Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage

Phillip J. Porter M.D.1, Robert A. Willinsky M.D., F.R.C.P.(C)1, William Harper M.D.1, and M. Christopher Wallace M.D., F.R.C.S.(C)1
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  • 1 The University of Toronto Brain Vascular Malformation Study Group, Toronto, Ontario, Canada
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✓ Despite recent studies of the natural history of cavernous malformations, there remains significant uncertainty concerning hemorrhage rates and the importance of lesion location. Controversy arises over varying definitions of “hemorrhage.” What is ultimately important to the patient is the occurrence of a neurological event, which may or may not be associated with radiologically documented hemorrhage, as well as the chance of recovery after such an event. The purpose of this study was to determine the rates of occurrence and sequelae of neurological events in 173 patients referred to our vascular malformation clinic with cavernous malformations.

All patient data were entered into a database. The mean age at presentation for the 173 patients was 37.5 years. The lesion location was deep (brainstem, cerebellar nuclei, thalamus, or basal ganglia) in 64 patients (37%) and superficial in 109 (63%). Thirty-one patients (18%) had multiple lesions. Disease presentation was due to seizures in 62 patients (36%), hemorrhage in 44 (25%), focal neurological deficit without documented hemorrhage in 35 (20%), headache alone in 11 (6%), and incidental findings in 21 patients (12%). The results obtained in the 110 patients eligible for follow-up review were used to derive information on the rates of hemorrhage and neurological events. An interval event (neurological deterioration) required both symptoms and signs. The total mean follow-up period was 46 months, the majority (65%) of which was prospective. There were 18 interval events in 427 patient-years of follow-up review, for an overall annual event rate of 4.2%. Location was the most important factor for predicting interval event occurrence, with significantly higher rates for deeply located (10.6%/year) compared with superficially located lesions (0%/year) (p = 0.0001). Of patients suffering a neurological event, only 37% had complete resolution of their deficits.

This largely prospective study indicates that deep cavernous malformations carry a worse prognosis than superficial lesions with respect to annual rates of neurological deterioration. The alarming rate of adverse clinical events occurring in patients with deep lesions is punctuated by the fact that less than one-half of them recover fully during long-term follow-up review.

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Contributor Notes

Address reprint requests to: M. Christopher Wallace, M.D., F.R.C.S.(C), The Toronto Hospital, Western Division, Suite 2–427, McLaughlin Pavilion, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2S8. email: p.porter@utoronto.ca.
  • 1.

    Aiba T, , Tanaka R, & Koike T, et al. : Natural history of intracranial cavernous malformations. J Neurosurg 83:5659, 1995 Aiba T, Tanaka R, Koike T, et al: Natural history of intracranial cavernous malformations. J Neurosurg 83:56–59, 1995

    • Search Google Scholar
    • Export Citation
  • 2.

    Barrow DL, & Krisht A: Cavernous malformations and hemorrhage, in Awad IA, & Barrow DL (eds): Cavernous Malformations. Park Ridge, Ill: American Association of Neurological Surgeons, 1993, pp 6580 Barrow DL, Krisht A: Cavernous malformations and hemorrhage, in Awad IA, Barrow DL (eds): Cavernous Malformations. Park Ridge, Ill: American Association of Neurological Surgeons, 1993, pp 65–80

    • Search Google Scholar
    • Export Citation
  • 3.

    Curling OD Jr, , Kelly DL Jr, & Elster AD, et al. : An analysis of the natural history of cavernous angiomas. J Neurosurg 75:702708, 1991 Curling OD Jr, Kelly DL Jr, Elster AD, et al: An analysis of the natural history of cavernous angiomas. J Neurosurg 75:702–708, 1991

    • Search Google Scholar
    • Export Citation
  • 4.

    Fritschi JA, , Reulen HJ, & Spetzler RF, et al. : Cavernous malformations of the brain stem. A review of 139 cases. Acta Neurochir 130:3546, 1994 Fritschi JA, Reulen HJ, Spetzler RF, et al: Cavernous malformations of the brain stem. A review of 139 cases. Acta Neurochir 130:35–46, 1994

    • Search Google Scholar
    • Export Citation
  • 5.

    Giombini S, & Morello G: Cavernous angiomas of the brain. Account of fourteen personal cases and review of the literature. Acta Neurochir 40:6182, 1978 Giombini S, Morello G: Cavernous angiomas of the brain. Account of fourteen personal cases and review of the literature. Acta Neurochir 40:61–82, 1978

    • Search Google Scholar
    • Export Citation
  • 6.

    Horowitz M, & Kondziolka D: Multiple familial cavernous malformations evaluated over three generations with MR. AJNR 16:13531355, 1995 Horowitz M, Kondziolka D: Multiple familial cavernous malformations evaluated over three generations with MR. AJNR 16:1353–1355, 1995

    • Search Google Scholar
    • Export Citation
  • 7.

    Kondziolka D, , Lunsford LD, & Flickinger JC, et al. : Reduction of hemorrhage risk after stereotactic radiosurgery for cavernous malformations. J Neurosurg 83:825831, 1995 Kondziolka D, Lunsford LD, Flickinger JC, et al: Reduction of hemorrhage risk after stereotactic radiosurgery for cavernous malformations. J Neurosurg 83:825–831, 1995

    • Search Google Scholar
    • Export Citation
  • 8.

    Kondziolka D, , Lunsford LD, & Kestle JRW: The natural history of cerebral cavernous malformations. J Neurosurg 83:820824, 1995 Kondziolka D, Lunsford LD, Kestle JRW: The natural history of cerebral cavernous malformations. J Neurosurg 83:820–824, 1995

    • Search Google Scholar
    • Export Citation
  • 9.

    Lemme-Pleghos L, , Kucharczyk W, & Brant-Zawadzki M, et al. : MRI of angiographically occult vascular malformations. AJNR 6:863870, 1985 Lemme-Pleghos L, Kucharczyk W, Brant-Zawadzki M, et al: MRI of angiographically occult vascular malformations. AJNR 6:863–870, 1985

    • Search Google Scholar
    • Export Citation
  • 10.

    Maraire JN, & Awad IA: Intracranial cavernous malformations: lesion behavior and management strategies. Neurosurgery 37:591605, 1995 Maraire JN, Awad IA: Intracranial cavernous malformations: lesion behavior and management strategies. Neurosurgery 37:591–605, 1995

    • Search Google Scholar
    • Export Citation
  • 11.

    Pozzati E, , Acciarri N, & Tognetti F, et al. : Growth, subsequent bleeding, and de novo appearance of cerebral cavernous malformations. Neurosurgery 38:662670, 1996 Pozzati E, Acciarri N, Tognetti F, et al: Growth, subsequent bleeding, and de novo appearance of cerebral cavernous malformations. Neurosurgery 38:662–670, 1996

    • Search Google Scholar
    • Export Citation
  • 12.

    Pozzati E, , Giangaspero F, & Marliani F, et al. : Occult cerebrovascular malformations after irradiation. Neurosurgery 39:677684, 1996 Pozzati E, Giangaspero F, Marliani F, et al: Occult cerebrovascular malformations after irradiation. Neurosurgery 39:677–684, 1996

    • Search Google Scholar
    • Export Citation
  • 13.

    Rigamonti D, , Drayer BP, & Johnson PC, et al. : The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67:518524, 1987 Rigamonti D, Drayer BP, Johnson PC, et al: The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67:518–524, 1987

    • Search Google Scholar
    • Export Citation
  • 14.

    Robinson JR, , Awad IA, & Little JR: Natural history of the cavernous angioma. J Neurosurg 75:709714, 1991 Robinson JR, Awad IA, Little JR: Natural history of the cavernous angioma. J Neurosurg 75:709–714, 1991

    • Search Google Scholar
    • Export Citation
  • 15.

    Robinson JR, , Awad IA, & Magdinec M, et al. : Factors predisposing to clinical disability in patients with cavernous malformations of the brain. Neurosurgery 32:730736, 1993 Robinson JR, Awad IA, Magdinec M, et al: Factors predisposing to clinical disability in patients with cavernous malformations of the brain. Neurosurgery 32:730–736, 1993

    • Search Google Scholar
    • Export Citation
  • 16.

    Steiger HJ, & Tew JM Jr: Hemorrhage and epilepsy in cryptic cerebrovascular malformations. Arch Neurol 41:722724, 1984 Steiger HJ, Tew JM Jr: Hemorrhage and epilepsy in cryptic cerebrovascular malformations. Arch Neurol 41:722–724, 1984

    • Search Google Scholar
    • Export Citation
  • 17.

    Tomlinson FH, , Houser OW, & Scheithauer BW, et al. : Angiographically occult vascular malformations: a correlative study of features on magnetic resonance imaging and histological examination. Neurosurgery 34:792800, 1994 Tomlinson FH, Houser OW, Scheithauer BW, et al: Angiographically occult vascular malformations: a correlative study of features on magnetic resonance imaging and histological examination. Neurosurgery 34:792–800, 1994

    • Search Google Scholar
    • Export Citation
  • 18.

    Tung H, , Giannotta SL, & Chandrasoma PT, et al. : Recurrent intraparenchymal hemorrhages from angiographically occult vascular malformations. J Neurosurg 73:174180, 1990 Tung H, Giannotta SL, Chandrasoma PT, et al: Recurrent intraparenchymal hemorrhages from angiographically occult vascular malformations. J Neurosurg 73:174–180, 1990

    • Search Google Scholar
    • Export Citation
  • 19.

    Willinsky R, , Harper W, & Wallace MC, et al. : Follow-up MR of intracranial cavernomas. The relationship between haemorrhagic events and morphology. Intervent Neuroradiol 2:127135, 1996 Willinsky R, Harper W, Wallace MC, et al: Follow-up MR of intracranial cavernomas. The relationship between haemorrhagic events and morphology. Intervent Neuroradiol 2:127–135, 1996

    • Search Google Scholar
    • Export Citation
  • 20.

    Zabramski JM, , Wascher TM, & Spetzler RF, et al. : The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 80:422432, 1994 Zabramski JM, Wascher TM, Spetzler RF, et al: The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 80:422–432, 1994

    • Search Google Scholar
    • Export Citation
  • 21.

    Zimmerman RS, , Spetzler RF, & Lee KS, et al. : Cavernous malformations of the brain stem. J Neurosurg 75:3239, 1991 Zimmerman RS, Spetzler RF, Lee KS, et al: Cavernous malformations of the brain stem. J Neurosurg 75:32–39, 1991

    • Search Google Scholar
    • Export Citation

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