Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations.
OBJECT.: Cerebral cavernous malformations have been studied widely, but the natural history of brainstem cavernous malformations (CMs) is not well defined, and hemorrhages caused by brainstem CMs are devastating. The goal of this study was to quantify the hemorrhage risks and functional outcomes of patients with brainstem CMs.


METHODS
This prospective, longitudinal, cohort study included patients with brainstem CMs diagnosed between 1985 and 2012. The clinical courses of all patients were recorded. Predictors of hemorrhage and the overall untreated outcomes were evaluated.


RESULTS
A total of 331 patients (46.5% female) were included, with a mean follow-up duration of 6.5 years. The annual hemorrhage rates in patients initially presenting with hemorrhage with (n = 215) or without (n = 34) focal neurological deficits were 15.9% and 12.4%, respectively. However, the annual hemorrhage rate was 8.7% in patients initially presenting without hemorrhage (n = 82). The risk factors for hemorrhage were female sex (hazard ratio [HR] 1.445, p = 0.041), prior hemorrhage (HR 1.277, p = 0.029), and perilesional edema (HR 1.830, p = 0.002). Overall, neurological function at the most recent assessment was improved compared with neurological function at diagnosis. Additionally, 307 patients (92.7%) improved or stabilized, 268 (81.0%) lived independently, and 95 (28.7%) completely recovered. Predictors favoring complete recovery were no prospective hemorrhage (HR 1.958, p = 0.001), younger age (HR 1.268, p = 0.001), and small lesion size (HR 1.578, p = 0.004).


CONCLUSIONS
Patients' initial presentation predicts their prospective annual hemorrhage rate. This study suggests that several strong risk factors for hemorrhage and predictors of brainstem CM outcomes may enable clinicians to evaluate the potential hemorrhage risks of their patients and design personalized treatments.
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
Da
D
Li
Shu-Yu
SY
Hao
Gui-Jun
GJ
Jia
Zhen
Z
Wu
Li-Wei
LW
Zhang
Jun-Ting
JT
Zhang
D000328:Adult; D001933:Brain Stem / Q000473:pathology*; D020295:Brain Stem Neoplasms / Q000150:complications* / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D020786:Hemangioma, Cavernous, Central Nervous System / Q000150:complications* / Q000473:pathology; D006801:Humans; D015994:Incidence; D020300:Intracranial Hemorrhages / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
CM = cavernous malformation; DVA = developmental venous anomaly; GKS = Gamma Knife surgery; HR = hazard ratio; angioma; brainstem; cavernous malformation; mRS = modified Rankin Scale; natural history; vascular disorders