Dandy-Walker malformation associated with subarachnoid hemorrhage. A case report

Dandy-Walker malformation is a rare congenital brain defect characterized by vermian agenesia with cystic dilatation of the fourth ventricle, and posterior fossa enlargement. The etiology is still poorly understood but is presupposed to be multifactorial, infrequently caused by intracranial hemorrhage. We describe a case of male newborn known to have Dandy-Walker malformation associated with subarachnoid bleeding after the delivery, which is a quiet rare presentation only discussed in a few literatures before.

We present a rare case of a full-term male baby delivered vaginally, who was diagnosed with Dandy-Walker malformation during antenatal anomaly scan. At birth, the baby presented with a weak cry, cyanosis, respiratory distress and seizure. Post-delivery computed tomography scan revealed subarachnoid hemorrhage. In addition, a hydrocephalus was noted on the imaging and treated with ventriculoperitoneal shunt insertion with marked improvement of the posterior fossa cyst and the hydrocephalus as an outcome of early intervention.

Few literature studies showed an association between intracranial bleeding during early fetal life and the development of Dandy-Walker malformation as it affects the posterior fossa components growth. However, our case highlights on an unusual presentation of the spontaneous subarachnoid hemorrhage after the delivery in a full-term baby diagnosed with Dandy-Walker malformation earlier.

This report highlights the importance of early recognition and implementing appropriate management of the hydrocephalus that associated with intracerebral bleeding to prevent the complications of high intracranial pressure plus brainstem herniation and achieve the best possible outcome.