Natural course of untreated spinal cord cavernous malformations: a follow-up study within the initial 5 years after diagnosis

Abstract

OBJECTIVE Cavernous spinal cord malformations (SCMs) are believed to have a high rate of bleeding. The risk of intramedullary hemorrhage (IMH) or recurrent IMH and the neurological impact of bleeding events are important for clinical decision-making and could impact current treatment strategies. METHODS The authors screened their institutional database for patients with cavernous SCM treated between 2003 and 2020. Patients with complete MRI data sets and clinical baseline characteristics were included. Surgically treated patients were censored after cavernous SCM removal. Neurological functional status was obtained using the modified McCormick (MMcC) scale at diagnosis, first IMH, and second IMH. Kaplan-Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for hemorrhage or rehemorrhage. RESULTS Seventy-one patients with cavernous SCM were analyzed. Cox regression analysis identified previous IMH (hazard ratio 7.86, 95% confidence interval 1.01–61.47, p = 0.049) as an independent predictor for rehemorrhage during the 5-year follow-up. The cumulative 5-year risk of bleeding or rebleeding was 41.3% for cavernous SCM. The MMcC score significantly deteriorated in 75% of patients after recurrent hemorrhage (p = 0.012). CONCLUSIONS During untreated 5-year follow-up, a considerably increased risk for hemorrhage or rehemorrhage was found in cavernous malformations of the spinal cord compared to cerebral cavernous malformations. Neurological function significantly deteriorates after the second bleeding. The probability of recurrent IMH increased significantly after initial presentation with hemorrhage.

Central nervous system cavernous malformations: cross‐sectional study assessing rebleeding risk after a second haemorrhage

“…The available literature describes a cumulative risk of ≈20% over a 5‐year timeline for patients with CCM [1, 2, 7, 8], as well as ≈40% for patients with SCM [4, 5, 9]. Risk factors such as ICH/IMH as the mode of presentation or recurrent bleeding significantly increase this risk up to 30% and 55% respectively [1, 4, 5, 20].…”

“…Cavernous malformations (CMs) of the central nervous system (CNS) [1–3] are known to be a major source of intracerebral haemorrhage (ICH), as well as intramedullary haemorrhage (IMH) [1, 4–6]. They have an estimated cumulative risk of ≈20% over a 5‐year timeline for patients with cerebral cavernous malformations (CCMs) [1, 2, 7, 8], as well as ≈40% for patients with cavernous malformations of the spinal cord (SCM) [4, 5, 9]. Although they tend to have a benign history, lesions that bleed can lead to considerable morbidity with seizures and/or severe focal neurological deficits [1, 4, 9–15].…”

“…Cavernous malformations (CMs) of the central nervous system (CNS) [1][2][3] are known to be a major source of intracerebral haemorrhage (ICH), as well as intramedullary haemorrhage (IMH) [1,[4][5][6]. They have an estimated cumulative risk of ≈20% over a 5-year timeline for patients with cerebral cavernous malformations (CCMs) [1,2,7,8], as well as ≈40% for patients with cavernous malformations of the spinal cord (SCM) [4,5,9]. Although they tend to have a benign history, lesions that bleed can lead to considerable morbidity with seizures and/or severe focal neurological deficits [1,4,[9][10][11][12][13][14][15].…”

“…Although they tend to have a benign history, lesions that bleed can lead to considerable morbidity with seizures and/or severe focal neurological deficits [1,4,[9][10][11][12][13][14][15]. To this extent, a significant number of studies have assessed the natural course of the disease and risk factors that increase the bleeding risk (e.g., lesion localization or history of previous haemorrhage) [1,5,7,8,[16][17][18][19]. Such studies report a considerable increase of bleeding risk after an initial bleeding of up to ≈30% for CCM patients [1,2,7,8] and up to ≈55% for SCM patients [4,5].…”

“…To this extent, a significant number of studies have assessed the natural course of the disease and risk factors that increase the bleeding risk (e.g., lesion localization or history of previous haemorrhage) [1,5,7,8,[16][17][18][19]. Such studies report a considerable increase of bleeding risk after an initial bleeding of up to ≈30% for CCM patients [1,2,7,8] and up to ≈55% for SCM patients [4,5]. Unfortunately, such studies mainly assess the risk of initial and recurrent bleeding, leaving the risk of a third bleeding yet to be discovered [1,4,5,7,8,10,18,20].…”