A taxonomy for brainstem cavernous malformations: subtypes of pontine lesions. Part 2: inferior peduncular, rhomboid, and supraolivary.

Abstract

Part 2 of this 2-part series on pontine cavernomas presents the taxonomy for subtypes 4-6: inferior peduncular (IP) (subtype 4), rhomboid (5), and supraolivary (6). (Subtypes 1-3 are presented in Part 1.) The authors have proposed a novel taxonomy for pontine cavernous malformations based on clinical presentation (syndromes) and anatomical location (MRI findings). The details of taxonomy development are described fully in Part 1 of this series. In brief, pontine lesions (323 of 601 [53.7%] total lesions) were subtyped on the basis of predominant surface presentation identified on preoperative MRI. Neurological outcomes were assessed according to the modified Rankin Scale, with score ≤ 2 defined as favorable. The 323 pontine brainstem cavernous malformations were classified into 6 distinct subtypes: basilar (6 [1.9%]), peritrigeminal (53 [16.4%]), middle peduncular (100 [31.0%]), IP (47 [14.6%]), rhomboid (80 [24.8%]), and supraolivary (37 [11.5%]). Subtypes 4-6 are the subject of the current report. IP lesions are located in the inferolateral pons and are associated with acute vestibular syndrome. Rhomboid lesions present to the fourth ventricle floor and are associated with disconjugate eye movements. Larger lesions may cause ipsilateral facial weakness. Supraolivary lesions present to the surface at the ventral pontine underbelly. Ipsilateral abducens palsy is a strong localizing sign for this subtype. A single surgical approach and strategy were preferred for subtypes 4-6: for IP cavernomas, the suboccipital craniotomy and telovelar approach predominated; for rhomboid lesions, the suboccipital craniotomy and transventricular approach were preferred; and for supraolivary malformations, the far lateral craniotomy and transpontomedullary sulcus approach were preferred. Favorable outcomes were observed in 132 of 150 (88%) patients with follow-up. There were no significant differences in outcomes between subtypes. The neurological symptoms and signs associated with a hemorrhagic pontine subtype can help define that subtype clinically with key localizing signs. The proposed taxonomy for pontine cavernous malformation subtypes 4-6 meaningfully guides surgical strategy and may improve patient outcomes.