Emine Arzu Kose1, Bulent Bakar2, Gokay Ates3, Didem Aliefendioglu4, Alpaslan Apan3. 1. Department of Anesthesiology and Reanimation, School of Medicine, Kirikkale University, Kirikkale, Turkey. Electronic address: arzuhct@hotmail.com. 2. Department of Neurosurgery, School of Medicine, Kirikkale University, Kirikkale, Turkey. 3. Department of Anesthesiology and Reanimation, School of Medicine, Kirikkale University, Kirikkale, Turkey. 4. Department of Neonatology, School of Medicine, Kirikkale University, Kirikkale, Turkey.
Abstract
BACKGROUND AND OBJECTIVES: Walker-Warburg Syndrome is a rare, autosomal recessive congenital muscular dystrophy manifested by central nervous system, eye malformations and possible multisystem involvement. The diagnosis is established by the presence of four criteria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinal malformation. Most of the syndromic children die in the first three years of life because of respiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation. CASE REPORT: The anesthetic management of a two-months-old male child listed for elective ventriculo-peritoneal shunt operation was discussed. CONCLUSIONS: A careful anesthetic management is necessary due to the multisystem involvement. We reported anesthetic management of a two-months-old male child with Walker-Warburg Syndrome who was listed for elective ventriculo-peritoneal shunt operation.
BACKGROUND AND OBJECTIVES:Walker-Warburg Syndrome is a rare, autosomal recessive congenital muscular dystrophy manifested by central nervous system, eye malformations and possible multisystem involvement. The diagnosis is established by the presence of four criteria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinal malformation. Most of the syndromic children die in the first three years of life because of respiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation. CASE REPORT: The anesthetic management of a two-months-old male child listed for elective ventriculo-peritoneal shunt operation was discussed. CONCLUSIONS: A careful anesthetic management is necessary due to the multisystem involvement. We reported anesthetic management of a two-months-old male child with Walker-Warburg Syndrome who was listed for elective ventriculo-peritoneal shunt operation.