Neurovascular Manifestations of Hereditary Hemorrhagic Telangiectasia: A Consecutive Series of 376 Patients during 15 Years.

BACKGROUND AND PURPOSE: Hereditary hemorrhagic telangiectasia is associated with a wide range of neurovascular abnormalities. The aim of this study was to characterize the spectrum of cerebrovascular lesions, including brain arteriovenous malformations, in patients with hereditary hemorrhagic telangiectasia and to study associations between brain arteriovenous malformations and demographic variables, genetic mutations, and the presence of AVMs in other organs. MATERIALS AND METHODS: Consecutive patients with definite hereditary hemorrhagic telangiectasia who underwent brain MR imaging/MRA, CTA, or DSA at our institution from 2001 to 2015 were included. All studies were re-evaluated by 2 senior neuroradiologists for the presence, characteristics, location, and number of brain arteriovenous malformations, intracranial aneurysms, and nonshunting lesions. Brain arteriovenous malformations were categorized as high-flow pial fistulas, nidus-type brain AVMs, and capillary vascular malformations and were assigned a Spetzler-Martin score. We examined the association between baseline clinical and genetic mutational status and the presence/multiplicity of brain arteriovenous malformations. RESULTS: Three hundred seventy-six patients with definite hereditary hemorrhagic telangiectasia were included. One hundred ten brain arteriovenous malformations were noted in 48 patients (12.8%), with multiple brain arteriovenous malformations in 26 patients. These included 51 nidal brain arteriovenous malformations (46.4%), 58 capillary vascular malformations (52.7%), and 1 pial arteriovenous fistula (0.9%). Five patients (10.4%) with single nidal brain arteriovenous malformation presented with hemorrhage. Of brain arteriovenous malformations, 88.9% (88/99) had a Spetzler-Martin score of ≤2. Patients with brain arteriovenous malformations were more likely to be female (75.0% versus 57.6%, P = .01) and have a family history of hereditary hemorrhagic telangiectasia (95.8% versus 84.8%, P = .04). The prevalence of brain arteriovenous malformation was 19.7% in endoglin (ENG) mutations and 12.5% in activin receptor-like kinase (1ACVRL1) mutations. CONCLUSIONS: Our study of 376 patients with hereditary hemorrhagic telangiectasia demonstrated a high prevalence of brain arteriovenous malformations. Nidal brain arteriovenous malformations and capillary vascular malformations occurred in roughly equal numbers.

Endovascular Treatment of Very Small Intracranial Aneurysms: Meta-Analysis.

BACKGROUND AND PURPOSE: Outcomes of endovascular treatment of very small intracranial aneurysms are still not well-characterized. Recently, several series assessing coil embolization of tiny aneurysms have presented new promising results. Thus, we performed a systematic review and meta-analysis of studies evaluating endovascular treatment of very small intracranial aneurysms. MATERIALS AND METHODS: We conducted a computerized search of Scopus, Medline, and the Web of Science for studies on endovascular treatment of very small (≤3 mm in diameter) intracranial aneurysms published between January 1996 and May 2015. Using a random-effects model, we evaluated clinical and angiographic outcomes. RESULTS: Twenty-two studies with 1105 tiny aneurysms (844 ruptured and 261 unruptured) endovascularly treated were included. Postoperative and long-term complete occlusion was achieved in 85% (95% CI, 78%-90%) and 91% (95% CI, 87%-94%) of aneurysms, respectively. The recanalization rate was 6% (95% CI, 4%-11%) and retreatment occurred in 7% (95% CI, 5%-9%) of cases. Seventy-nine percent (95% CI, 64%-89%) of patients had good neurologic outcome at long-term follow-up. Intraprocedural rupture occurred in 7% (95% CI, 5%-9%) of the coiling procedures, while thromboembolic complications occurred in 4% (95% CI, 3%-6%). CONCLUSIONS: Coil embolization of very small intracranial aneurysms can be performed safely and effectively. In the case of unruptured aneurysms, procedure-related complications are not negligible. Patients and providers should consider such risks when engaged in a shared decision-making process.