Objective To investigate the long term outcomes after conservative and surgical treatment for haemorrhagic moyamoya disease.

Methods 97 consecutive patients with haemorrhagic moyamoya disease from 1997 to 2009 were enrolled in this study (mean age 31±10 years; range 5–56 years). We reviewed the clinical charts and radiographs of patients at the first bleeding episode. Follow-up was obtained prospectively by questionnaires and radiographic examinations. Outcomes were compared based on initial treatment (conservative vs surgical).

Results After a median follow-up of 7.1 years, 21 of the 97 (21.7%) patients developed a second episode of bleeding, and six patients (6.2%) died of intracranial rebleeding. The median interval from initial episode to subsequent rebleeding was 9.1 years (0.1–23.2 years). 17 of 43 (37.1%) conservatively treated patients and four of 54 (7.4%) surgically treated patients experienced a rebleeding event (OR 8.1; 95% CI 2.4 to 26.8; p<0.001). There was a difference in the Kaplan–Meier curve of rebleeding between the two groups (Breslow test p=0.047; log rank test p=0.05). The rebleeding ratio in patients who underwent direct bypass was lower than that in patients treated with indirect bypass alone (0% vs 28.5%, 95% CI 1.0 to 1.9; p=0.002). No significant correlation was found between rebleeding and the patient's age, sex, location of haemorrhage, hypertension status or presence of cerebral aneurysm (p>0.05).

Conclusions There is a high risk of rebleeding after the first haemorrhagic episode in Chinese patients with haemorrhagic moyamoya disease. Revascularisation surgery can improve regional blood flow and have greater efficacy at preventing rebleeding than conservative treatment.