, commonly known as Pierre Robin sequence.
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Tonkin SL, Gunn TR, Bennet L, Vogel SA, Gunn AJ. A review of the anatomy of the upper airway in early infancy and its possible relevance to SIDS. Early Hum Dev. 2002;66(2):107–21.
Randall P, Krogman WM, Jahins S. Pierre Robin and the syndrome that bears his name. Cleft Palate J. 1965;36:237–46.
Robin P. 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):1301–3.
Singh DJ, Bartlett SP. Congenital mandibular hypoplasia: analysis and classification. J Craniofac Surg. 2005;16(2):291–300.
Diewert VM. Craniofacial growth during human secondary palate formation and potential relevance of experimental cleft palate observations. J Craniofac Genet Dev Biol Suppl. 1986;22:67–76.
Mackay DR. Controversies in the diagnosis and management of the Robin sequence. J Craniofac Surg. 2011;22(2):415–20.
Jakobsen LP, Knudsen MA, Lespinasse J, García Ayuso C, Ramos C, Fryns J-P, et al. The genetic basis of the Pierre Robin Sequence. Cleft Palate Craniofac J. 2006;43(2):155–9.
Jakobsen LP, Ullmann R, Christensen SB, Jensen KE, Mølsted K, Henriksen KF, et al. J Med Genet. 2007;44(6):381–6.
Izumi K, Konczal LL, Mitchell AL, Jones MC. Underlying genetic diagnosis of Pierre Robin sequence: retrospective chart review at two children’s hospitals and a systematic literature review. J Pediatr. 2012;160(4):645–50. e2.
Cozzi F, Pierro A. Glossoptosis-apnea syndrome in infancy. Pediatrics. 1985;75(5):836–43.
Marques IL, de Sousa TV, Carneiro AF, Barbieri MA, Bettiol H, Gutierrez MR. Clinical experience with infants with Robin sequence: a prospective study. Cleft Palate Craniofac J. 2001;38(2):171–8.
Gorlin RJ, Cohen MM, Hennekam RCM. Syndromes of the head and neck. 4th ed. New York: Oxford University Press; 2001.
Bush PG, Williams AJ. Incidence of the Robin Anomalad (Pierre Robin syndrome). Br J Plast Surg. 1983;36(4):434–7.
Schaefer RB, Stadler JA, Gosain AK. To distract or not to distract: an algorithm for airway management in isolated Pierre Robin sequence. Plast Reconstr Surg. 2004;113(4):1113–25.
Glander K, Cisneros GJ. Comparison of the craniofacial characteristics of two syndromes associated with the Pierre Robin sequence. Cleft Palate Craniofac J. 1992;29(3):210–9.
Cole A, Lynch P, Slator R. A new grading of Pierre Robin sequence. Cleft Palate Craniofac J. 2008;45(6):603–6.
Rogers GF, Murthy AS, Labrie RA, Mulliken JB. The GILLS score: part I. Patient selection for tongue-lip adhesion in Robin sequence. Plast Reconstr Surg. 2011;128(1):243–51.
Abramowicz S, Bacic JD, Mulliken JB, Rogers GF. Validation of the GILLS score for tongue-lip adhesion in Robin sequence patients. J Craniofac Surg. 2012;23(2):382–6.
Dudkiewicz Z, Sekuła E, Nielepiec-Jałosin´ska A. Gastroesophageal reflux in Pierre Robin sequence—early surgical treatment. Cleft Palate Craniofac J. 2000;37(2):205–8.
Caouette-Laberge L, Bayet B, Larocque Y. The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities. Plast Reconstr Surg. 1994;93(5):934–42.
Wheeler DS, Wong HR, Shanley TP. Pediatric critical care medicine: basic science and clinical evidence. In: Mehta RL, editor. Pediatric critical care medicine: basic science and clinical evidence. London: Springer; 2007.
Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML. Robin sequence: from diagnosis to development of an effective management plan. Pediatrics. 2011;127(5):936–48.
Berry RB, Budhiraja R, Gottlieb DJ, Gozal D, Iber C, Kapur VK, 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. 2012;8(5):597–619.
Freed G, Pearlman MA, Brown AS, Barot LR. Polysomnographic indications for surgical intervention in Pierre Robin sequence: acute airway management and follow-up studies after repair and take-down of tongue-lip adhesion. Cleft Palate J. 1988;25(2):151–5.
Gilhooly JT, Smith JD, Howell LL, Deschaine BL, Richey SL. Bedside polysomnography as an adjunct in the management of infants with Robin sequence. Plast Reconstr Surg. 1993;92(1):23–7.
Bookman LB, Melton KR, Pan BS, Bender PL, Chini BA, Greenberg JM, et al. Neonates with tongue-based airway obstruction: a systematic review. Otolaryngol Head Neck Surg. 2012;146(1):8–18.
Hong P, Bezuhly M, Mark Taylor S, Hart RD, Kearns DB, Corsten G. Tracheostomy versus mandibular distraction osteogenesis in Canadian children with Pierre Robin sequence: a comparative cost analysis. J Otolaryngol Head Neck Surg. 2012;41(3):207–14.
Chang AB, Masters IB, Williams GR, Harris M, O’Neil MC. A modified nasopharyngeal tube to relieve high upper airway obstruction. Pediatr Pulmonol. 2000;29(4):299–306.
Anderson KD, Cole A, Chuo CB, Slator R. Home management of upper airway obstruction in Pierre Robin sequence using a nasopharyngeal airway. Cleft Palate Craniofac J. 2007;44(3):269–73.
Bull MJ, Givan DC, Sadove AM, Bixler D, Hearn D. Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management. Pediatrics. 1990;86(2):294–301.
Meyer AC, Lidsky ME, Sampson DE, Lander TA, Liu M, Sidman JD. Airway interventions in children with Pierre Robin Sequence. Otolaryngol Head Neck Surg. 2008;138(6):782–7.
Abel F, Bajaj Y, Wyatt M, Wallis C. The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience. Arch Dis Child. 2012;97(4):331–4.
Douglas B. The treatment of micrognathia associated with obstruction by a plastic procedure. Plast Reconstr Surg. 1946;1(3):300–8.
Kirschner RE, Low DW, Randall P, Bartlett SP, McDonald-McGinn DM, Schultz PJ, et al. Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion? Cleft Palate Craniofac J. 2003;40(1):13–8.
Evans AK, Rahbar R, Rogers GF, Mulliken JB, Volk MS. Robin sequence: a retrospective review of 115 patients. Int J Pediatr Otorhinolaryngol. 2006;70(6):973–80.
Bijnen CL, Don Griot PJ, Mulder WJ, Haumann TJ, Van Hagen AJ. Tongue-lip adhesion in the treatment of Pierre Robin sequence. J Craniofac Surg. 2009;20(2):315–20.
Denny A, Kalantarian B. Mandibular distraction in neonates: a strategy to avoid tracheostomy. Plast Reconstr Surg. 2002;109(3):896–904; discussion 905–6.
Marcellus L. The infant with Pierre Robin sequence: review and implications for nursing practice. J Pediatr Nurs. 2001;16(1):23–34.
Figueroa AA. Commentary on “Craniofacial morphology in Treacher Collins syndrome”, by Arvystas, M., Shprintzen, R.J. Cleft Palate Craniofac J. 1991;28:230–1.
Figueroa AA, Glupker TJ, Fitz MG, Begole EA. The mandible, tongue and airway in Pierre Robin sequence (PRS): a longitudinal cephalometric study. Cleft Palate Craniofac J. 1991;28:425–34.
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Goldstein, J.A., Taylor, J.A. (2015). Impact of Micro- and Retrognathia on the Neonatal Airway. In: Lioy, J., Sobol, S. (eds) Disorders of the Neonatal Airway. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1610-8_5
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