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Revista Brasileira de Anestesiologia

Print version ISSN 0034-7094

Abstract

OLIVEIRA, Carlos Rogério Degrandi  and  ELIAS, Luciana. Anesthesia in patient with Rubinstein-Taybi syndrome: case report. Rev. Bras. Anestesiol. [online]. 2005, vol.55, n.5, pp. 546-551. ISSN 0034-7094.  http://dx.doi.org/10.1590/S0034-70942005000500010.

BACKGROUND AND OBJECTIVES: Rubinstein-Taybi syndrome (RTS) is a genetic disease caused by mutation or deletion of chromosome 16, and characterized by growth and mental retardation, clubbing thumbs and toes and craniofacial abnormalities. There is little information about RTS in the anesthetic literature. This report aimed at describing the anesthetic management of a patient with Rubinstein-Taybi syndrome submitted to dentistry surgery  and at discussing aspects of this syndrome that may be relevant to anesthesia. CASE REPORT: Male patient, 9-year-old, 28 kg, to be submitted to general anesthesia for extraction of abnormally positioned teeth. Patient presented with typical RTS characteristics (growth and mental retardation, clubbing thumbs and toes and craniofacial abnormalities). Anesthesia was induced and initially maintained with sevoflurane under facial mask; after tracheal intubation it was maintained under manually controlled ventilation with Bain system. There have been no complications and procedure was performed in outpatient regimen. CONCLUSIONS: The importance of thorough preanesthetic evaluation is emphasized due to possible associated malformations, including heart diseases. In this particular case, there was no heart disease, which is reported to be present in one-third of cases. Attention to difficult airway maintenance is recommended.

Keywords : ANESTHESIA [Pediatric]; DISEASES [Rubinstein-Taybi syndrome].

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