A taxonomy for cerebellar cavernous malformations: subtypes of cerebellar lesions

Abstract

OBJECTIVE An anatomical taxonomy has been established to guide surgical approach selection for resecting brainstem and deep and superficial cerebral cavernous malformations (CMs). The authors propose a novel taxonomy for cerebellar CMs, introduce 6 distinct neuroanatomical subtypes, and assess their clinical outcomes. METHODS This bi-institutional, 2-surgeon cohort study included 143 cerebellar CMs that were microsurgically treated over a 25-year period. The proposed taxonomy classifies cerebellar CMs into 6 subtypes on the basis of anatomical location as identified on preoperative MR imaging. Neurological outcomes were assessed using the modified Rankin Scale (mRS), and outcomes were compared among the subtypes, with favorable outcomes defined as mRS scores = 2. RESULTS A total of 143 cerebellar CMs were resected in 140 patients. The mean (SD) age was 42.3 (15.2) years; 86 (60%) of the cerebellar CMs were in women, and 57 (40%) were in men. Cerebellar subtypes were suboccipital (17%, 25/143); tentorial (9%, 13/143); petrosal (43%, 62/143); vermian (13%, 18/143); tonsillar (2%, 3/143); and deep nuclear (15%, 22/143). Overall, 78 of 143 (55%) cerebellar CMs presenting to a cerebellar surface were resected without tissue transgression, and the remaining CMs (65/143, 45%) required translobular or transsulcal approaches. Complete resection was achieved in 134 of 143 cases (94%). Favorable outcomes were achieved in 91% (129/141) of cases with followup at a mean (SD) follow-up duration of 37.4 (53.8) months. Relative outcomes were unchanged or improved relative to the preoperative baseline in 93% (131/141) of cases with follow-up, without differences between subtypes. CONCLUSIONS Most cerebellar CMs are convexity lesions that do not require deep dissection. However, transsulcal and fissural approaches are used for those beneath the cerebellar surface to minimize tissue transgression and preserve associated function. Complete resection without any new deficit is accomplished in most patients. The proposed taxonomy for cerebellar CMs (suboccipital, tentorial, petrosal, vermian, tonsillar, and deep nuclear) guides the selection of craniotomy and approach to enhance patient safety and optimize neurological outcomes.