**8. Treatment of MMD**

In the acute stage, treatment is symptomatic for those patients with ischemic or hemorrhagic stroke as per acceptable guidelines. It should be focused on treating elevated intracranial pressure, evacuation of hematoma, draining intraventricular hemorrhage by external ventricular drain (EVD), control of seizures, glycemic control, and treatment of fever.

In children diagnosed with MMD, crying and hyperventilation should be avoided as it can induce cerebral vasoconstriction. Pain management also should be taken care of. Supplemental oxygen also helps in avoiding hypoxia. Generally, hypotension, hyperthermia, hypoxia, hypocarbia, and hypovolemia should be avoided.

There are no interventions proved to improve outcome in MMD. The utility of thrombolysis has not been studied yet and may be risky due to the possibility of development of hemorrhage from the fragile collateral vessels [36]. Aspirin has been recommended by the American College of Chest Physicians (ACCP) for children with ischemic stroke [37].

There were no significant differences in outcome between medically and surgically treated patients with MMD as per a large survey from Japan, but a later review revealed that 38% of 651 patients with MMD who initially underwent medical management ultimately had to go for surgery because of progressive symptoms [38].

#### **8.1 Secondary stroke prevention**

Treatment of underlying diseases like sickle cell disease is helpful in secondary prevention. Surgical revascularization is the mainstay of secondary prevention. Antiplatelet medications like aspirin have an important role in preventing stroke in mild disease or asymptomatic patients who are at high risk for surgery [39]. Oral anticoagulants are not helpful as they can increase the chance of hemorrhage. Calcium channel blockers were used in some reports [40]. Endovascular embolization has been used for the obliteration of aneurysm associated with MMD.

### **9. Surgical management of moyamoya disease**

The aim of surgical management of moyamoya disease is to vascularize and restore the blood supply by bypassing the stenosed area. It will stabilize the cerebrovascular hemodynamics. It prevents bleeding by preventing the progression of the thin moyamoya vessels. Improvement and or normalization of cerebral hemodynamics by the surgical intervention will also prevent secondary stroke prevention and improve the neurological and neurocognitive outcome.

Revascularization surgery is the most effective treatment for hemorrhagic MMD [41] and can be effective in preventing future stroke events in adults [42].

The main indications for the surgical interventions are cerebral hemodynamic impairment and repeat ischemic symptoms. The severity of the disease to assign a patient for surgical intervention can be made by the Berlin moyamoya grading system (**Table 2**). But the literature has not mentioned the severity at which there is an indication for surgical intervention.


#### **Table 2.**

*The Berlin moyamoya grading [43].*
