Vascular Malformations of the External Ear: A Systematic Review of Clinical Presentation, Diagnosis, and Management.

Background: Arteriovenous malformations of the external ear are extremely rare but can have significant aesthetic and functional implications. These malformations often originate from disruptions in the embryological development of the external ear, presenting as swelling accompanied by a bruit, bleeding, and a pulsatile mass. This systematic review aimed to examine the presentation, diagnosis, and management of congenital arteriovenous malformations of the external ear. Methods: A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Databases including MEDLINE, Embase, PubMed, and Cochrane were searched, using keywords related to congenital vascular malformations of the external ear without time restriction until September 2024. Results: Sixteen studies involving 40 patients were included. The median age was 19 years, with a male predominance (n = 22, 55%). Clinical presentations frequently included swelling (n = 20, 50%) and bruit/thrill (n = 17, 42.5%). The Schobinger classification of most patients was classified as stage II (n = 27, 67.5%). Angiography (n = 28, 70%) was the most commonly used diagnostic modality. Ethanol embolization was the predominant treatment, followed by combined surgical excision and preoperative embolization. Complications included blistering and skin necrosis, with a recurrence rate of 7.4% over a median follow-up period of 15 months. Conclusions: Ethanol embolization is effective in managing auricular arteriovenous malformations with higher success rates compared with other management approaches. Future studies should aim to optimize embolization protocols and explore combined treatment approaches to improve outcomes for complex vascular malformations of the external ear.