Skip to main content

Pierre Robin Sequence

Pediatric Surgery

Abstract

Pierre Robin sequence is a rare cause of neonatal airway obstruction considered to be “surgical,” and some of the affected patients truly require surgical procedures to achieve appropriate airway and nutritional management. The clinical presentation, diagnostics, and the subsequent conservative or surgical treatment of Pierre Robin syndrome will be described within the following chapter.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

References

  • Albino FP, Wood BC, Han KD, Yi S, Seruya M, Rogers GF, Oh AK. Clinical factors associated with the non-operative airway management of patients with Robin sequence. Arch Plast Surg. 2016;43(6):506–11.

    Article  PubMed  PubMed Central  Google Scholar 

  • Amaddeo A, Abadie V, Chalouhi C, et al. Continuous positive airway pressure for upper airway obstruction in infants with Pierre Robin sequence. Plast Reconstr Surg. 2016;137(2):609–12.

    Article  CAS  PubMed  Google Scholar 

  • Bacher M, Bacher U, Göz G, Pham T, Cornelius CP, Speer CP, Goelz R, Arand J, Wendling F, Buchner P, Bacher A. Three-dimensional computer morphometry of the maxilla and face in infants with Pierre Robin sequence – a comparative study. Cleft Palate-Craniofac J. 2000;37:292–302.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  • Breugem CC, Evans KN, Poets CF, 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.

    Article  PubMed  Google Scholar 

  • Brosch S, Flaig S, Bacher M, Michels L, de Maddalena H, Reinert S, Mauz P. 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. 2006;54:756–60.

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  • Bush PG, Williams AJ. Incidence of the Robin anomalad (Pierre Robin syndrome). Br J Plast Surg. 1983;36:434–7.

    Article  CAS  PubMed  Google Scholar 

  • Bütow KW, Zwahlen RA, Morkel JA, Naidoo S. Pierre Robin sequence: subdivision, data, theories, and treatment – part 1: history, subdivisions, and data. Ann Maxillofac Surg. 2016;6(1):31–4.

    Article  PubMed  PubMed Central  Google Scholar 

  • Carey JC, Fineman RM, Ziter FA. The Robin sequence as a consequence of malformation, dysplasia, and neuromuscular syndromes. J Pediatr. 1982;101:858–64.

    Article  CAS  PubMed  Google Scholar 

  • Cohen MM. Syndromes with cleft lip and cleft palate. Cleft Palate J. 1978;15:306–28.

    PubMed  Google Scholar 

  • Cohen Jr MM. Robin sequences and complexes: causal heterogeneity and pathogenetic/phenotypic variability. Am J Med Genet. 1999;84:311–5.

    Article  PubMed  Google Scholar 

  • Cozzi F, Pierro A. Glossoptosis-apnoea syndrome in infancy. Pediatrics. 1985;75:836–43.

    CAS  PubMed  Google Scholar 

  • Daskalogiannakis J, Ross RB, Tompson BD. The mandibular catch-up growth controversy in Pierre Robin sequence. Am J Orthod Dentofac Orthop. 2001;120:280–5.

    Article  CAS  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  • Elliot M, Studen-Pavlovich D, Ranalli DN. Prevalence of selected pediatric conditions in children with Pierre Robin syndrome. Pediatr Dent. 1995;17:106–11.

    Google Scholar 

  • 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;127:936–48.

    Article  PubMed  PubMed Central  Google Scholar 

  • Fairbairn P. Suffocation in an infant from retraction of the base of the tongue, connected with defect of the frenum. Month J Med Sci. 1846;6:280–1.

    Google Scholar 

  • Figueroa AA, Glupker TJ, Fitz MG, BeGole EA. Mandible, tongue, and airway in Pierre Robin sequence: a longitudinal cephalometric study. Cleft Palate-Craniofac J. 1991;28:425–34.

    Article  CAS  PubMed  Google Scholar 

  • Frohberg U, Lange RT. Surgical treatment of Robin sequence and sleep apnea syndrome; case report and review of the literature. J Oral Maxillofac Surg. 1993;51:1274–7.

    Article  CAS  PubMed  Google Scholar 

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

    Article  Google Scholar 

  • Handžić J, Ćuk V, Rišavi R, Katić V, Katušić D, Bagatin M, Štajner-Katušić S, Gortan D. Pierre Robin syndrome: characteristics of hearing loss, effect of age on hearing level and possibilities in therapy planning. J Laryngol Otol. 1996;110:830–5.

    Google Scholar 

  • Hanson JW, Smith DW. U-shaped palatal defect in the Robin anomalad: developmental and clinical relevance. J Pediatr. 1975;87:30–3.

    Article  CAS  PubMed  Google Scholar 

  • Hatch DJ. Anaesthesia for paediatric surgery. In: Summer E, Hatch DJ, editors. Textbook of paediatric anaesthesia practice. London: Baillière Tindall; 1989. p. 275–304.

    Google Scholar 

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

    Article  CAS  Google Scholar 

  • Kam K, McKay M, MacLean J, Witmans M, Spier S, Mitchell I. Surgical versus nonsurgical interventions to relieve upper airway obstruction in children with Pierre Robin sequence. Can Respir J. 2015;22(3):171–5.

    Article  PubMed  PubMed Central  Google Scholar 

  • Laitinen SH, Heliövaara A, Ranta RE. Craniofacial morphology in young adults with the Pierre Robin sequence and isolated cleft palate. Acta Odontol Scand. 1997;55:223–8.

    Article  CAS  PubMed  Google Scholar 

  • Leboulanger N, Picard A, Soupre V, Aubertin G, Denoyelle F, Galliani E, Roger G, Garabedian EN, Fauroux B. Physiologic and clinical benefits of noninvasive ventilation in infants with Pierre Robin sequence. Pediatrics. 2010;126:e1056–63.

    Article  PubMed  Google Scholar 

  • Ludwig B, Glasl B, Sader R, Schopf P. Conservative orthodontic primary Care of Four Newborns with the Pierre-Robin sequence triad. J Orofacial Orthopedics. 2007;68:56–61.

    Article  Google Scholar 

  • Myer CM, Reed JM, Cotton RT, Willging JP, Shott SR. Airway management in Pierre Robin sequence. Otolaryngol Head Neck Surg. 1998;118:630–5.

    Article  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  • Opitz JM, France T, Herrman J, Spranger JW. The stickler syndrome. N Engl J Med. 1972;286:546–7.

    Article  CAS  PubMed  Google Scholar 

  • Pashayan HM, Lewis MB. Clinical experience with the Robin sequence. Cleft Palate J. 1984;21:270–6.

    Google Scholar 

  • Pearl W. Congenital heart disease in the Pierre Robin syndrome. Pediatr Cardiol. 1982;2:307–9.

    Article  CAS  PubMed  Google Scholar 

  • Robin P. La chute de la base de la langue consideree comme une nouvelle cause de gene dans larespiration naso-pharyngienne. Bull Acad Med Paris. 1923;89:37–41.

    Google Scholar 

  • Robin P. Glossoptosis due to atresia and hypotrophy of the mandible. Am J Dis Child. 1934;48:541–7.

    Google Scholar 

  • Sadewitz VL. Robin sequence: changes in thinking leading to changes in patient care. Cleft Palate Craniofac J. 1992;29(3):236–53.

    Article  Google Scholar 

  • Schubert J, Jahn H, Berginski M. Experimental aspects of the pathogenesis of Robin sequence. Cleft Palate Craniofac J. 2005;42:372–6.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  • Shukowsky WP. Zur aetiologie des stridor inspiratorius congenitus. Jahrb Kinderheilk. 1911;73:459–74.

    Google Scholar 

  • Smith JL, Stowe FR. The Pierre Robin syndrome (glossoptosis, micrognathia, cleft palate). Pediatrics. 1961;27:128–33.

    Google Scholar 

  • Smith DW. Recognizable patterns of human malformation. 3rd ed. Phildelphia: WB Saunders; 1982.

    Google Scholar 

  • St. Hilaire H. Sphenocephalus. Philos Anat. 1822;2:97–8.

    Google Scholar 

  • Williams AJ, Williams MA, Walker CA, Bush PG. The Robin anomalad (Pierre Robin syndrome) – a follow up study. Arch Dis Child. 1981;45:663–8.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Udo Rolle .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer-Verlag GmbH Germany

About this entry

Cite this entry

Rolle, U., Ifert, A., Sader, R. (2016). Pierre Robin Sequence. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_44-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-38482-0_44-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Chapter history

  1. Latest

    Pierre Robin Sequence
    Published:
    23 August 2019

    DOI: https://doi.org/10.1007/978-3-642-38482-0_44-2

  2. Original

    Pierre Robin Sequence
    Published:
    01 March 2017

    DOI: https://doi.org/10.1007/978-3-642-38482-0_44-1