Article
Prospective risk of hemorrhage in untreated single sporadic cerebral cavernous malformations (CCM) – A study of 198 symptomatic and incidental cases
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Authors
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Philipp Dammann
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
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Karsten Wrede
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
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Ramazan Jabbarli
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
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Marvin Oppong
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
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Oliver Müller
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
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Ulrich Sure
- Klinik für Neurochirurgie, Universitätsklinikum Essen, Essen, Germany
| Published: | June 8, 2016 |
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Outline
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Objective: The risk of (re-)hemorrhage in CCM is still being discussed controversially with annual (re-) hemorrhage rates ranging from 0.5% - 30%. Previous studies have often mixed symptomatic and incidental cases, different types of symptomatic cases (symptomatic hemorrhage vs. non-hemorrhage focal neurological deficit) as well as sporadic and familial disease. In this study we aimed to evaluate the annual hemorrhage risk as well as potential predictors in the clinically most relevant cohorts of CCM: symptomatic and incidental single sporadic CCM.
Method: A consecutive series of 198 symptomatic (n=134, mean follow-up 35.5 months) and incidental (n=64, mean follow-up 64.1 months) patients with untreated single sporadic CCMs was consecutively followed up in our department by clinical evaluation and MRI (minimum follow-up 12 months). We assessed the following events: symptomatic hemorrhage, hemorrhage on MRI and lesion size increase/decrease on MRI. Statistical analysis aimed for potential predictors of hemorrhage. Data was assessed according to the reporting standards of CCM.
Results: Annual hemorrhage rates were 9.6% (first three years after ictus) for the symptomatic cases and 0.9% (first five years after diagnosis) for the incidental cases. Among the symptomatic cases, the risk was 1.7% for patients with initial non-hemorrhage focal neurological deficit and 15.9% for patients after initial symptomatic hemorrhage (MRI = Zabramski Ia and Ib). Signs of hemorrhage (>0.5 cm) on MRI follow-up not causing symptoms were found in 23.8% of symptomatic cases and 7.8% of incidental cases. Increase in lesion size (>1/3 of initial size) was found in 17.9% of symptomatic cases and 9.3% of incidental cases. Predictors for hemorrhage in symptomatic cases were initial extra-lesional hemorrhage (p<0.005) and brainstem location (p<0.005).
Conclusions: Our results confirm that the re-hemorrhage risk after an initial symptomatic hemorrhage is relatively high, especially for patients with brainstem CCM and initial extra-lesional hemorrhage. Hemorrhage risk for incidental CCM on the other hand is relatively low. Our results also show that significant signs of bleeding and increase of size (without associated symptoms) are common findings in CCM. These findings will be helpful in the counselling of patients with symptomatic or incidental CCM.
