Aljobeh, Ahmad

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder that results in arteriovenous malformations (AVMs) in the nose, mucocutaneous surfaces and visceral organs, including lung, brain, liver, bowel and rarely spinal cord. We describe a case of a young child with HHT who presented with acute paraparesis due to acute thrombosis of a spinal perimedullary arteriovenous fistula. Patient underwent coil embolization of spinal arteriovenous shunt with resolution of clinical symptoms. This case highlights the possibility of catastrophic complications in young children with HHT, the potential preventive role of screening for spinal AVMs in HHT and the importance of timely intervention.

OBJECTIVECavernous internal carotid artery (ICA) aneurysms are frequently diagnosed incidentally and the benign natural history of these lesions is well known, but there is limited information assessing the risk of growth in untreated patients. The authors sought to assess and analyze risk factors in patients with cavernous ICA aneurysms and compare them to those of patients with intracranial berry aneurysms in other locations.METHODSData from consecutive patients who were diagnosed with a cavernous ICA aneurysm were retrospectively reviewed. The authors evaluated patients for the incidence of cavernous ICA aneurysm growth and rupture. In addition, the authors analyzed risk factors for cavernous ICA aneurysm growth and compared them to risk factors in a population of patients diagnosed with intracranial berry aneurysms in locations other than the cavernous ICA during the same period.RESULTSIn 194 patients with 208 cavernous ICA aneurysms, the authors found a high risk of aneurysm growth (19.2% per patient-year) in patients with large/giant aneurysms. Size was significantly associated with higher risk of growth. Compared to patients with intracranial berry aneurysms in other locations, patients with cavernous ICA aneurysms were significantly more likely to be female and have a lower incidence of hypertension.CONCLUSIONSAneurysms of the cavernous ICA are benign lesions with a negligible risk of rupture but a definite risk of growth. Aneurysm size was found to be associated with aneurysm growth, which can be associated with new onset of symptoms. Serial follow-up imaging of a cavernous ICA aneurysm might be indicated to monitor for asymptomatic growth, especially in patients with larger lesions.