Object Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder characterized by mucocutaneous telangiectasias, frequent nosebleeds, and visceral arteriovenous malformations (AVMs). Few reports have outlined the prevalence of the various cerebral vascular malformations found in patients with HHT. The authors set out to define the prevalence of cerebral vascular malformations in a population of HHT patients who underwent imaging with 3-T imaging (MRI/MR angiography [MRA]) of the brain. Methods A retrospective review of prospectively collected data was carried out using a database of 372 HHT patients who were seen and examined at the Georgia Regents University HHT Center and screened with 3-T MRI/MRA. Data were tabulated for numbers and types of vascular malformations in this population. Results Arteriovenous malformations were identified in 7.7%, developmental venous anomalies in 4.3%, and cerebral aneurysms in 2.4% of HHT patients. The HHT AVMs tended to be supratentorial, small, and cortical in this series, findings consistent with other recent studies in the literature. An arteriovenous fistula, cavernous malformation, and capillary telangiectasia were identified in 0.5%, 1%, and 1.9% of HHT patients, respectively. Conclusions Few studies have investigated the prevalence of the various vascular malformations found in HHT patients screened with 3-T MRI/MRA of the brain. Hereditary hemorrhagic telangiectasia AVMs are more likely to be multiple and have a tendency toward small size and cortical location. As such, they are often treated using a single-modality therapy.

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BACKGROUND Cavernous malformations in structures in and around the third ventricle are a challenging conceptual and surgical problem. No consensus exists on the ideal approach to such lesions. OBJECTIVE To perform a retrospective review of our institutional database to identify and evaluate approaches used to treat cavernous malformations located in and around the third ventricle. METHODS Information was extracted regarding lesion size and location, extent of resection, time to last follow-up, surgical approach, presenting symptoms, preoperative and postoperative neurological status, and specific approach-related morbidity. RESULTS All 39 neurosurgical operations (in 36 patients) were either an anterior interhemispheric (AIH) (44%, 17/39) or a supracerebellar infratentorial (SCIT) (56%, 22/39) approach. Gross-total resection was achieved in 23 of 39 procedures (59%), a near-total resection in 1 (3%), and subtotal resection in 15 (38%). For the 31 patients with at least 3 mo of follow-up, the mean modified Rankin Scale (mRS) score was 1.5. Of the 31 patients, 25 (81%) had an mRS score of 0 to 2, 4 had a mRS score of 3 (13%), and 1 each had a mRS score of 4 (3%) or 5 (3%). CONCLUSION Most approaches to cavernous malformations in and around the third ventricle treated at our institution have been either an AIH or a SCIT approach. The AIH approach was used for lesions involving the lateral wall of the third ventricle or the midline third ventricular floor, whereas the SCIT approach was used for lesions extending from the third ventricle into the dorsolateral midbrain, with acceptable clinical results.

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