Background: Due to its eloquent location and potentially devastating neurological consequences, the management of brainstem cavernous malformations (CCMs) attracts considerable debate. There is currently a paucity of Level 1 evidence for their management. The aim of this literature review is to explore the current evidence on the risk-benefit profile of different management options.

Methods: A systemic literature search, following the PRISMA algorithm was performed on publications between 2010 and 2018 using the Pubmed database, with the relevant keywords. Only English articles were included. Articles focusing on spinal CCMs and studies with less than 30 participants were excluded.

Results: A total of 222 search results were reviewed and after removal of duplicates and screening of abstracts, 28 clinical papers comprising 30 or more brainstem CCM cases were included in the study. The heterogeneity of the publications precluded a formal meta-analysis of results. The general consensus is that for CCMs presenting with severe symptoms and/or multiple haemorrhages that reach an accessible pial surface, surgery is considered to be the gold-standard treatment, with some authors suggesting the optimal timing to be within two to six weeks of ictus. For those patients with multiple, deep-seated CCM related haemorrhages that do not reach the pial surface, stereotactic radiosurgery (SRS) can be considered. Conservative treatment is generally considered in incidental cases. Management of brainstem cavernomas of other categories still remains controversial.

Conclusions: Due to their highly eloquent location, brainstem CCMs are challenging lesions to manage. Management must be balanced by the risk-benefit profile and tailored to the individual patients and their treating clinicians. This review provides a comprehensive reference considering all treatment options and provides a basis for evidence-based patient counselling.