Obstructive hydrocephalus secondary to an anterior mesencephalic cavernous malformation with familial cerebral cavernous malformation syndrome: A case report

Cerebral Cavernous malformations (CCM) are the second most common vascular malformation of the central nervous system accounting (CNS) for 5%-10% of vascular malformations in the CNS with a prevalence of 0.5%. Most CCM are supratentorial with fewer than 20% located in the brainstem, mostly in the pons but very rarely in the anterior mesencephalon. The optimal treatment strategy for anterior mesencephalic cerebral cavernous malformations (AMCCM) is still debatable. Up to 20% of CCM are familial with patients presenting with multiple CCM. We report the case of a 62 year-old female who presented with diplopia, gait disturbance and poor speech. No clinical improvement was noted after an initial treatment with steroids for a supposedly unidentified neuroinflammatory disease. When she later presented with impaired consciousness, aphasia, right abducens nerve palsy and left hemiparesia, an urgent brain MRI with gradient echo sequence revealed an obstructive hydrocephalus secondary to an anterior mesencephalic cerebral cavernous malformation with subacute hemorrhage (Zabramski type I) along with numerous CCM typical of familial cerebral cavernous malformation (FCCM) syndrome. The urgent insertion of a ventriculoperitoneal (VP) shunt allowed progressive recovery of a normal consciousness as well as a normal motor function in all 4 limbs, which enabled her to regain autonomous ambulation albeit with a broad-based gait. No CCM re-hemorrhage occurred after 9 months of follow-up. Obstructive hydrocephalus secondary to anterior mesencephalic cerebral cavernous malformations was treated with VP shunt without re-hemorrhage.