Cerebral cavernous malformation in a child leading to a fatal subarachnoid hemorrhage - "silent but sinister:" A case report and literature review

Abstract

Background: Cerebral cavernous malformations (CCMs), otherwise known as cavernous hemangiomas/cavernomas, are a type of vascular malformation. It is the third most common cerebral vascular malformation, histologically characterized by ectatic, fibrous, blood filled "caverns" with thin-walled vasculature without intervening normal brain parenchyma. Case Description: Herein, we present a case of an original, spontaneous hemorrhage from a sporadic form of CCM without associated gross developmental venous anomaly in an 11-year-old child, which is an extremely rare occurrence, with the special emphasis on the demographic data of the affected population, risk factors associated with hemorrhage, and correlation of histopathological and radiological findings with an in-depth literature review. Conclusion: The significant majority of the CCM are clinically occult. Hence, the development of risk assessment tools and guidelines for timely neurosurgical intervention poses a greater clinical challenge for medical experts rendering the management of the affected individuals with CCM in an anecdotal situation. Presentation of life-threatening rebleeds and neurological deficits in the diagnosed population albeit uncommon is possibly preventable outcomes.