Hemorrhagic Stroke in a Young Adult with Undiagnosed Asymptomatic Dandy–Walker Malformation

Abstract

The Dandy–Walker Malformation was first described in 1914 by Dandy and Blackfan and is characterized by hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses, and anteroposterior enlargement of the posterior fossa. This syndrome commonly manifests as hydrocephalus in children, though rare adult cases have been reported. The literature reveals adult symptomatology including brainstem infarction, psychosis, and neuromuscular disease. Stroke is an exceptionally rare presentation of this malformation, with only one ischemic event reported in the literature. This case offers a rare opportunity for diagnosis in an adult presenting with a hemorrhagic stroke of the basal ganglia in an otherwise asymptomatic young adult male. To the best of our knowledge, this is the first reported case of a hemorrhagic stroke in an adult patient with Dandy–Walker Malformation.

Asymptomatic Dandy Walker malformation in an elderly male with acute haemorrhagic stroke – a case report

“…One plausible explanation is that the hydrocephalus that develops in DWM due to 4th ventricle distention along with hypertension, stresses the cerebral vessels making them prone to rupture. 1 We describe the case of a 60-year-old male with haemorrhagic stroke who was incidentally found to have DWM on imaging. The patient had no symptoms of the disease and led a regular life.…”

“…Unfortunately, the patient died due to acute respiratory distress syndrome. 1 Rakesh reported a case of incidental finding of the Dandy-Walker variant in a 66-year-old male with an ischaemic stroke. The patient had no symptoms of hydrocephalus and cerebellar involvement.…”

“…It is characterised by hypoplasia of the cerebellar vermis, enlarged posterior fossa, and cystic dilatation of the fourth ventricle, displacing the torcula and tentorium upwards. 1 It usually presents in children before the age of 1 year. The most common manifestation is macrocephaly.…”

“…4 Stroke is an extremely rare presentation of DWM, with only one case of haemorrhagic stroke reported in the literature. 1 Here, we have a case of a 60-year-old male presenting with a haemorrhagic stroke who was incidentally diagnosed with DWM based on imaging. To the best of our knowledge, this is the second reported case of an elderly male with DWM in the literature presenting as haemorrhagic stroke who had no symptoms of the disease.…”