Cerebral cavernous malformation: Management, outcomes, and surveillance strategies - A single centre retrospective cohort study

Ciaran S Hill; Anouk Borg; Hugo Layard Horsfall; Alaa Al-Mohammad; Patrick Grover; Neil Kitchen

doi:10.1016/j.clineuro.2022.107576

Cerebral cavernous malformation: Management, outcomes, and surveillance strategies - A single centre retrospective cohort study

Clin Neurol Neurosurg. 2023 Feb:225:107576. doi: 10.1016/j.clineuro.2022.107576. Epub 2022 Dec 26.

Authors

Ciaran S Hill¹, Anouk Borg², Hugo Layard Horsfall², Alaa Al-Mohammad², Patrick Grover², Neil Kitchen²

Affiliations

¹ The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BB, UK; The UCL Cancer Institute, University College London, London WC1E 6DD, UK. Electronic address: ciaran.hill@ucl.ac.uk.
² The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BB, UK.

PMID: 36608471
DOI: 10.1016/j.clineuro.2022.107576

Abstract

Background: Cerebral cavernous malformations (CCM) may undergo a period of clinical and/or radiographical surveillance that precedes or follows definitive treatment. There are no international guidelines on the optimal surveillance strategy. This study describes the surveillance strategies at our centre and explore the related clinical outcomes.

Methods: We performed a retrospective study of adult patients with CCMs referred to a neurovascular service over an 8-year period, to determine the frequency and type of surveillance, intervention, and explore the associated outcomes. We report our findings adhering to STROBE guidelines.

Results: 133 patients (Male:Female 73:60; men age 42 years; range 12-82) were included. CCMs were identified in patients first presenting with symptomatic intracerebral haemorrhage (42.11%); headache, focal neurological deficit, or seizure without haemorrhage (41.35%); or, as an incidental finding (16.54%). The most common CCM location was supratentorial (59.40%), followed by brain stem (21.80%), cerebellum (10.53%) and basal ganglia (6.02%). Of the 133 patients, 77 patients (57.89%) were managed conservatively, 49 patients (36.84%) were managed by surgical resection alone, and seven patients (5.26%) were managed with stereotactic radiosurgery (SRS). Patients follow-up had a mean duration of 65.94 months, and varied widely (SD = 52.59; range 0-265), for a total of 730.83 person-years of follow up. During surveillance, 16 patients suffered an ICH equating to a bleeding rate of 2.19 per 100 patient years. CCMs that increased in size had a higher bleeding rate (p = 8.58 ×10^-4). There were 8 (6.02%) cases where routine clinic review or MRI resulted in a change in management.

Conclusions: Our single centre retrospective study supports existing literature relating to presentation and sequalae of CCM, with an increase in CCM size being associated with higher rates of detected bleeding. There remains heterogeneity, even within a single centre, on the frequency and modality of surveillance. Further, there are no international guidelines or high-quality data that recommends the optimal duration and frequency of surveillance, and its effect on clinical outcomes. This is a future research direction.

Keywords: Cavernoma; Cavernous Malformation; Haemorrhage; Surveillance.

MeSH terms

Adult
Cerebral Hemorrhage / complications
Female
Hemangioma, Cavernous, Central Nervous System* / diagnostic imaging
Hemangioma, Cavernous, Central Nervous System* / surgery
Humans
Magnetic Resonance Imaging
Male
Retrospective Studies
Seizures / complications

Supplementary concepts

Cerebrocostomandibular Syndrome