Prospective Hemorrhage Rates of Cerebral Cavernous Malformations in Children and Adolescents Based on MRI Appearance.
OBJECTIVE
Current classifications of cerebral cavernous malformations focus solely on morphologic aspects. Our aim was to provide a morphologic classification that reflects hemorrhage rates.


METHODS
We retrospectively categorized 355 cavernous malformations of 70 children and adolescents according to their morphologic appearance on MR imaging and calculated prospective hemorrhage rates on the basis of survival functions for 255 lesions in 25 patients with a radiologic observation period of >180 days.


RESULTS
Overall, there were 199 MR imaging examinations with 1558 distinct cavernous malformation observations during a cumulative observation period of 1094.2 lesion-years. The mean hemorrhage rate of all 355 cavernous malformations was 4.5% per lesion-year. According to Kaplan-Meier survival models, Zabramski type I and II cavernous malformations had a significantly higher hemorrhage rate than type III and IV lesions. The presence of acute or subacute blood-degradation products was the strongest indicator for an increased hemorrhage risk (P = .036, Cox regression): The mean annual hemorrhage rate and mean hemorrhage-free interval for cavernous malformations with and without signs of acute or subacute blood degradation products were 23.4% and 22.6 months and 3.4% and 27.9 months, respectively. Dot-sized cavernous malformations, visible in T2* and not or barely visible in T1WI and T2WI sequences, had a mean annual hemorrhage rate of 1.3% and a mean hemorrhage-free interval of 37.8 months.


CONCLUSIONS
It is possible to predict hemorrhage rates based on the Zabramski classification. Our findings imply a tripartite classification distinguishing lesions with and without acute or subacute blood degradation products and dot-sized cavernous malformations.
From the Department of Neuroradiology (O.N., M.W.), University Hospital Aachen, Aachen, Germany Institute for Neuroscience and Medicine 4 (O.N.), Science Centre Jülich, Jülich, Germany.
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http://orcid.org/0000-0002-2055-4217
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Nikoubashman
Service de Neurochirurgie Pédiatrique (F.D.R., M.Z.), Hôpital Necker-Enfants Malades, Paris, France.
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Di Rocco
Department of Neuroradiology (I.D.), National Hospital for Neurology and Neurosurgery, London, UK Brain Repair & Rehabilitation Unit (I.D.), UCL Institute of Neurology, London, UK.
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Davagnanam
Department of Paediatric Neuroradiology (K.M.), Great Ormond Street Hospital, London, UK.
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Mankad
Service de Neurochirurgie Pédiatrique (F.D.R., M.Z.), Hôpital Necker-Enfants Malades, Paris, France.
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Zerah
From the Department of Neuroradiology (O.N., M.W.), University Hospital Aachen, Aachen, Germany mwiesmann@ukaachen.de.
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Wiesmann
D000293:Adolescent; D002543:Cerebral Hemorrhage / Q000453:epidemiology* / Q000209:etiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D020786:Hemangioma, Cavernous, Central Nervous System / Q000145:classification* / Q000150:complications* / Q000473:pathology; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000145:classification* / Q000150:complications* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies