| Objective: Summarize the clinical features and neuroimaging of subarachnoid hemorrhagic moyamoya disease(MMD) by collecting and analyzing the clinical and imaging data. Observe the surgical efficacy of encephalo-duro-arterio-synangiosis(EDAS) for subarachnoid hemorrhagic moyamoya disease by following up.Methods: Retrospectively analyze the moyamoya disease patients admitted in 307 Hospital, PLA from Januuary 2002 to April 2013, and choose the subarachnoid hemorrhagic moyamoya disease patients according to the inclusion and exclusion criteria. Collect the clinical data, such as gender, age, bleeding time, onset performance, operation time and sides, from the medical records. The right and left cerebral hemispheres of each patient were identified as hemorrhagic or non-hemorrhagic according to the location of subarachnoid hemorrhage(SAH). Collect the imaging data of hemorrhagic and non-hemorrhagic hemispheres, such as Suzuki stage, anterior choroidal artery(ACh A) grade, posterior communicating artery(PCo A) grade, transdural anastomosis grade and posterior circulation compensation grade, from the results of digital subtraction angiography(DSA). Follow-up was conducted for each patient by hospitalization, telephone or letter. Collect the rebleeding data and analyze using Kaplan-Meier survival analysis. Use modified Rankin Scale(m RS) to evaluate the clinical symptoms and compare the m RS scores of preoperative and postoperative. Use positron emission computed tomography(PET) to evaluate the cerebral metabolism and compare the cerebral metabolism of preoperative and postoperative. To evaluate the revascularization efficacy, the superficial temporal artery(STA) compensation was divided into 4 grades, grade I was considered inefficaciousand grade II to IV were considered efficacious. All analyses were performed with SPSS software for Windows, version 17.0, and differences of P<0.05 were considered statistically significant. Summarize the clinical and imaging features of subarachnoid hemorrhagic moyamoya disease and analyze the cause of bleeding and efficacy of EDAS according to the results of statistical analysis.Results: 1.Clinical features. Three hundred fifhty-three hemorrhagic MMD patients were treated in our cohort between January 2002 and April 2013. Among these patients, the first hemorrhage episodes were intraventricular hemorrhages(IVHs) in 139(39.3%) patients, intracerebral hemorrhages(ICH) in 127(36.0%), intracerebral hemorrhages breaking into ventricles in 49(13.9%) and subarachnoid hemorrhages in 38(10.8%) patients. There were 9 men and 29 women among the 34 patients, and the ratio of female to male patients was 3.22:1. The mean age at hemorrhage onset was 38.55±11.74(range, 12-59) years old, and 37 of the patients were adults. Only one patient was under 18 years old. A single peak pattern of age distribution was revealed, and the peak was 40 to 49 ages. For the hemorrhage onset performance, 24 patients were headache, 11 patients were disturbance of consciousness and 3 patients were weakness. Thirty-two of the patients suffered only one episode of hemorrhage prior to surgery, and six of the patients experienced second episodes of hemorrhage prior to surgery. All of the rebleedings occurred in the same side as the first episode of hemorrhage. Among the second hemorrhages, one was an intracerebral hemorrhage, two were intraventricular hemorrhages, two were intracerebral hemorrhages extending to the ventricles and one was an subarachnoid hemorrhage.And the time of rebleeding was 1day, 18 days, 1month, 5.5years, 7years and 10.7 years respectively.2. Neuroimaging. Regarding the sides of the hemorrhages, left-side hemorrhages accounted for 65.8%(25hemorrhagic hemispheres), and 13 of the right hemispheres were hemorrhagic. Four patients were found combined with aneurysms, 2 were located in the top of basilar artery, 1 was left posterior cerebral artery and 1 was right middle cerebral artery. For the remaining 34 patients, the Suzuki stage, ACh A grade, PCo A grade, transdural anastomosis grade and posterior circulation compension grade were collected. The majority of the hemorrhagic and non-hemorrhagic hemispheres presented with Suzuki angiographic stages of IV or V, and the difference in the Suzuki angiographic stage distribution between the hemorrhagic and non-hemorrhagic hemispheres was not statistically significant(P=0.114). For the hemorrhagic sides, 1 was stage I, 3 were stage II, 2 were stage III, 11 was stage IV, 11 were stage V and 6 were stage VI. For the non-hemorrhagic sides, 3 were stage I, 6 were stage II, 4 were stage III, 9 was stage IV, 7 were stage V and 5 were stage VI.In 7 of the 34 hemorrhagic sides, the ACh A was dilated and exhibited branching(grades 1 and 2). Among the non-hemorrhagic sides, 8 were dilated and exhibited branching(grade 1 and 2), and the difference was not statistically significant(P=0.770).The rates of PCo A dilatation(grades 1 and 2) in the hemorrhagic and non-hemorrhagic sides were 35.3% and 38.3%, respectively, and this difference was not statistically significant(P=0.801).Twenty-five of the 34 hemorrhagic sides were positive for transdural anastomosis(grade 1), and 15 of the non-hemorrhagic sides were positive for transdural anastomosis. This difference was statistically significant(P =0.026).Twenty-seven of the 34 hemorrhagic sides and 30 of the 34 non-hemorrhagic sides were positive for posterior circulation compensation(grade 1). This rate was higher among the non-hemorrhagic sides, but the difference was not statistically significant(P=0.512).3. Treatment. Twenty-nine(76.8%) of the 38 patients underwent bilateral EDAS surgeries, six(15.8%) patients underwent unilateral EDAS surgeries, and three(7.9%) patients did not undergo revascularization surgery. For the 4 patients with aneurysms,endovascular embolizations were conducted first.4. Follow-up. After a mean follow-up time of 51.34 months(range, 13-125 months), two(5.7%) of the 35 surgically treated patients suffered an episode of rebleeding. One patient developed an episode of intraventricular hemorrhage 2 months after surgety, and another patient died of intracerebral hemorrhage 87 moths after surgery. The Kaplan-Meier survival analysis revealedthe annual rebleeding risk was 2.9%. All of the 3 patients who received no revascularization surgery didn’t suffer rebleeding. The postoperative m RS scores decreased significantly in the 35 surgically treated patients(P=0.000). For the preoperative, 10 patients scored 0, 17 patients scored 1, 2 patients scored 2, 2 patients scored 3, 3 patients scored 4, 1 patient scored 5 and no patient scored 6. For the postoperative, 21 patients scored 0, 9 patients scored 1, 3 patients scored 2, 1 patient scored 3, 1 patient scored 4, and no patient scored 5 or 6. For the 3 patients who received no revascularization surgery, the m RS scores were still 1. After a mean time of 9.75 months(range, 3-30 months), 16 patients rechecked PET. For the hemorrhagic sides, the cerebral metabolism of 11 hemispheres was improved, and 5 hemispheres didn’t change significantly. For the non-hemorrhagic sides, the cerebral metabolism of 6 hemispheres was improved, and 1 hemisphere didn’t change significantly. After a mean time of 13.38 months(range, 5-30 months), 13 patients rechecked DSA. In 10 of the 13 hemorrhagic hemispheres, the revascularizations were efficacious(grade II-IV). Among the 10 non-hemorrhagic hemispheres, 9 hemispheres received efficacious revascularizations(grade II-IV).Conclusions: 1. Subarachnoid hemorrhage is the most rare type of hemorrhage in moyamoya disease patients. It mainly occurs in adult women, and the peak of hemorrhage onset is 40-49 years old.2. The rupture of aneurysm is one of the causes of subarachnoid hemorrhage in moyamoya disease patients, but most of the subarachnoid hemorrhages are nonaneurysmal, and the rupture of the transdural anastomosis might be the main cause of this condition.3. EDAS can achieve efficacious revascularization, improve the cerebral metabolism and finally alleviate clinical symptoms. And on the same time, it may reduce the risk of rebleeding. |
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