Coexistence of intracranial and spinal cavernous malformations: A study of prevalence and natural history

Aaron A. Cohen-Gadol, Jeffrey T. Jacob, Diane A. Edwards, William E. Krauss

Research output: Contribution to journalReview article

48 Citations (Scopus)

Abstract

Object. The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods. The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients' mean age at presentation was 50 years (range 13-82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions. As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

Original languageEnglish (US)
Pages (from-to)376-381
Number of pages6
JournalJournal of Neurosurgery
Volume104
Issue number3
DOIs
StatePublished - Mar 2006

Fingerprint

Natural History
Cross-Sectional Studies
Magnetic Resonance Imaging
Hemorrhage
Central Nervous System Cavernous Hemangioma
Neuroimaging

Keywords

  • Cavernous malformation
  • Central nervous system
  • Cerebral cavernous hemangioma
  • Hemorrhage
  • Spinal cavernous hemangioma

ASJC Scopus subject areas

  • Surgery
  • Medicine(all)
  • Clinical Neurology

Cite this

Coexistence of intracranial and spinal cavernous malformations : A study of prevalence and natural history. / Cohen-Gadol, Aaron A.; Jacob, Jeffrey T.; Edwards, Diane A.; Krauss, William E.

In: Journal of Neurosurgery, Vol. 104, No. 3, 03.2006, p. 376-381.

Research output: Contribution to journalReview article

Cohen-Gadol, Aaron A. ; Jacob, Jeffrey T. ; Edwards, Diane A. ; Krauss, William E. / Coexistence of intracranial and spinal cavernous malformations : A study of prevalence and natural history. In: Journal of Neurosurgery. 2006 ; Vol. 104, No. 3. pp. 376-381.
@article{09a94ccbe78e4efcb771790fdc135523,
title = "Coexistence of intracranial and spinal cavernous malformations: A study of prevalence and natural history",
abstract = "Object. The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods. The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients' mean age at presentation was 50 years (range 13-82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49{\%}) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42{\%}) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43{\%}) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6{\%} per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions. As many as 40{\%} of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40{\%} of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.",
keywords = "Cavernous malformation, Central nervous system, Cerebral cavernous hemangioma, Hemorrhage, Spinal cavernous hemangioma",
author = "Cohen-Gadol, {Aaron A.} and Jacob, {Jeffrey T.} and Edwards, {Diane A.} and Krauss, {William E.}",
year = "2006",
month = "3",
doi = "10.3171/jns.2006.104.3.376",
language = "English (US)",
volume = "104",
pages = "376--381",
journal = "Journal of Neurosurgery",
issn = "0022-3085",
publisher = "American Association of Neurological Surgeons",
number = "3",

}

TY - JOUR

T1 - Coexistence of intracranial and spinal cavernous malformations

T2 - A study of prevalence and natural history

AU - Cohen-Gadol, Aaron A.

AU - Jacob, Jeffrey T.

AU - Edwards, Diane A.

AU - Krauss, William E.

PY - 2006/3

Y1 - 2006/3

N2 - Object. The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods. The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients' mean age at presentation was 50 years (range 13-82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions. As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

AB - Object. The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods. The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients' mean age at presentation was 50 years (range 13-82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions. As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

KW - Cavernous malformation

KW - Central nervous system

KW - Cerebral cavernous hemangioma

KW - Hemorrhage

KW - Spinal cavernous hemangioma

UR - http://www.scopus.com/inward/record.url?scp=33644653761&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33644653761&partnerID=8YFLogxK

U2 - 10.3171/jns.2006.104.3.376

DO - 10.3171/jns.2006.104.3.376

M3 - Review article

C2 - 16572649

AN - SCOPUS:33644653761

VL - 104

SP - 376

EP - 381

JO - Journal of Neurosurgery

JF - Journal of Neurosurgery

SN - 0022-3085

IS - 3

ER -