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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1999 May;66(5):561–568. doi: 10.1136/jnnp.66.5.561

Supratentorial cavernous haemangiomas and epilepsy: a review of the literature and case series

N Moran 1, D Fish 1, N Kitchen 1, S Shorvon 1, B Kendall 1, J Stevens 1
PMCID: PMC1736368  PMID: 10209164

Abstract

OBJECTIVES—To characterise the clinical features and response to treatment of supratentorial cavernomas associated with epilepsy.
METHODS—A systematic review of the literature was carried out and a retrospective case series of patients with cavernoma diagnosed by MRI and/or histology was compiled. Patient selection biases in the literature review were reduced as far as possible by selection of unbiased publications.
RESULTS—In the literature, cavernomas were relatively less common in the frontal lobes. There were multiple cavernomas in 23% of cases. The main clinical manifestations were seizures (79%) and haemorrhage (16%). The annual haemorrhage rate was 0.7%. The outcome after excision was good with improvement in seizures in 92% of patients. In the case series the surgical outcome was less favourable, reflecting inclusion of a higher proportion of patients with intractable epilepsy. In both the literature review and the case series, outcome was poorer in cases with a longer duration of seizures at the time of surgery.
CONCLUSIONS—The good surgical results, particularly in cases treated earlier, and the significant cumulative haemorrhage rate, suggest that excision is the optimum treatment. However, these factors have not been examined prospectively and, despite the availability of several retrospective studies, the optimum treatment, particularly for non-intractable cases, will only be determined by a prospective study.



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Selected References

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  1. Acciarri N., Padovani R., Giulioni M., Gaist G., Acciarri R. Intracranial and orbital cavernous angiomas: a review of 74 surgical cases. Br J Neurosurg. 1993;7(5):529–539. doi: 10.3109/02688699308995075. [DOI] [PubMed] [Google Scholar]
  2. Aiba T., Tanaka R., Koike T., Kameyama S., Takeda N., Komata T. Natural history of intracranial cavernous malformations. J Neurosurg. 1995 Jul;83(1):56–59. doi: 10.3171/jns.1995.83.1.0056. [DOI] [PubMed] [Google Scholar]
  3. Bartlett J. E., Kishore P. R. Intracranial cavernous angioma. AJR Am J Roentgenol. 1977 Apr;128(4):653–656. doi: 10.2214/ajr.128.4.653. [DOI] [PubMed] [Google Scholar]
  4. Becker D. H., Townsend J. J., Kramer R. A., Newton T. H. Occult cerebrovascular malformations. A series of 18 histologically verified cases with negative angiography. Brain. 1979 Jun;102(2):249–287. doi: 10.1093/brain/102.2.249. [DOI] [PubMed] [Google Scholar]
  5. Bertalanffy H., Kühn G., Scheremet R., Seeger W. Indications for surgery and prognosis in patients with cerebral cavernous angiomas. Neurol Med Chir (Tokyo) 1992 Aug;32(9):659–666. doi: 10.2176/nmc.32.659. [DOI] [PubMed] [Google Scholar]
  6. Bicknell J. M., Carlow T. J., Kornfeld M., Stovring J., Turner P. Familial cavernous angiomas. Arch Neurol. 1978 Nov;35(11):746–749. doi: 10.1001/archneur.1978.00500350050010. [DOI] [PubMed] [Google Scholar]
  7. Boecher-Schwarz H. G., Grunert P., Guenthner M., Kessel G., Mueller-Forell W. Stereotactically guided cavernous malformation surgery. Minim Invasive Neurosurg. 1996 Jun;39(2):50–55. doi: 10.1055/s-2008-1052216. [DOI] [PubMed] [Google Scholar]
  8. Buckingham M. J., Crone K. R., Ball W. S., Berger T. S. Management of cerebral cavernous angiomas in children presenting with seizures. Childs Nerv Syst. 1989 Dec;5(6):347–349. doi: 10.1007/BF00271890. [DOI] [PubMed] [Google Scholar]
  9. Cappabianca P., Alfieri A., Maiuri F., Mariniello G., Cirillo S., de Divitiis E. Supratentorial cavernous malformations and epilepsy: seizure outcome after lesionectomy on a series of 35 patients. Clin Neurol Neurosurg. 1997 Aug;99(3):179–183. doi: 10.1016/s0303-8467(97)00023-1. [DOI] [PubMed] [Google Scholar]
  10. Casazza M., Broggi G., Franzini A., Avanzini G., Spreafico R., Bracchi M., Valentini M. C. Supratentorial cavernous angiomas and epileptic seizures: preoperative course and postoperative outcome. Neurosurgery. 1996 Jul;39(1):26–34. doi: 10.1097/00006123-199607000-00007. [DOI] [PubMed] [Google Scholar]
  11. Cascino G. D., Jack C. R., Jr, Parisi J. E., Sharbrough F. W., Schreiber C. P., Kelly P. J., Trenerry M. R. Operative strategy in patients with MRI-identified dual pathology and temporal lobe epilepsy. Epilepsy Res. 1993 Feb;14(2):175–182. doi: 10.1016/0920-1211(93)90022-y. [DOI] [PubMed] [Google Scholar]
  12. Churchyard A., Khangure M., Grainger K. Cerebral cavernous angioma: a potentially benign condition? Successful treatment in 16 cases. J Neurol Neurosurg Psychiatry. 1992 Nov;55(11):1040–1045. doi: 10.1136/jnnp.55.11.1040. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Cohen D. S., Zubay G. P., Goodman R. R. Seizure outcome after lesionectomy for cavernous malformations. J Neurosurg. 1995 Aug;83(2):237–242. doi: 10.3171/jns.1995.83.2.0237. [DOI] [PubMed] [Google Scholar]
  14. Del Curling O., Jr, Kelly D. L., Jr, Elster A. D., Craven T. E. An analysis of the natural history of cavernous angiomas. J Neurosurg. 1991 Nov;75(5):702–708. doi: 10.3171/jns.1991.75.5.0702. [DOI] [PubMed] [Google Scholar]
  15. Di Rocco C., Iannelli A., Tamburrini G. Cavernomas of the central nervous system in children. A report of 22 cases. Acta Neurochir (Wien) 1996;138(11):1267–1274. doi: 10.1007/BF01411054. [DOI] [PubMed] [Google Scholar]
  16. Dubovsky J., Zabramski J. M., Kurth J., Spetzler R. F., Rich S. S., Orr H. T., Weber J. L. A gene responsible for cavernous malformations of the brain maps to chromosome 7q. Hum Mol Genet. 1995 Mar;4(3):453–458. doi: 10.1093/hmg/4.3.453. [DOI] [PubMed] [Google Scholar]
  17. Farmer J. P., Cosgrove G. R., Villemure J. G., Meagher-Villemure K., Tampieri D., Melanson D. Intracerebral cavernous angiomas. Neurology. 1988 Nov;38(11):1699–1704. doi: 10.1212/wnl.38.11.1699. [DOI] [PubMed] [Google Scholar]
  18. Ferrante L., Palma L., d'Addetta R., Mastronardi L., Acqui M., Fortuna A. Intracranial cavernous angioma. Neurosurg Rev. 1992;15(2):125–133. doi: 10.1007/BF00313508. [DOI] [PubMed] [Google Scholar]
  19. Frima-Verhoeven P. A., Op de Coul A. A., Tijssen C. C., Maat B. Intracranial cavernous angiomas: diagnosis and therapy. Eur Neurol. 1989;29(1):56–60. doi: 10.1159/000116378. [DOI] [PubMed] [Google Scholar]
  20. Gewirtz R. J., Steinberg G. K., Crowley R., Levy R. P. Pathological changes in surgically resected angiographically occult vascular malformations after radiation. Neurosurgery. 1998 Apr;42(4):738–743. doi: 10.1097/00006123-199804000-00031. [DOI] [PubMed] [Google Scholar]
  21. Gil-Nagel A., Dubovsky J., Wilcox K. J., Stewart J. M., Anderson V. E., Leppik I. E., Orr H. T., Johnson E. W., Weber J. L., Rich S. S. Familial cerebral cavernous angioma: a gene localized to a 15-cM interval on chromosome 7q. Ann Neurol. 1996 Jun;39(6):807–810. doi: 10.1002/ana.410390619. [DOI] [PubMed] [Google Scholar]
  22. Giombini S., Morello G. Cavernous angiomas of the brain. Account of fourteen personal cases and review of the literature. Acta Neurochir (Wien) 1978;40(1-2):61–82. doi: 10.1007/BF01773116. [DOI] [PubMed] [Google Scholar]
  23. Gomori J. M., Grossman R. I., Goldberg H. I., Hackney D. B., Zimmerman R. A., Bilaniuk L. T. Occult cerebral vascular malformations: high-field MR imaging. Radiology. 1986 Mar;158(3):707–713. doi: 10.1148/radiology.158.3.3945744. [DOI] [PubMed] [Google Scholar]
  24. Günel M., Awad I. A., Anson J., Lifton R. P. Mapping a gene causing cerebral cavernous malformation to 7q11.2-q21. Proc Natl Acad Sci U S A. 1995 Jul 3;92(14):6620–6624. doi: 10.1073/pnas.92.14.6620. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Hayman L. A., Evans R. A., Ferrell R. E., Fahr L. M., Ostrow P., Riccardi V. M. Familial cavernous angiomas: natural history and genetic study over a 5-year period. Am J Med Genet. 1982 Feb;11(2):147–160. doi: 10.1002/ajmg.1320110205. [DOI] [PubMed] [Google Scholar]
  26. Herter T., Brandt M., Szüwart U. Cavernous hemangiomas in children. Childs Nerv Syst. 1988 Jun;4(3):123–127. doi: 10.1007/BF00270900. [DOI] [PubMed] [Google Scholar]
  27. Hoeldtke N. J., Floyd D., Werschkul J. D., Calhoun B. C., Hume R. F. Intracranial cavernous angioma initially presenting in pregnancy with new-onset seizures. Am J Obstet Gynecol. 1998 Mar;178(3):612–613. doi: 10.1016/s0002-9378(98)70449-1. [DOI] [PubMed] [Google Scholar]
  28. Johnson E. W., Iyer L. M., Rich S. S., Orr H. T., Gil-Nagel A., Kurth J. H., Zabramski J. M., Marchuk D. A., Weissenbach J., Clericuzio C. L. Refined localization of the cerebral cavernous malformation gene (CCM1) to a 4-cM interval of chromosome 7q contained in a well-defined YAC contig. Genome Res. 1995 Nov;5(4):368–380. doi: 10.1101/gr.5.4.368. [DOI] [PubMed] [Google Scholar]
  29. Karlsson B., Kihlström L., Lindquist C., Ericson K., Steiner L. Radiosurgery for cavernous malformations. J Neurosurg. 1998 Feb;88(2):293–297. doi: 10.3171/jns.1998.88.2.0293. [DOI] [PubMed] [Google Scholar]
  30. Kattapong V. J., Hart B. L., Davis L. E. Familial cerebral cavernous angiomas: clinical and radiologic studies. Neurology. 1995 Mar;45(3 Pt 1):492–497. doi: 10.1212/wnl.45.3.492. [DOI] [PubMed] [Google Scholar]
  31. Kida Y., Kobayashi T., Tanaka T. Treatment of symptomatic AOVMs with radiosurgery. Acta Neurochir Suppl. 1995;63:68–72. doi: 10.1007/978-3-7091-9399-0_14. [DOI] [PubMed] [Google Scholar]
  32. Kitamura K., Fukui M., Oka K., Matsushima T., Hasuo K., Fukushima T., Tomonaga M., Okudera T. Hemangiomas of the central nervous system in Japan: an epidemiological and clinicopathological study with special reference to venous and cavernous malformations. Neurosurg Rev. 1986;9(3):221–231. doi: 10.1007/BF01743137. [DOI] [PubMed] [Google Scholar]
  33. Kondziolka D., Lunsford L. D., Flickinger J. C., Kestle J. R. Reduction of hemorrhage risk after stereotactic radiosurgery for cavernous malformations. J Neurosurg. 1995 Nov;83(5):825–831. doi: 10.3171/jns.1995.83.5.0825. [DOI] [PubMed] [Google Scholar]
  34. Kondziolka D., Lunsford L. D., Kestle J. R. The natural history of cerebral cavernous malformations. J Neurosurg. 1995 Nov;83(5):820–824. doi: 10.3171/jns.1995.83.5.0820. [DOI] [PubMed] [Google Scholar]
  35. Lunardi P., Acqui M. The echo-guided removal of cerebral cavernous angiomas. Acta Neurochir (Wien) 1993;123(3-4):113–117. doi: 10.1007/BF01401865. [DOI] [PubMed] [Google Scholar]
  36. Marchuk D. A., Gallione C. J., Morrison L. A., Clericuzio C. L., Hart B. L., Kosofsky B. E., Louis D. N., Gusella J. F., Davis L. E., Prenger V. L. A locus for cerebral cavernous malformations maps to chromosome 7q in two families. Genomics. 1995 Jul 20;28(2):311–314. doi: 10.1006/geno.1995.1147. [DOI] [PubMed] [Google Scholar]
  37. Mason I., Aase J. M., Orrison W. W., Wicks J. D., Seigel R. S., Bicknell J. M. Familial cavernous angiomas of the brain in an Hispanic family. Neurology. 1988 Feb;38(2):324–326. doi: 10.1212/wnl.38.2.324. [DOI] [PubMed] [Google Scholar]
  38. Matz P., McDermott M., Gutin P., Dillon W., Wilson C. Cavernous malformations: results of image-guided resection. J Image Guid Surg. 1995;1(5):273–279. doi: 10.1002/(SICI)1522-712X(1995)1:5<273::AID-IGS3>3.0.CO;2-7. [DOI] [PubMed] [Google Scholar]
  39. McConnell T. H., Leonard J. S. Microangiomatous malformations with intraventricular hemorrhage. Report of two unusual cases. Neurology. 1967 Jun;17(6):618–620. doi: 10.1212/wnl.17.6.618. [DOI] [PubMed] [Google Scholar]
  40. McCormick W. F. The pathology of vascular ("arteriovenous") malformations. J Neurosurg. 1966 Apr;24(4):807–816. doi: 10.3171/jns.1966.24.4.0807. [DOI] [PubMed] [Google Scholar]
  41. Moritake K., Handa H., Nozaki K., Tomiwa K. Tentorial cavernous angioma with calcification in a neonate. Neurosurgery. 1985 Feb;16(2):207–211. doi: 10.1227/00006123-198502000-00013. [DOI] [PubMed] [Google Scholar]
  42. Morrell F. Secondary epileptogenesis in man. Arch Neurol. 1985 Apr;42(4):318–335. doi: 10.1001/archneur.1985.04060040028009. [DOI] [PubMed] [Google Scholar]
  43. Ondra S. L., Doty J. R., Mahla M. E., George E. D. Surgical excision of a cavernous hemangioma of the rostral brain stem: case report. Neurosurgery. 1988 Oct;23(4):490–493. doi: 10.1227/00006123-198810000-00015. [DOI] [PubMed] [Google Scholar]
  44. Otten P., Pizzolato G. P., Rilliet B., Berney J. A propos de 131 cas d'angiomes caverneux (cavernomes) du s.n.c., repérés par l'analyse rétrospective de 24 535 autopsies. Neurochirurgie. 1989;35(2):82-3, 128-31. [PubMed] [Google Scholar]
  45. Perlemuter G., Béjanin H., Fritsch J., Prat F., Gaudric M., Chaussade S., Buffet C. Biliary obstruction caused by portal cavernoma: a study of 8 cases. J Hepatol. 1996 Jul;25(1):58–63. doi: 10.1016/s0168-8278(96)80328-x. [DOI] [PubMed] [Google Scholar]
  46. Porter P. J., Willinsky R. A., Harper W., Wallace M. C. Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage. J Neurosurg. 1997 Aug;87(2):190–197. doi: 10.3171/jns.1997.87.2.0190. [DOI] [PubMed] [Google Scholar]
  47. Pozzati E., Giuliani G., Nuzzo G., Poppi M. The growth of cerebral cavernous angiomas. Neurosurgery. 1989 Jul;25(1):92–97. doi: 10.1097/00006123-198907000-00017. [DOI] [PubMed] [Google Scholar]
  48. Pozzati E., Padovani R., Morrone B., Finizio F., Gaist G. Cerebral cavernous angiomas in children. J Neurosurg. 1980 Dec;53(6):826–832. doi: 10.3171/jns.1980.53.6.0826. [DOI] [PubMed] [Google Scholar]
  49. Requena I., Arias M., López-Ibor L., Pereiro I., Barba A., Alonso A., Montón E. Cavernomas of the central nervous system: clinical and neuroimaging manifestations in 47 patients. J Neurol Neurosurg Psychiatry. 1991 Jul;54(7):590–594. doi: 10.1136/jnnp.54.7.590. [DOI] [PMC free article] [PubMed] [Google Scholar]
  50. Rigamonti D., Drayer B. P., Johnson P. C., Hadley M. N., Zabramski J., Spetzler R. F. The MRI appearance of cavernous malformations (angiomas). J Neurosurg. 1987 Oct;67(4):518–524. doi: 10.3171/jns.1987.67.4.0518. [DOI] [PubMed] [Google Scholar]
  51. Rigamonti D., Hadley M. N., Drayer B. P., Johnson P. C., Hoenig-Rigamonti K., Knight J. T., Spetzler R. F. Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med. 1988 Aug 11;319(6):343–347. doi: 10.1056/NEJM198808113190605. [DOI] [PubMed] [Google Scholar]
  52. Robinson J. R., Awad I. A., Little J. R. Natural history of the cavernous angioma. J Neurosurg. 1991 Nov;75(5):709–714. doi: 10.3171/jns.1991.75.5.0709. [DOI] [PubMed] [Google Scholar]
  53. Ryvlin P., Mauguière F., Sindou M., Froment J. C., Cinotti L. Interictal cerebral metabolism and epilepsy in cavernous angiomas. Brain. 1995 Jun;118(Pt 3):677–687. doi: 10.1093/brain/118.3.677. [DOI] [PubMed] [Google Scholar]
  54. Sabatier J., Gigaud M., Dubois G., Tremoulet M. Cavernome de l'enfant. A propos d'une forme néo-natale avec récidive dans l'enfance. Neurochirurgie. 1989;35(2):109–110. [PubMed] [Google Scholar]
  55. Sage M. R., Brophy B. P., Sweeney C., Phipps S., Perrett L. V., Sandhu A., Albertyn L. E. Cavernous haemangiomas (angiomas) of the brain: clinically significant lesions. Australas Radiol. 1993 May;37(2):147–155. doi: 10.1111/j.1440-1673.1993.tb00039.x. [DOI] [PubMed] [Google Scholar]
  56. Scott R. M., Barnes P., Kupsky W., Adelman L. S. Cavernous angiomas of the central nervous system in children. J Neurosurg. 1992 Jan;76(1):38–46. doi: 10.3171/jns.1992.76.1.0038. [DOI] [PubMed] [Google Scholar]
  57. Seo Y., Fukuoka S., Takanashi M., Nakagawara J., Suematsu K., Nakamura J., Nagashima K. Gamma Knife surgery for angiographically occult vascular malformations. Stereotact Funct Neurosurg. 1995;64 (Suppl 1):98–109. doi: 10.1159/000098769. [DOI] [PubMed] [Google Scholar]
  58. Simard J. M., Garcia-Bengochea F., Ballinger W. E., Jr, Mickle J. P., Quisling R. G. Cavernous angioma: a review of 126 collected and 12 new clinical cases. Neurosurgery. 1986 Feb;18(2):162–172. doi: 10.1227/00006123-198602000-00008. [DOI] [PubMed] [Google Scholar]
  59. Stea R. A., Schicker L., King G. A., Winfield J. A. Stereotactic linear radiosurgery for cavernous angiomas. Stereotact Funct Neurosurg. 1994;63(1-4):255–265. doi: 10.1159/000100323. [DOI] [PubMed] [Google Scholar]
  60. Steiger H. J., Markwalder T. M., Reulen H. J. Clinicopathological relations of cerebral cavernous angiomas: observations in eleven cases. Neurosurgery. 1987 Dec;21(6):879–884. doi: 10.1227/00006123-198712000-00016. [DOI] [PubMed] [Google Scholar]
  61. Symon L., Jackowski A., Bills D. Surgical treatment of pontomedullary cavernomas. Br J Neurosurg. 1991;5(4):339–347. doi: 10.3109/02688699109002860. [DOI] [PubMed] [Google Scholar]
  62. Tagle P., Huete I., Méndez J., del Villar S. Intracranial cavernous angioma: presentation and management. J Neurosurg. 1986 May;64(5):720–723. doi: 10.3171/jns.1986.64.5.0720. [DOI] [PubMed] [Google Scholar]
  63. Tomlinson F. H., Houser O. W., Scheithauer B. W., Sundt T. M., Jr, Okazaki H., Parisi J. E. Angiographically occult vascular malformations: a correlative study of features on magnetic resonance imaging and histological examination. Neurosurgery. 1994 May;34(5):792–800. doi: 10.1227/00006123-199405000-00002. [DOI] [PubMed] [Google Scholar]
  64. Vaquero J., Salazar J., Martínez R., Martínez P., Bravo G. Cavernomas of the central nervous system: clinical syndromes, CT scan diagnosis, and prognosis after surgical treatment in 25 cases. Acta Neurochir (Wien) 1987;85(1-2):29–33. doi: 10.1007/BF01402366. [DOI] [PubMed] [Google Scholar]
  65. Villani R. M., Arienta C., Caroli M. Cavernous angiomas of the central nervous system. J Neurosurg Sci. 1989 Jul-Sep;33(3):229–252. [PubMed] [Google Scholar]
  66. Yamasaki T., Handa H., Yamashita J., Paine J. T., Tashiro Y., Uno A., Ishikawa M., Asato R. Intracranial and orbital cavernous angiomas. A review of 30 cases. J Neurosurg. 1986 Feb;64(2):197–208. doi: 10.3171/jns.1986.64.2.0197. [DOI] [PubMed] [Google Scholar]
  67. Yeh H. S., Privitera M. D. Secondary epileptogenesis in cerebral arteriovenous malformations. Arch Neurol. 1991 Nov;48(11):1122–1124. doi: 10.1001/archneur.1991.00530230030016. [DOI] [PubMed] [Google Scholar]
  68. Zabramski J. M., Wascher T. M., Spetzler R. F., Johnson B., Golfinos J., Drayer B. P., Brown B., Rigamonti D., Brown G. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg. 1994 Mar;80(3):422–432. doi: 10.3171/jns.1994.80.3.0422. [DOI] [PubMed] [Google Scholar]
  69. Zevgaridis D., van Velthoven V., Ebeling U., Reulen H. J. Seizure control following surgery in supratentorial cavernous malformations: a retrospective study in 77 patients. Acta Neurochir (Wien) 1996;138(6):672–677. doi: 10.1007/BF01411470. [DOI] [PubMed] [Google Scholar]

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