Growth of Infants with Isolated Robin Sequence
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.
KeywordsSevere Case Cleft Palate Oral Feeding Feeding Difficulty Length Growth
Centers for Disease Control and Prevention
Feeding facilitating techniques
Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo.
Isolated 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.
- 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
- 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
- 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