Natural History of Spinal Cord Cavernous Malformations: A Multicenter Cohort Study

Abstract

BACKGROUND:The natural history of spinal cord cavernous malformations (SCCMs) remains relatively unclear. OBJECTIVE: To investigate the natural history for hemorrhagic risks and neurological outcomes, as well as relevant predicting factors, of SCCMs. METHODS: All patients between 2002 and 2019 with diagnosis of SCCMs were identified retrospectively. An observational study of patients with conservative management was performed to reveal the natural history of SCCMs. RESULTS: We identified 305 patients in the full cohort, including 126 patients who were conservatively treated for at least 6 months (median observational period, 24.0 months). Fortyfive hemorrhage events occurred during 527 person-years of follow-up, yielding an annual hemorrhage rate of 8.5% per person-year. The 1-, 2-, and 5-year cumulative risks of hemorrhage were 13.9%, 26.1%, and 35.1%, respectively. Prior hemorrhage (hazard ratio [HR] = 12.948, P = .012) and pediatric patients (HR = 2.841, P = .031) were independent predictors of hemorrhage in the long-term follow-up. Familial form (adjusted odds ratio [OR] = 30.695, P = .010) and subsequent hemorrhage events (adjusted OR = 16.333, P = .000) were independent risk factors for worsening of neurological function, and baseline neurological status (adjusted OR = 78.984, P = .000) and presence of subsequent hemorrhage (adjusted OR = 9.611, P = .001) were significantly associated with neurological outcomes. CONCLUSION: The natural history of SCCMs varies. Baseline characteristics, such as pediatric patients, familial form, and baseline neurological status, as well as prior and subsequent hemorrhagic events, significantly affect the natural history of the SCCMs, which prompts a differentiated treatment strategy.

Residual and Recurrent Spinal Cord Cavernous Malformations: Outcomes and Techniques to Optimize Resection and a Systematic Review of the Literature

“…Increased experience leads to less hesitation in both offering surgery early in the clinical course and exploring the resection cavity boundaries. 28 Ren et al 29 found that the annual hemorrhage risk for 305 patients with SCCMs was 8.5% per person-year, and the risk of hemorrhage increased every year (1-year, 2-year, and 5-year cumulative risks were 13.9%, 26.1%, and 35.1%, respectively). Santos et al 30 reported a cumulative 5-year hemorrhage risk of 41.3% among 71 patients.…”

Surgical timing and long-term outcomes in patients with severe haemorrhagic spinal cord cavernous malformations

“…database including data from consecutive patients with spinal vascular malformations from three referral centres. [9][10][11] Patients diagnosed with SCCMs who were surgically treated between January 2002 and December 2021 were selected and retrospectively reviewed from this database.…”

The Reality of Benefit in Surgical Removal for Spinal Cord Cavernous Malformation: Commentary on “Acceptance of Early Surgery for Treatment of Spinal Cord Cavernous Malformation in Contemporary Japan”

“…In this study, prior hemorrhage was independent predictors of subsequent hemorrhage and subsequent hemorrhage events were independent risk factors for worsening of neurological function. 4 Moreover, even in conservative management for SCCMs, 11.3% of cases presented exacerbation of neurological function suggesting the natural history of SCCMs totally seems to be progressive deterioration over time. 2 Therefore, it become a reasonable consensus on surgical removal in symptomatic patients of SCCMs in spite of exiting the potential risk of operative neurological complication.…”

“…From the recent analysis of 305 symptomatic patients (83% of patients presented with hemorrhage), an annual hemorrhage rate was calculated to be 8.5% per person-year with 35.1% of 5-year cumulative hemorrhage risk. In this study, prior hemorrhage was independent predictors of subsequent hemorrhage and subsequent hemorrhage events were independent risk factors for worsening of neurological function [ 4 ]. Moreover, even in conservative management for SCCMs, 11.3% of cases presented exacerbation of neurological function suggesting the natural history of SCCMs totally seems to be progressive deterioration over time [ 2 ].…”

“…They concluded surgery may be postponed after maximum motor recovery if patients have preoperative paresis. In addition, surgical outcome is highly influenced by spinal cord level and location presumed to be negative factors especially in thoracolumbar and deep-seated lesion [ 4 , 8 ]. The potential of neurological recovery is also controversial in accordance with preoperative neurological severality.…”