Growth of Infants with Isolated Robin Sequence

Abstract

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 Reabilitacao de Anomalias Craniofaciais – Universidade de Sao 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.