Prevalence and Characteristics of Intracranial Aneurysms in Hereditary Hemorrhagic Telangiectasia

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Fig 1.
Representative images of a patient with HHT with an IA unrelated to AVMs. A, MRA demonstrates an ophthalmic segment aneurysm of the right ICA (arrowhead) projecting medially. B, 3D reconstruction of a DSA shows a bilobed, wide-neck, right ophthalmic segment aneurysm of the right ICA (arrow).
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Fig 2.
Representative images of a patient with HHT with flow-related cerebral aneurysms. T1 contrast-enhanced MR images demonstrate a Spetzler-Martin grade III right frontal AVM with a nidus size of 4 cm (A, asterisk) and venous varices (B, arrowhead), supplied by several dilated arterial feeders from the right anterior cerebral arteries (B, arrow) and the right middle cerebral arteries (C, arrow). MRA demonstrates a clinoid segment aneurysm of the right ICA (D, arrowhead) and another aneurysm arising from the ophthalmic segment of the left ICA (E, arrowhead).

Table 1:

Basic demographics of 180 subjects with HHT and brain VMs

Characteristic	Summary^{^a} (%)
Age at enrollment (mean) (yr)	34.2 (SD, 21.1)
Women	111/180 (61.7%)
Curaçao criteria
Epistaxis	168/180 (93.3%)
Mucosal telangiectasias	141/180 (78.3%)
Pulmonary AVMs	91/180 (50.6%)
Liver VMs	14/180 (7.8%)
Gastrointestinal bleeding	9/180 (5.0%)
Affected first-degree relative	135/180 (75%)
Genetic mutation
ENG	109/131 (83.2%)
ACVRL1	21/131 (16.0%)
SMAD4	1/131 (0.7%)
DSA	116/180 (64.4%)
MRA	114/180 (63.3%)
CTA	5/180 (2.8%)
AVM	113/180 (62.8%)
AVF	16/180 (8.9%)
IAs	9/180 (5%)

↵a Summary is the number of observations with each value over the total available numbers (and the percentage) or the mean (SD) for continuous variables.

Table 2:

Bivariate analysis between baseline demographics and IAs

Outcome	Patients with IAs (n = 9)	Patients without IAs (n =171)	P Value
Age (mean) (yr)	48.1 (SD, 18.2)	33.5 (SD, 21.0)	.042
Women	7/9 (77.8%)	104/171 (60.8%)	.486
Curaçao criteria
Epistaxis	9/9 (100%)	159/171 (93.0%)	1.000
Telangiectasias	8/9 (88.9%)	133/171 (77.8%)	.687
Pulmonary AVMs	6/9 (66.7%)	85/171 (49.7%)	.497
Liver VMs	2/9 (22.2%)	12/171 (7.0%)	.148
Gastrointestinal bleeding	0/9 (0%)	9/171 (5%)	1.000
Affected first-degree relative	5/9 (55.6%)	130/171 (76.0%)	.230
AVM	6/9 (66.7%)	107/171 (62.6%)	1.000
AVF	1/9 (11.1%)	15/171 (8.8%)	.576
AVM or AVF	7/9 (77.8%)	122/171 (71.3%)	1.000