Auricular arteriovenous malformation: Evaluation, management, and outcome

Background: The external ear is the second most common site for extracranial arterioverrous malformation in the head and neck. Methods: This retrospective review of 41 patients with auricular arteriovenous malformation was based on medical records, imaging studies, and photographs. Data were collected on natural history, progression, and outcome; patients were questioned about quality of life after treatment. Results: The median age at initial presentation was 26 years (range, 1 to 55 years), and Schobinger stage was 1 in two patients, II in 19 patients, and III in 20 patients. No patients had a Schobinger stage of IV. Expansion occurred during childhood in seven patients, adolescence in 14 patients, pregnancy in 10 patients, and adulthood in 10 patients. Distribution of auricular and extra-auricular arteriovenous malformation was not limited to "watershed" areas between vascular territories (angiosomes). Twelve patients were untreated (follow-up, 0.5 to 6 years). Mean follow-up time for the 29 treated patients was 5.19 years (range, 1 to 18.75 years). Proximal ligation in nine patients caused progression: eight of them underwent amputation and one had embolization. Fifteen patients had embolization only: the arterioverrous malformation worsened and amputation was necessary in six patients; in the remaining nine patients, two improved, four persisted, and three worsened. Of 20 patients who had auricular amputation, 16 (80 percent) were controlled, three (15 percent) improved, and one had unresectable, residual cervicofacial arterioverrous malformation. Of 22 of 29 treated patients surveyed, 81 percent were satisfied with their management. Hearing was either unaffected (n = 15) or diminished (n = 5); two patients noted decreased sound localization. Conclusions: The authors recommend periodic evaluation for stage I to II auricular arteriovenous malformation and intervention if there is evolution to stage III. Preoperative embolization and partial or total amputation effectively control auricular and para-auricular arteriovenous malformation. Embolization can be palliative in children or in patients who are not psychologically prepared for amputation. Extensive extra-auricular arteriovenous malformation requires individualized endovascular therapy and resection.