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Pierre Robin Sequence

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Pediatric Surgery

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Abstract

Pierre Robin sequence is a rare cause of neonatal airway obstruction that requires surgery in some affected patients. The goals of the treatment are appropriate airway and nutritional management. This chapter describes the clinical presentation, diagnostics, and subsequent conservative or surgical treatment of Pierre Robin sequence.

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References

  • Acke FR, Dhooge IJ, Malfait F et al (2012) Hearing impairment in Stickler syndrome: a systematic review. Orphanet J Rare Dis 7:84

    Article  PubMed  PubMed Central  Google Scholar 

  • Albino FP, Wood BC, Han KD, Yi S, Seruya M, Rogers GF, Oh AK (2016) Clinical factors associated with the non-operative airway management of patients with Robin sequence. Arch Plast Surg 43(6):506–511

    Article  PubMed  PubMed Central  Google Scholar 

  • Amaddeo A, Abadie V, Chalouhi C et al (2016) Continuous positive airway pressure for upper airway obstruction in infants with Pierre Robin sequence. Plast Reconstr Surg 137(2):609–612

    Article  CAS  PubMed  Google Scholar 

  • Borovik HR, Kveton JF (1987) Pierre Robin syndrome combined with unilateral choanal atresia. Otolaryngol Head Neck Surg 96:67–70

    Article  CAS  PubMed  Google Scholar 

  • Brosch S, Flaig S, Bacher M, Michels L, de Maddalena H, Reinert S, Mauz P (2006) The influence of the Tübingen soft palate plate and early cleft closure on swallowing and Eustachian tube function in children with Pierre Robin sequence. HNO 54:756–760

    Article  CAS  PubMed  Google Scholar 

  • Broucqsault H, Lamblin MD, Hosana G, Fayoux P (2018) Evaluation of the efficacy of tongue-lip adhesion in Pierre-Robin-Sequence. Eur Ann Otorhinolaryngol Head Neck Diseases 135:155–158

    Article  CAS  Google Scholar 

  • Buchanan EP, Xue AS, Hollier LH Jr (2014) Craniofacial syndromes. Plast Reconstr Surg 134(1):128e–153e

    Article  CAS  PubMed  Google Scholar 

  • Buchenau W, Wenzel S, Bacher M, Müller-Hagedorn S, Arand J, Poets CF (2017). Functional treatment of airway obstruction and feeding problems in infants with Robin sequence Arch Dis Child Fetal Neonatal 102(2):142–146. https://doi.org/10.1136/archdischild-2016-311407. Epub 2016 Jul 19

  • Bull MJ, Givan DC, Sadove AM, Bixler D, Hearn D (1990) Improved outcome in Pierre Robin sequence: effect of multidisciplinary management. Pediatrics 86:294–301

    Article  CAS  PubMed  Google Scholar 

  • Carey JC, Fineman RM, Ziter FA (1982) The Robin sequence as a consequence of malformation, dysplasia, and neuromuscular syndromes. J Pediatr 101:858–864

    Article  CAS  PubMed  Google Scholar 

  • Cladis F, Kumar A, Grunwaldt L et al (2014) Pierre Robin sequence: a perioperative review. Anesth Analg 119(2):400–412

    Article  PubMed  Google Scholar 

  • Costa MA, Tu MM, Murage KP et al (2014) Robin sequence: mortality, causes of death, and clinical outcomes. Plast Reconstr Surg 134(4):738–745

    Article  CAS  PubMed  Google Scholar 

  • Cote A, Fanous A, Almajed A et al (2015) Pierre Robin sequence: review of diagnostic and treatment challenges. Int J Pediatr Otorhinolaryngol 79(4):451–464

    Article  PubMed  Google Scholar 

  • Cozzi F, Pierro A (1985) Glossoptosis-apnoea syndrome in infancy. Pediatrics 75:836–843

    Article  CAS  PubMed  Google Scholar 

  • Daskalogiannakis J, Ross RB, Tompson BD (2001) The mandibular catch-up growth controversy in Pierre Robin sequence. Am J Orthod Dentofac Orthop 120:280–285

    Article  CAS  Google Scholar 

  • Dykes EH, Raine PAM, Arthur DS, Drainer IK, Young DG (1985) Pierre Robin syndrome and pulmonary hypertension. J Pediatr Surg 20:49–52

    Article  CAS  PubMed  Google Scholar 

  • Ehsan Z, Weaver KN, Pan BS, Huang G, Hossain MM, Simakajornboon N (2020) Sleep outcomes in neonates with Pierre Robin sequence undergoing external mandibular distraction: a longitudinal analysis. Plast Reconstr Surg; Aug 19. https://doi.org/10.1097/PRS.000000000007289

  • Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML, Robin sequence. (2011) From diagnosis to development of an effective management plan. Pediatrics 127:936–948

    Article  PubMed  PubMed Central  Google Scholar 

  • Figueroa AA, Glupker TJ, Fitz MG, BeGole EA (1991) Mandible, tongue, and airway in Pierre Robin sequence: a longitudinal cephalometric study. Cleft Palate-Craniofac J 28:425–434

    Article  CAS  PubMed  Google Scholar 

  • Frohberg U, Lange RT (1993) Surgical treatment of Robin sequence and sleep apnea syndrome; case report and review of the literature. J Oral Maxillofac Surg 51:1274–1277

    Article  CAS  PubMed  Google Scholar 

  • Gangopadhyay N, Mendoonca DA, Woo AS (2012) Pierre Robin sequence. Sem Plast Surg 26:76–82

    Article  Google Scholar 

  • Handžić J, Ćuk V, RiÅ¡avi R, Katić V, KatuÅ¡ić D, Bagatin M, Å tajner-KatuÅ¡ić S, Gortan D (1996) Pierre Robin syndrome: characteristics of hearing loss, effect of age on hearing level and possibilities in therapy planning. J Laryngol Otol 110:830–835

    Article  Google Scholar 

  • Hanson JW, Smith DW (1975) U-shaped palatal defect in the Robin anomalad: developmental and clinical relevance. J Pediatr 87:30–33

    Article  CAS  PubMed  Google Scholar 

  • Hatch DJ (1989) Anaesthesia for paediatric surgery. In: Summer E, Hatch DJ (eds) Textbook of paediatric anaesthesia practice. Baillière Tindall, London, pp 275–304

    Google Scholar 

  • Hohoff A, Ehmer U (1999) Short-term and long-term results after early treatment with the Castillo Morales stimulating plate – a longitudinal study. J Orofac Othop 60(1):2–12

    Article  CAS  Google Scholar 

  • Kadakia S, Helman SN, Badhey AK et al (2014) Treacher Collins syndrome: the genetics of a craniofacial disease. Int J Pediatr Otorhinolaryngol 78(6):893–898

    Article  PubMed  Google Scholar 

  • Kam K, McKay M, MacLean J, Witmans M, Spier S, Mitchell I (2015) Surgical versus nonsurgical interventions to relieve upper airway obstruction in children with Pierre Robin sequence. Can Respir J 22(3):171–175

    Article  PubMed  PubMed Central  Google Scholar 

  • Khansa I, Hall C, Madhoun LL et al (2017) Airway and feeding outcomes of mandibular distraction, tongue-lip adhesion, and conservative management in Pierre Robin sequence: a prospective study. Plast Reconstr Surg 139(4):975e–983e

    Article  CAS  PubMed  Google Scholar 

  • Laitinen SH, Heliövaara A, Ranta RE (1997) Craniofacial morphology in young adults with the Pierre Robin sequence and isolated cleft palate. Acta Odontol Scand 55:223–228

    Article  CAS  PubMed  Google Scholar 

  • Leboulanger N, Picard A, Soupre V, Aubertin G, Denoyelle F, Galliani E, Roger G, Garabedian EN, Fauroux B (2010) Physiologic and clinical benefits of noninvasive ventilation in infants with Pierre Robin sequence. Pediatrics 126:e1056–e1063

    Article  PubMed  Google Scholar 

  • Li L, Scott AR (2020) Weight gain in infants with Pierre Robin Sequence in the first year of life. Otolaryngol Head Neck Surg 2020, May 19; https://doi.org/10.1177/0194599820925454

  • Logjes RJH, Haasnoot M, Lemmers PMA, Nicolaije MFA, van den Boogaard MJH, van der Molen ABM, Breugem CC (2018) Mortality in Robin sequence: identification of risk factors. Eur J Pediatr 177:781–789

    Article  PubMed  PubMed Central  Google Scholar 

  • Müller-Hagedorn S, Buchenau W, Arand J, Bacher M, Poets CF (2017). Treatment of infants with Syndromic Robin sequence with modified palatal plates: a minimally invasive treatment option. Head Face Med 30;13(1):4. https://doi.org/10.1186/s13005-017-0137-1

  • Myer CM, Reed JM, Cotton RT, Willging JP, Shott SR (1998) Airway management in Pierre Robin sequence. Otolaryngol Head Neck Surg 118:630–635

    Article  PubMed  Google Scholar 

  • Ogborn MR, Pemberton PJ (1985) Late development of airway obstruction in the Robin anomalad (Pierre Robin syndrome) in the newborn. Aust Paediatr J 21:199–200

    CAS  PubMed  Google Scholar 

  • Opitz JM, France T, Herrman J, Spranger JW (1972) The stickler syndrome. N Engl J Med 286:546–547

    Article  CAS  PubMed  Google Scholar 

  • Pearl W (1982) Congenital heart disease in the Pierre Robin syndrome. Pediatr Cardiol 2:307–309

    Article  CAS  PubMed  Google Scholar 

  • Sader R (2009) Lippen-Kiefer-Gaumenspalten. Pädiatrie up2date 2:183–205. https://doi.org/10.1055/s−0029−1214683

  • Sadewitz VL (1992) Robin sequence: changes in thinking leading to changes in patient care. Cleft Palate Craniofac J 29(3):236–253

    Article  Google Scholar 

  • Sheffield LJ, Reiss JA, Strohm K, Gilding M (1987) A genetic follow-up study of 64 patients with the Pierre Robin complex. Am J Med Genet 28:25–36

    Article  CAS  PubMed  Google Scholar 

  • Smith JL, Stowe FR (1961) The Pierre Robin syndrome (glossoptosis, micrognathia, cleft palate). Pediatrics 27:128–133

    Article  Google Scholar 

  • Tan TY, Kilpatrick N, Parlie PG (2013) Developmental and genetic perspectives on Pierre Robin sequence. Am J Med Genet C Semin Med Genet 163C(4):295–305

    Article  PubMed  Google Scholar 

  • Trainor PA, Andrews BT (2013) Facial dysostoses: etiology, pathogenesis and management. Am J Med Genet C Semin Med Genet 163C(4):283–294

    Article  PubMed  Google Scholar 

  • Williams AJ, Williams MA, Walker CA, Bush PG (1981) The Robin anomalad (Pierre Robin syndrome) – a follow up study. Arch Dis Child 45:663–668

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, University Hospital Frankfurt, Frankfurt/Main, Germany

    Udo Rolle

  2. Department of Pediatric Surgery, Goethe University Frankfurt, Frankfurt, Germany

    Udo Rolle

  3. Carolinum, Institute of Dentistry, Frankfurt, Germany

    Aranka Ifert

  4. Department of Oral, Maxillofacial, and Plastic Facial Surgery, Goethe University Frankfurt, Frankfurt, Germany

    Robert Sader

Authors
  1. Udo Rolle
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  2. Aranka Ifert
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  3. Robert Sader
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Corresponding author

Correspondence to Udo Rolle .

Editor information

Editors and Affiliations

  1. Beacon Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

  2. University Clinic of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria

    Michael E. Höllwarth

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Rolle, U., Ifert, A., Sader, R. (2023). Pierre Robin Sequence. In: Puri, P., Höllwarth, M.E. (eds) Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-81488-5_27

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  • DOI: https://doi.org/10.1007/978-3-030-81488-5_27

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-81487-8

  • Online ISBN: 978-3-030-81488-5

  • eBook Packages: MedicineMedicine (R0)

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