Growth of Infants with Isolated Robin Sequence

  • Ilza Lazarini Marques


Robin sequence (RS), defined as retromicrognathia and glossoptosis, with or without cleft palate, is clinically characterized by obstruction of the upper airway and respiratory and feeding difficulties, which are more frequent and more severe during the first months of life. This anomaly may arise as an isolated entity – isolated Robin sequence (IRS) – as a component of a known syndrome or in association with other malformations which do not characterize a known syndrome. Feeding difficulty and failure to thrive are usually determined by airway obstruction and are aggravated by the cleft palate. The treatment modalities for airway obstruction in IRS are postural treatment (the infant is placed in the prone position), glossopexy, osteogenic mandibular distraction, and tracheotomy. Nasopharyngeal intubation (NPI) is frequently used in Hospital de Reabilitação de Anomalias Craniofaciais – Universidade de São Paulo (HRAC-USP) – as a modality of treatment even in severe cases of IRS; according to the literature, this prevents surgical procedures in early infancy. Most infants with IRS improved their respiratory difficulty without surgical procedures. Improvement of respiratory difficulty may lead to improvement of feeding and growth. Various techniques have been developed to improve feeding difficulty: (1) the diet is given initially through feeding tube and oral feeding is gradually introduced according to ability of each child and (2) some feeding facilitating techniques, applied after airway release, can improve oral feeding. Infants with IRS present a more significant impairment of weight gain than of length gain during the first 6 months of life, but a hypercaloric diet has been used to improve weight gain. The techniques developed to improve feeding are not sufficient to improve growth of infants with IRS considered as severe cases. Despite all techniques developed to treat respiratory and feeding difficulties, it has become a great challenge to bring the growth of children with IRS closer to that of normal children.


Severe Case Cleft Palate Oral Feeding Feeding Difficulty Length Growth 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Centers for Disease Control and Prevention


Feeding facilitating techniques


Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo.


Isolated Robin sequence


nasopharyngeal intubation


Robin sequence



The author acknowledges Arshad R. Muzaffar, Editor of The Cleft Palate Craniofacial Journal; Valerie Pierce, Publishing Coordinator of Allen Press Publishing Services; Katie B Wade, Permissions Assistant of Wiley–Blackwell who granted permission rights for the original publication; Ana A. Gomes Grigolli, Librarian at Hospital de Reabilitação de Anomalias Craniofaciais – Universidade de São Paulo (HRAC-USP); Ricardo Pimentel Nogueira, Documentation and Information Technician at HRAC-USP; Lucas Ribeiro de Aguiar, Designer Assistant at HRAC-USP; and Elaine de Oliveira Martins, English teacher, for their support and cooperation for the completion of this chapter.


  1. Anderson KD, Cole A, Chuo CB, Slator R. Home management of upper airway obstructionin Pierre Robin sequence using a nasopharyngeal airway. Cleft Palate Craniofac J. 2007;44:269–73.PubMedCrossRefGoogle Scholar
  2. Dawson DV, Todorov AB, Elston RC. Confidence bands for the growth of head circumference in achondroplastic children during the first year of life. Am J Med Genet. 1980;7:529–36.PubMedCrossRefGoogle Scholar
  3. Denny A, Kalantarian B. Mandibular distraction in neonates: a strategy to avoid  tracheostomy. Plast Reconstr Surg. 2002;109:896–906.PubMedCrossRefGoogle Scholar
  4. Freeman MK, Manners JM. Cor pulmonale and the Pierre Robin anomaly. Airway management with a nasopharyngeal tube. Anaesthesia. 1980;35:282–6.PubMedCrossRefGoogle Scholar
  5. Kuczmarski RJ, Ogden CL, Grummer-Strawn LM, Flegal KM, Guo SS, Wei R, Mei Z, Curtin LR, Roche AF, Johnson CL. 2000 CDC Growth Charts for the United States: methods and development. Adv Data. 2000;1–27.Google Scholar
  6. Marques IL, Sousa TV, Carneiro AF. A large experience with infants with Robin sequence: a prospective study on 159 cases. In: Transactions of the 9th international congress on cleft palate and related craniofacial anomalies, 2001 June 25–29; Göterborg, Sweden. Göterborg: Cleft Palate Craniofacial Association; 2001a. pp. 81–7.Google Scholar
  7. Marques IL, Sousa TV, Carneiro AF, Barbieri MA, Bettiol H, Gutierrez MRP. Clinical experience with infants with Robin sequence: a prospective study. Cleft Palate Craniofac J. 2001b;38:171–8.PubMedCrossRefGoogle Scholar
  8. Marques IL, Peres SP, Bettiol H, Barbieri MA, Andréa M, Souza L. Growth of children with isolated Robin sequence treated by nasopharyngeal intubation: importance of a hypercaloric diet. Cleft Palate Craniofac J. 2004;41:53–8.PubMedCrossRefGoogle Scholar
  9. Marques IL, Sousa TV, Carneiro AF, Peres SP, Barbieri MA, Bettiol H. Seqüência de Robin: protocolo único de tratamento. J Pediatr. 2005;81:14–22.Google Scholar
  10. Marques IL, Bettiol H, Souza L, Barbieri MA, Bachega MI. Longitudinal study of the growth of infants with isolated Robin sequence considered being severe cases. Acta Paediatr. 2008;97:371–5.PubMedCrossRefGoogle Scholar
  11. Nassar E, Marques IL, Trindade Jr AS, Bettiol H. Feeding facilitating techniques for the nursing infant with Robin sequence. Cleft Palate Craniofac J. 2006;43:55–60.PubMedCrossRefGoogle Scholar
  12. Shprintzen RJ. The implications of the diagnosis of Robin sequence. Cleft Palate Craniofac J. 1992;29:205–9.PubMedCrossRefGoogle Scholar
  13. Sousa TV, Marques IL, Carneiro AF, Bettiol H, Freitas JAS. Nasopharyngoscopy in Robin sequence: clinical and predictive value. Cleft Palate Craniofac J. 2003;40:618–23.PubMedCrossRefGoogle Scholar
  14. Spring MA, Mount DL. Pediatric feeding disorder and growth decline following mandibular distraction osteogenesis. Plast Reconstr Surg. 2006;118:476–82.PubMedCrossRefGoogle Scholar
  15. Wagener S, Rayatt SS, Tatman AJ, Gornall P, Slator R. Management of infants with Pierre Robin sequence. Cleft Palate Craniofac J. 2003;40:180–5.PubMedCrossRefGoogle Scholar
  16. Wittenborn W, Panchal J, Marsh JL, Sekar KC, Gurley J. Neonatal distraction surgery for micrognathia reduces obstructive apnea and the need for tracheotomy. J Craniofac Surg. 2004;15:623–30.PubMedCrossRefGoogle Scholar

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© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Hospital DepartmentBauru, SPBrazil

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