How to Treat Hemorrhagic Moyamoya Disease: Effective Strategies for Brain Bleeding

Understanding Hemorrhagic Moyamoya and Its Risks

Moyamoya disease is a rare cerebrovascular disorder characterized by the progressive narrowing of arteries in the brain, leading to reduced blood flow. In hemorrhagic cases, fragile collateral vessels may rupture, causing intracranial bleeding. This can manifest as intraventricular hemorrhage, subarachnoid hemorrhage, or even brain parenchymal bleeding. These conditions require immediate medical intervention to prevent severe neurological damage or fatality.

Immediate Treatment: External Ventricular Drainage (EVD)

External ventricular drainage plays a critical role in managing acute bleeding within the brain's ventricles. When blood fills the ventricular system, it obstructs normal cerebrospinal fluid (CSF) circulation, increasing intracranial pressure. A drainage catheter is inserted to remove bloody CSF and decompress the brain. In cases with large clots, physicians may administer urokinase directly into the ventricles to dissolve the clot and improve drainage efficiency. This procedure helps stabilize the patient and prevents secondary brain injury.

Managing Parenchymal Hemorrhage Without Surgery

For patients with small to moderate-sized brain bleeds that do not meet surgical criteria, conservative management is often recommended. This includes osmotic diuretics such as mannitol to reduce cerebral edema and strict control of blood pressure to prevent rebleeding. Close monitoring in a neurocritical care unit ensures timely response to any neurological deterioration. Once the patient stabilizes, typically after 2–3 months, definitive surgical treatment like direct or indirect revascularization procedures can be considered to restore long-term cerebral perfusion.

Surgical Intervention for Large Hematomas

In cases of extensive intraparenchymal hemorrhage, surgical evacuation of the hematoma becomes necessary to relieve mass effect and prevent herniation. During this procedure, neurosurgeons may combine hematoma removal with encephalo-myo-synangiosis (EMS)—a technique involving the placement of temporalis muscle onto the brain surface to promote new blood vessel formation. Additionally, dural inversion may be performed to enhance vascular growth from the external carotid system, improving cortical perfusion over time.

Addressing Subarachnoid Hemorrhage and Associated Aneurysms

When minor subarachnoid hemorrhage occurs alongside aneurysms—common in advanced moyamoya—targeted interventions are essential. These aneurysms, often located on fragile collateral vessels, pose a high risk of rupture. Treatment options include surgical clipping or endovascular coiling to secure the aneurysm and prevent future bleeding. Following aneurysm management, indirect bypass techniques such as temporal muscle grafting and dural flipping are performed to augment cerebral blood supply and reduce stroke risk.

Long-Term Management and Recovery Outlook

Successful treatment of hemorrhagic moyamoya requires a multidisciplinary approach involving neurologists, neurosurgeons, and rehabilitation specialists. Postoperative care focuses on preventing complications like seizures, hydrocephalus, or recurrent strokes. Physical therapy, cognitive support, and regular imaging follow-ups are crucial for optimal recovery. With timely diagnosis and comprehensive treatment, many patients achieve improved neurological function and a better quality of life.