Prevalence of Intracranial Aneurysms in Hereditary Hemorrhagic Telangiectasia: Report from a Single Reference Center

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FIG 1.
Cerebral MRA (A) and DSA (B) images of a 39-year-old female patient that meet 4 of 4 Curaçao criteria show an ophthalmic segment of a left carotid artery aneurysm (arrowhead) and AVMs in the parietal and occipital contralateral lobes (arrows).
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FIG 2.
Cerebral MRA (A) and DSA (B) images of a 78-year-old female patient that meet 4 of 4 Curaçao criteria show a right MCA bifurcation aneurysm (arrowhead) and another one located in the right posterior communicating artery (arrow).

Table 1:

Population characteristics

Baseline Characteristics	n = 228
Sex (female) (No.) (%)	141 (61.8%)
Age (median) (IQR) (yr)	49.0 (38.4–64.1)
Curaçao criteria (No.) (%)
Epistaxis	209 (91.7%)
Telangiectasias	219 (96.1%)
Arteriovenous malformations	163 (71.5%)
Family history	197 (86.4%)
Curaçao criteria (3 of 4) (No.) (%)	90 (39.5%)
Curaçao criteria (4 of 4) (No.) (%)	126 (55.3%)
CTA (No.) (%)	24 (10.5%)
MRA (No.) (%)	203 (89.0%)
Cerebral angiography (No.) (%)	36 (15.8%)
BVM (No.) (%)	60 (26.3%)
AVM (No.) (%)	39 (17.1%)
IA	33 (14.5%)

Table 2:

Intracranial aneurysm characteristics

Characteristics	n = 37
Location (No.) (%)
MCA	9 (24.3%)
Intracavernous carotid artery	9 (24.3%)
Ophthalmic artery	8 (21.6%)
Posterior communicating artery	3 (8.1%)
Anterior communicating artery	2 (5.4%)
Anterior cerebral artery	1 (2.7%)
Posterior cerebral artery	2 (5.4%)
Basilar artery	3 (8.1%)
Width (mm)^{^a}	3.2 (2.6–4.4)
Height (mm)^{^a}	2.3 (1.9–2.9)
Neck (mm)^{^a}	2.6 (2.0–3.8)
Aspect (mm)^{^a}	0.83 (0.6–1.3)
Size (mm)^{^a}	1.2 (1.0–1.5)

↵^aNon-normally distributed variables are expressed in median and IQR (25th–75th percentiles).

Table 3:

Bivariate analysis between baseline variables and intracranial aneurysms

Baseline Characteristics	Intracranial Aneurysm (n = 33)	No Intracranial Aneurysm (n = 195)	P Value^{^a}
Sex (female) (No.) (%)	21 (63.6%)	120 (61.5%)	.85
Age (median) (IQR) (yr)	51.4 (43.3–69.3)	48.0 (38–63.2)	.10
Curaçao criteria (No.) (%)
Epistaxis	33 (100%)	176 (90.3%)	.08
Telangiectasias	32 (97.0%)	187 (95.9%)	1.00
AVMs	25 (75.8%)	138 (70.8%)	.68
Family history	26 (78.8%)	171 (87.7%)	.17
BVMs (No.) (%)	13 (39.4%)	47 (24.1%)	.09
AVMs (No.) (%)	7 (21.2%)	32 (16.4%)	.46
Hypertension (n = 198) (No.) (%)^{^b}	8 (25.8%)	29 (18.0%)	.32
Tobacco use (n = 189) (No.) (%)^{^b}	11 (33.3%)	39 (25.0%)	.39
Extracranial aneurysms (No.) (%) (n = 140)^{^b}	7 (35.0%)	20 (16.7%)	.07
Genetic results (No.) (%) (n = 81)^{^b}	9 (27.3%)	72 (36.9%)
ENG	4 (44.4%)	35 (48.6%)
ACVRL1	3 (33.3%)	22 (30.6%)	.43
MADH4	0	10 (13.9%)
Noninformative	2 (22.2%)	5 (6.9%)

↵^aTwo-sided P values. Medians are analyzed with Mann-Whitney U tests, and dichotomous variables, with the Fisher exact test.
↵^bIncluded patients with available data for analysis from the medical record system.