Anesthesia for a child with Walker-Warburg syndrome

Abstract

Background and objectives: Walker-Warburg Syndrome is a rare, autosomal recessive con-genital muscular dystrophy manifested by central nervous system, eye malformations andpossible multisystem involvement. The diagnosis is established by the presence of four crite-ria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinalmalformation. Most of the syndromic children die in the first three years of life because ofrespiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation. Case Report : The anesthetic management of a two-months-old male child listed for electiveventriculo-peritoneal shunt operation was discussed. Conclusions : A careful anesthetic management is necessary due to the multisysteminvolvement. We reported anesthetic management of a two-months-old male child withWalker-Warburg Syndrome who was listed for elective ventriculo-peritoneal shunt operation. (C) 2013 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rightsreserved.