Clinical course of untreated thalamic cavernous malformations: hemorrhage risk and neurological outcomes.
OBJECTIVE The natural history of cerebral cavernous malformations (CMs) has been widely studied, but the clinical course of untreated thalamic CMs is largely unknown. Hemorrhage of these lesions can be devastating. The authors undertook this study to obtain a prospective hemorrhage rate and provide a better understanding of the prognosis of untreated thalamic CMs. METHODS This longitudinal cohort study included patients with thalamic CMs who were diagnosed between 2000 and 2015. Clinical data were recorded, radiological studies were extensively reviewed, and follow-up evaluations were performed. RESULTS A total of 121 patients were included in the study (56.2% female), with a mean follow-up duration of 3.6 years. The overall annual hemorrhage rate (subsequent to the initial presentation) was calculated to be 9.7% based on the occurrence of 42 hemorrhages over 433.1 patient-years. This rate was highest in patients (n = 87) who initially presented with hemorrhage and focal neurological deficits (FNDs) (14.1%) (χ2= 15.358, p < 0.001), followed by patients (n = 19) with hemorrhage but without FND (4.5%) and patients (n = 15) without hemorrhage regardless of symptoms (1.2%). The initial patient presentations of hemorrhage with FND (hazard ratio [HR] 2.767, 95% CI 1.336-5.731, p = 0.006) and associated developmental venous anomaly (DVA) (HR 2.510, 95% CI 1.275-4.942, p = 0.008) were identified as independent hemorrhage risk factors. The annual hemorrhage rate was significantly higher in patients with hemorrhagic pres entation at diagnosis (11.7%, p = 0.004) or DVA (15.7%, p = 0.002). Compared with the modified Rankin Scale (mRS) score at diagnosis (mean 2.2), the final mRS score (mean 2.0) was improved in 37 patients (30.6%), stable in 59 patients (48.8%), and worse in 25 patients (20.7%). Lesion size (odds ratio [OR] per 0.1 cm increase 3.410, 95% CI 1.272-9.146, p = 0.015) and mRS score at diagnosis (OR per 1 point increase 3.548, 95% CI 1.815-6.937, p < 0.001) were independent adverse risk factors for poor neurological outcome (mRS score ≥ 2). Patients experiencing hemorrhage after the initial ictus (OR per 1 ictus increase 6.923, 95% CI 3.023-15.855, p < 0.001) had a greater chance of worsened neurological status. CONCLUSIONS This study verified the adverse predictors for hemorrhage and functional outcomes of thalamic CMs and demonstrated an overall annual symptomatic hemorrhage rate of 9.7% after the initial presentation. These findings and the mode of initial presentation are useful for clinicians and patients when selecting an appropriate treatment, although the tertiary referral bias of the series should be taken into account.
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Kai-Bing
KB
Tian
Department of Obstetrics and Gynecology, First Affiliated Hospital of Wenzhou Medical University, Wenzhou, People's Republic of China.
Jing-Jie
JJ
Zheng
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Jun-Peng
JP
Ma
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Shu-Yu
SY
Hao
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Liang
L
Wang
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Li-Wei
LW
Zhang
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Zhen
Z
Wu
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Jun-Ting
JT
Zhang
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.|Beijing Key Laboratory of Brian Tumor, Beijing; and.
Da
D
Li
D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000150:complications*; D002543:Cerebral Hemorrhage / Q000453:epidemiology / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D020786:Hemangioma, Cavernous, Central Nervous System / Q000150:complications*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology; D011379:Prognosis; D012307:Risk Factors; D013788:Thalamus / Q000002:abnormalities*; D055815:Young Adult
CCM = cerebral cavernous malformation; CM = cavernous malformation; DVA = developmental venous anomaly; FND = focal neurological deficit; GK = Gamma Knife; HR = hazard ratio; OR = odds ratio; cavernoma; cavernous malformation; mRS = modified Rankin Scale; natural history; thalamus; vascular disorders