When do you refer patients diagnosed with Moyamoya disease to neurosurgery?

I almost always refer patients with Moyamoya to neurosurgery. The only treatment for this condition is either direct or indirect bypass surgery. If they have any active symptoms, have an old infarct, or recent stroke visible on scans, surgery is worth considering.

Referral indications include symptomatic individuals or those with impaired blood flow evident on perfusion/vessel imaging. This broadly encompasses the majority of patients. Typically, asymptomatic cases with normal imaging are monitored regularly, essentially awaiting progression as most patients tend to progress. In the absence of randomized trials exploring early surgery before asymptomatic, imaging-based hemodynamic compromise, it becomes a risk-benefit discussion involving you, the patient, and a neurosurgeon. It should be considered, particularly if high-risk comorbidities for vascular compromise are present.