ABSTRACT
Objective To investigate the clinical features, treatment strategies and outcomes of patients with Moyamoya disease and lenticulostriate artery (LSA) aneurysm rupture. Methods Patients with moyamoya disease and LSA aneurysm rupture admitted to Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medicine School from October 2012 to March 2015 were analyzed retrospectively. They were followed up for 1 year. The modified Rankin Scale (mRS) was used to evaluate the outcomes, and 0-2 was defined as good outcome. The demographic characteristics, image anatomical features, treatment schemes, and outcomes of the patients were summarized. The Pubmed database was used to retrieve other similar studies, which combined with this group of cases for analysis. Results A total of 10 patients were enrolled, 2 males and 8 females, aged 29-72 years, with an average of 43. 6 years. All cases were intracranial hemorrhage, including 3 cases of cerebral hemorrhage, 6 cases of ventricular hemorrhage, and 1 case of subarachnoid hemorrhage. At the time of admission, 7 patients had disturbance of consciousness, and 3 patients underwent emergency extraventricular drainage. Among them, 5 patients received endovascular embolization, 4 had good outcome, 1 had mild neurological deficit (mRS score 3); 2 received surgical treatment, all had good outcome; 3 received conservative treatment, all had re-bleeding, 2 died, and 1 had severe disability (mRS score 4). Fourteen eligible articles were included, and a total of 18 patients were included in the analysis: surgical treatment in 7 cases, endovascular treatment in 6 cases, and conservative treatment in 5 cases (self-healing in 1 case). According to the data of this group of patients and literature reports, the good outcome rate of the patients with early interventional embolization or craniotomy clipping treatment was significantly higher than that of conservative treatment (70. 6％vs. 22. 2％; P = 0. 038). Conclusion There is a certain risk of conservative treatment of Moyamoya disease complicated with LSA aneurysm rupture and requires active treatment. Interventional embolization of the parent artery or surgically clipping of aneurysm can effectively improve the clinical outcome of such patients. Interventional embolization of the parent artery and aneurysm can be selected simultaneously if the condition of the parent artery is allowed. Surgery can be selected when the condition of parent artery is poor and the aneurysm is located in the superficial part.