OBJECTIVE AND IMPORTANCE: Prevention of rebleeding is the most important aspect of the management of hemorrhagic moyamoya disease, because rebleeding causes significant morbidity and mortality. CLINICAL PRESENTATION: A 26-year-old male patient with a history of moyamoya disease since the age of 3 years and multiple strokes was in a semicomatose state at presentation. He was found to have intraventricular and periventricular hemorrhages abutting the atrium of the right ventricle. His hospital course was complicated by a second hemorrhage. Both bleeding events were believed to be secondary to a ruptured right lateral posterior choroidal aneurysm. INTERVENTION: The aneurysm was excised and revealed histopathology consistent with a true saccular aneurysm. Frameless stereotactic guidance was used during surgery to minimize damage to collateral vessels and to shorten the surgical corridor. CONCLUSION: The management of hemorrhagic moyamoya disease should be modified based on the source of hemorrhage and its relation to a specifically located aneurysm. In the case of aneurysms arising from the choroidal artery, the general belief is that most of these represent pseudoaneurysms and have a tendency to regress spontaneously. Because of the rebleeding risk, we recommend early intervention in treating ruptured intracranial aneurysms using the least invasive surgical techniques.
OBJECTIVE AND IMPORTANCE: Prevention of rebleeding is the most important aspect of the management of hemorrhagic moyamoya disease, because rebleeding causes significant morbidity and mortality. CLINICAL PRESENTATION: A 26-year-old male patient with a history of moyamoya disease since the age of 3 years and multiple strokes was in a semicomatose state at presentation. He was found to have intraventricular and periventricular hemorrhages abutting the atrium of the right ventricle. His hospital course was complicated by a second hemorrhage. Both bleeding events were believed to be secondary to a ruptured right lateral posterior choroidal aneurysm. INTERVENTION: The aneurysm was excised and revealed histopathology consistent with a true saccular aneurysm. Frameless stereotactic guidance was used during surgery to minimize damage to collateral vessels and to shorten the surgical corridor. CONCLUSION: The management of hemorrhagic moyamoya disease should be modified based on the source of hemorrhage and its relation to a specifically located aneurysm. In the case of aneurysms arising from the choroidal artery, the general belief is that most of these represent pseudoaneurysms and have a tendency to regress spontaneously. Because of the rebleeding risk, we recommend early intervention in treating ruptured intracranial aneurysms using the least invasive surgical techniques.