Tectocerebellar dysraphia and occipital encephalocele: an unusual association with abdominal situs inversus and congenital heart disease.

ABSTRACT

Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.