CASE REPORTS   

Minerva Pediatrica 2008 August;60(4):465-8

Copyright © 2008 EDIZIONI MINERVA MEDICA

language: English

Dandy-Walker syndrome together with occipital encephalocele

Cakmak A., Zeyrek D., Cekin A., Karazeybek H.

Department of Pediatrics Harran University School of Medicine Sanliurfa, Turkey

Dandy-Walker malformation is an anomaly characterized by dysgenesis of the foramina of Magendie and Lushka in the upper 4th ventricle, hypoplasia of the cerebellar vermis and agenesis of the corpus callosum. Encephalocele is diagnosed from the calvarium defect, cerebrospinal fluid (CSF) and herniation of the meninges. It is the rarest neural tube defect. A 7x9cm encephalocele was found on physical examination of a 6-day old baby boy patient. From cranial magnetic resonance, it was seen that the posterior fossa was enlarged with cysts and there was agenesis of the vermis. A connection was established between the ventricle and the development of cysts on the posterior fossa. These findings were evaluated as significant from the aspect of Dandy-Walker malformation. The extension of the bone defect in the left occipital area towards the posterior, and the cranio-caudal diameter reaching 9 cm was seen to be in accordance with encephalocele. It is rare for Dandy-Walker syndrome to occur together with occipital encephalocele. The authors present a case of Dandy-Walker syndrome together with occipital encephalocele.