Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia

A systematic review and meta-analysis

Waleed Brinjikji, Vivek N. Iyer, Christopher P. Wood, Giuseppe Lanzino

Research output: Contribution to journalReview article

12 Citations (Scopus)

Abstract

OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. METHODS To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990-March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model. RESULTS Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%-13.0%) with no significant difference between males (8.5%, 95% CI 4.9%-12.0%) and females (11.0%, 95% CI 5.9%-16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%-17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%-3.8%) (p < 0.0001). In 55.2% (95% CI 38.3%-72.1%) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9% (95% CI 67.5%-95.2%) of patients. CONCLUSIONS The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.

Original languageEnglish (US)
Pages (from-to)302-310
Number of pages9
JournalJournal of Neurosurgery
Volume127
Issue number2
DOIs
StatePublished - Aug 1 2017

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Arteriovenous Malformations
Meta-Analysis
Brain
Intracranial Arteriovenous Malformations
Population
Librarians
Arteriovenous Fistula

Keywords

  • Arteriovenous malformation
  • Hereditary hemorrhagic telangiectasia
  • Stroke
  • Vascular disorders

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia : A systematic review and meta-analysis. / Brinjikji, Waleed; Iyer, Vivek N.; Wood, Christopher P.; Lanzino, Giuseppe.

In: Journal of Neurosurgery, Vol. 127, No. 2, 01.08.2017, p. 302-310.

Research output: Contribution to journalReview article

@article{2e721ebff9224609a7821e69e4ae8f08,
title = "Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia: A systematic review and meta-analysis",
abstract = "OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. METHODS To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990-March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model. RESULTS Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4{\%} (95{\%} CI 7.9{\%}-13.0{\%}) with no significant difference between males (8.5{\%}, 95{\%} CI 4.9{\%}-12.0{\%}) and females (11.0{\%}, 95{\%} CI 5.9{\%}-16.1{\%}). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4{\%}, 95{\%} CI 9.5{\%}-17.4{\%}) compared with those with HHT Type 2 (HHT2) (2.4{\%}, 95{\%} CI 1.0{\%}-3.8{\%}) (p < 0.0001). In 55.2{\%} (95{\%} CI 38.3{\%}-72.1{\%}) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9{\%} (95{\%} CI 67.5{\%}-95.2{\%}) of patients. CONCLUSIONS The prevalence of brain AVMs in the HHT population is about 10{\%}. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90{\%} of patients.",
keywords = "Arteriovenous malformation, Hereditary hemorrhagic telangiectasia, Stroke, Vascular disorders",
author = "Waleed Brinjikji and Iyer, {Vivek N.} and Wood, {Christopher P.} and Giuseppe Lanzino",
year = "2017",
month = "8",
day = "1",
doi = "10.3171/2016.7.JNS16847",
language = "English (US)",
volume = "127",
pages = "302--310",
journal = "Journal of Neurosurgery",
issn = "0022-3085",
publisher = "American Association of Neurological Surgeons",
number = "2",

}

TY - JOUR

T1 - Prevalence and characteristics of brain arteriovenous malformations in hereditary hemorrhagic telangiectasia

T2 - A systematic review and meta-analysis

AU - Brinjikji, Waleed

AU - Iyer, Vivek N.

AU - Wood, Christopher P.

AU - Lanzino, Giuseppe

PY - 2017/8/1

Y1 - 2017/8/1

N2 - OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. METHODS To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990-March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model. RESULTS Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%-13.0%) with no significant difference between males (8.5%, 95% CI 4.9%-12.0%) and females (11.0%, 95% CI 5.9%-16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%-17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%-3.8%) (p < 0.0001). In 55.2% (95% CI 38.3%-72.1%) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9% (95% CI 67.5%-95.2%) of patients. CONCLUSIONS The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.

AB - OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are known to suffer from high rates of cerebral arteriovenous malformations (AVMs). The authors performed a systematic review and meta-analysis of the literature examining prevalence rates, characteristics, and clinical presentation of cerebral AVMs in the HHT population. METHODS To identify studies on AVM prevalence and characteristics in the HHT population, 4 databases (MEDLINE, EMBASE, Scopus and Web of Science) were searched by a reference librarian with over 30 years experience in systematic reviews and meta-analysis. The search period was January 1, 1990-March 2016. The following search terms were used: hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, AVM, brain AVM, arteriovenous malformation, arteriovenous fistula, prevalence, and epidemiology. The authors identified studies that examined the prevalence rates, characteristics, and clinical presentation of cerebral AVMs in patients with HHT. They assessed overall AVM prevalence rates as well as prevalence rates by age, sex, HHT type, and country/region. They also systematically reviewed the characteristics of AVMs, including rupture status, location, clinical presentation, angioarchitecture, and Spetzler-Martin grade. Data were analyzed using a random-effects meta-analysis model. RESULTS Thirty-nine studies were included in this meta-analysis. Thirty studies examined brain AVM prevalence rates in various HHT patient populations, and 18 studies examined AVM clinical and angiographic characteristics (9 studies examined both prevalence rates and AVM characteristics). The prevalence of brain AVMs in HHT patients was 10.4% (95% CI 7.9%-13.0%) with no significant difference between males (8.5%, 95% CI 4.9%-12.0%) and females (11.0%, 95% CI 5.9%-16.1%). Patients with HHT Type 1 (HHT1) had a significantly higher brain AVM prevalence (13.4%, 95% CI 9.5%-17.4%) compared with those with HHT Type 2 (HHT2) (2.4%, 95% CI 1.0%-3.8%) (p < 0.0001). In 55.2% (95% CI 38.3%-72.1%) of cases, the AVMs were symptomatic. Spetzler-Martin grade was 2 or less in 86.9% (95% CI 67.5%-95.2%) of patients. CONCLUSIONS The prevalence of brain AVMs in the HHT population is about 10%. HHT1 patients are significantly more likely to have brain AVMs than HHT2 patients. Most AVMs in the HHT population are symptomatic. The Spetzler-Martin grade for these lesions is 2 or less in nearly 90% of patients.

KW - Arteriovenous malformation

KW - Hereditary hemorrhagic telangiectasia

KW - Stroke

KW - Vascular disorders

UR - http://www.scopus.com/inward/record.url?scp=85026761818&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85026761818&partnerID=8YFLogxK

U2 - 10.3171/2016.7.JNS16847

DO - 10.3171/2016.7.JNS16847

M3 - Review article

VL - 127

SP - 302

EP - 310

JO - Journal of Neurosurgery

JF - Journal of Neurosurgery

SN - 0022-3085

IS - 2

ER -