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Natural Course of Moyamoya Disease in Patients with Prior Hemorrhagic Stroke Clinical Outcome and Risk Factors

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Natural Course of Moyamoya Disease in Patients with Prior Hemorrhagic Stroke Clinical Outcome and Risk Factors Original Contribution Natural Course of Moyamoya Disease in Patients With Prior Hemorrhagic Stroke Clinical Outcome and Risk Factors Shuai Kang, MD*; Xingju Liu, MD*; Dong Zhang, MD; Rong Wang, MD; Yan Zhang, MD; Qian Zhang, MD; Wuyang Yang, MD; Ji-zong Zhao, MD Background and Purpose—Evidence on the natural history of hemorrhagic moyamoya disease is still insufficient. We investigated the incidence of recurrent intracranial bleeding, mortality, and risk factors for rebleeding in patients with moyamoya disease. Methods—A total of 128 conservatively managed patients with hemorrhagic presentation and complete follow-up data were included. Recurrent hemorrhages during long-term follow-up were documented. Annual and cumulative incidence rate of bleeding was generated via Kaplan-Meier survival analysis, and risk factors were analyzed using logistic regression analysis. Results—The median follow-up time was 10.1 (1–27) years. During a total of 1300.7 patient-years, 47 (36.7%) patients experienced 59 occurrences of recurrent hemorrhages, rendering an average annual incidence of 4.5%. Among them, 9 patients (19.1%) died from rebleeding and 12 patients sustained severe disability (modified Ranking scale score of≥ 3). The cumulative risk of rebleeding was 7.8% at 5 years, 22.6% at 10 years, and 35.9% at 15 years. Only 4 (3.1%) patients experienced ischemic stroke, yielding an average annual incidence of 0.3%. Multivariate analysis showed that smoking (odds ratio, 4.85; P=0.04) was an independent risk factor of rebleeding. Rebleeding (hazard ratio, 11.04; P=0.02) and hypertension (hazard ratio, 4.16; P=0.04) were associated with increased mortality. Age, type of initial bleeding, digital subtraction angiography staging, family history, and coexisting cerebral aneurysms were not associated with increased risk of rebleeding. Conclusions—Rebleeding events were common and the main cause of death in patients with hemorrhagic moyamoya Downloaded from http://ahajournals.org by on April 1, 2019 disease. The risk of rebleeding steadily increased during long-term follow-up. Smoking was a risk factor for rebleeding, and hypertension was associated with increased mortality. (Stroke. 2019;50:00-00. DOI: 10.1161/ STROKEAHA.118.022771.) Key Words: hemorrhage ◼ hypertension ◼ moyamoya disease ◼ natural history ◼ stroke oyamoya disease (MMD) is a chronic, progressive, revascularization for hemorrhagic MMD remains controver- Msteno-occlusive disorder at the distal carotid and sial. The recent Japanese Adult Moyamoya trial demonstrated proximal middle and anterior cerebral arteries. Intracranial the protective effect of direct bypass against rebleeding, ischemia and hemorrhage are the 2 main manifestations as- whereas other studies have reported suboptimal outcomes in sociated with this disease. The exact underlying mechanism similar settings.2,5–7 At present, it is challenging to evaluate of MMD remains unknown. Although intracranial hemor- the outcome of surgical management of hemorrhagic MMD, rhage was less common than ischemic attack, it is the major largely because of the paucity of data on long-term natural cause of death in patients with MMD.1,2 The reported mor- history of hemorrhage. tality rate of intracranial hemorrhage in patients with MMD Recent studies have shown that the proportions of hem- may be as low as 6.8% or as high as 28.6%.3,4 Key among the orrhagic MMD in mainland China and Taiwan are much goals of treating hemorrhagic MMD is to reduce the risk of higher than that of Japan.8–10 The mean follow-up period per future intracranial hemorrhages. However, the effectiveness of patient was <5 years in most studies, and evidence is limited Received July 20, 2018; final revision received January 11, 2019; accepted January 30, 2019. From the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, China (S.K., X.L., D.Z., R.W., Y.Z., Q.Z., J.-z.Z.); China National Clinical Research Center for Neurological Diseases, Beijing (S.K., X.L., D.Z., R.W., Y.Z., Q.Z., J.-z.Z.); Center of Stroke, Beijing Institute for Brain Disorder, China (S.K., X.L., D.Z., R.W., Y.Z., Q.Z., J.-z.Z.); and Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, MD (W.Y.). *Drs Kang and Liu contributed equally. The online-only Data Supplement is available with this article at https://www.ahajournals.org/doi/suppl/10.1161/STROKEAHA.118.022771. Correspondence to Ji-zong Zhao, MD, Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan Xili, Chongwen District, Beijing 100050, China, Email [email protected] or Dong Zhang, MD, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan Xili, Chongwen District, Beijing 100050, China, Email [email protected] © 2019 American Heart Association, Inc. Stroke is available at https://www.ahajournals.org/journal/str DOI: 10.1161/STROKEAHA.118.022771 1 2 Stroke May 2019 in incidence hemorrhagic risk based on long-term natural his- before follow-up and included age (continuous variable), sex, types tory, such as the rebleeding rate, cumulative incidence, and of hemorrhage, coexisting with intracranial aneurysms, cigarette- risk factors. To better chart the natural course of the disease, smoking status or alcohol consumption, a family history of MMD, hypertension (systolic blood pressure >140 mm Hg or diastolic blood we observed the clinical course of 128 patients over a long- pressure >90 mm Hg, or use of antihypertensive medication), DSA term follow-up. stage, and characteristics of brain CT perfusion. A 2-tailed P value <0.05 was considered statistically significant. Methods Patients and Materials Results The data that support the findings of this study are available from the Patient Characteristics corresponding author on reasonable request. The final cohort consisted of 128 patients. Baseline presentation We conducted a retrospective study with long-term follow-up assessing the natural history of hemorrhagic MMD. This study was and characteristics of the patient cohort are presented in Table 1. approved by the Beijing Tiantan Hospital Institutional Review Board. The mean age of the patients at the time of the first bleeding The goal is to determine the risk of recurrent hemorrhages. From episode was 34.5±9.7 years (range, 8–61 years), and 95% were 1985 to 2012, a total of 274 patients with hemorrhagic MMD were adult patients. The female-to-male ratio was 2:1. Intraventricular identified in Beijing Tiantan Hospital. Among them, 128 patients who were treated conservatively were included in this study. The diagnosis hemorrhage and intracerebral hemorrhage were the most fre- of MMD was based on digital subtraction angiography (DSA). All quent presentations observed on CT scans, accounting for 43.0% patients initially presented with intracranial hemorrhage, which was and 28.9%, respectively. The DSA stage of patients mostly were confirmed by brain computed tomographic (CT) scan. It should be noted that Beijing Tiantan Hospital is one of the major referral cen- ters for MMD in China; therefore, most patients in our study cohort Table 1. Patient Demographics and Baseline Characteristics Grouped by were not in the acute stage of hemorrhage. Conservative treatment is Rebleeding determined on a basis of shared decision-making between physicians All No and patients and their family members. Provided with the rarity of Patients Rebleeding Rebleeding disease, there was no specific treatment protocol established for these Demographics (N=128) (n=81) (n=47) P Value patients. Patients with ischemic symptoms as the initial presentation, pseudo-MMD, and moyamoya syndrome caused by other systemic Women, % 86 (67) 50 (61) 36 (76) 0.08 diseases were excluded. Age, y; mean±SD 34.5±9.7 35.0±9.7 33.7±9.9 0.47 Follow-Up Methods History of risk factors, % Downloaded from http://ahajournals.org by on April 1, 2019 Baseline clinical characteristics and imaging data were reviewed, in- Family MMD 9 (7.0) 5 (6.1) 4 (8.5) 0.88 cluding age, sex, risk factors (hypertension, family history of MMD, Hypertension 15 (11.7) 7 (8.6) 8 (17.0) 0.15 smoking, drinking, and coexisting intracranial aneurysms), disease stage based on DSA review, and types of hemorrhage (intracerebral Aneurysm 14 (10.9) 10 (12.3) 4 (8.5) 0.70 hemorrhage, intraventricular hemorrhage, and subarachnoid hemor- rhage). All patients were solicited for annual follow-up visits in outpa- Smoking 16 (12.5) 6 (7.4) 10 (21.2) 0.02 tient clinic. At each visit, brain CT angiography or magnetic resonance Drinking 10 (7.8) 3 (3.7) 7 (14.8) 0.03 angiography was performed to evaluate cerebral vessels. In addition, brain CT scans were performed when patients experienced a recurrent Types of hemorrhage, % hemorrhage. Overall, all patients had completed annual follow-up vis- ICH 37 (28.9) 20 (24.7) 17 (36.1) 0.16 its in outpatient clinic at the first 3 years. For patients who were una- vailable for in-person interviews, we attempted to obtain basic clinical IVH with ICH 22 (17.1) 15 (18.5) 7 (14.8) 0.60 information over the phone for the assessment of onset of rebleeding, IVH 55 (43.0) 38 (46.9) 17 (36.1) 0.23 ischemic events, and survival status. The time interval from the in- itial episode to rebleed, site and treatment of recurrent hemorrhage SAH 14 (10.9) 8 (9.9) 6 (12.7) 0.61 were investigated. The following items constitute primary end points: (1) patient’s death from recurrent bleeding or other medical cause and DSA stage, % (2) patients who received bypass surgery. Clinical functional outcome I 6 (4.6) 4 (4.9) 2 (4.2) 0.81 after hemorrhage was recorded using the modified Rankin scale. II 15 (11.7) 10 (12.3) 5 (10.6) 0.77 Data Analysis III 42 (32.8) 26 (32.0) 16 (34.0) 0.82 All analyses were conducted using IBM SPSS statistical software IV 40 (31.2) 23 (28.3) 17 (36.1) 0.36 (version 19.0).
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