Clinical experience with infants with Robin sequence: A prospective study

Objective: To study the clinical course of patients with Robin sequence (RS ) during the first 6 months of life. Design: A longitudinal prospective study of children with RS. Setting: Hospital de Reabilitacao de Anomalias Craniofaciais, Universidade de Sao Paulo, Bauru-SP, Brazil, 1997 and 1998. Patients: Sixty-two children were studied from hospital admission to 6 mont hs of age. Thirty-three (53.2%) presented with probable isolated RS (PIRS), 25 (40.3%) presented with syndromes or other malformations associated with RS, and 4 (6.5%) presented with RS with neurological involvement. Interventions: The type of respiratory tract obstruction was defined by nas opharyngoscopy. The patients with type 1 and type 2 obstruction underwent n asopharyngeal intubation (NPI), and glossopexy was indicated in patients wi th type 1 obstruction who did not show clinical improvement with this proce dure. Tracheostomy was indicated in patients with type 2 obstruction who di d not show a good course after NPI, in patients with type 1 obstruction who did not show good course after glossopexy, and in patients with type 3 and type 4 obstruction. Results: Prone position treatment (PPT) or NPI was the definitive treatment in 25 cases (75.8%) of PIRS and in 13 cases (52%) of syndromes or other ma lformations. Among the children with type 1 obstruction, 24 (51.1%) were su bmitted exclusively to PPT and 12 (25.5%) to NPI. With the type 2 groups, o nly one (12.5%) received PPT, and three (37.5%) were treated exclusively wi th NPI. All 15 infants treated exclusively with NPI (24.4%) presented with good weight, length, and neuromotor development. Conclusions: Most patients with PIRS and type 1 obstruction improved withou t surgical intervention. NPI should be the initial treatment in all patient s with RS with type 1 and type 2 obstruction who present with important res piratory and feeding difficulties.