Arteriovenous malformation of the external ear: a clinical assessment with a scoping review of the literature / Malformação arteriovenosa da orelha externa: avaliação clínica com revisão abrangente da literatura

Abstract Introduction: Auricular Arteriovenous Malformation of the external ear is a rarely encountered disease; in particular, arteriovenous malformation arising from the auricle, with spontaneous bleeding, has seldom been reported. Objective: In the current study, we report an unusual case of late-onset auricular arteriovenous malformation originating from the posterior auricular artery that was confirmed by computed tomographic angiography. The case was successfully managed by pre-surgical intravascular embolization followed by total lesion excision. Prompted by this case, we also present a scoping review of the literature. Methods: A case of a 60 year-old man with right auricular arteriovenous malformation treated in our tertiary care center, and 52 patients with auricular arteriovenous malformation described in 10 case reports and a retrospective review are presented. Auricular arteriovenous malformation can manifest as swelling of the ear, pulsatile tinnitus, pain, and/or bleeding. On physical examination, a pulsatile swelling and/or a tender mass is evident. When arteriovenous malformation is suspected, the lesions should be visualized using imaging modalities that optimally detect vascular lesions, and managed via embolization, mass excision, or auricular resection. Effectiveness of the various diagnostic methods used and the treatment outcomes were analyzed. Results: Various imaging modalities including Doppler sonography, computed tomographic angiography, magnetic resonance angiography, and/or transfemoral cerebral angiography were used to diagnose 38 cases reported in the literature. In another 15 cases, no imaging was performed; treatment was determined solely by physical examination and auscultation. Of the total of 53 cases, 12 were not treated (their symptoms were merely observed) whereas 20 underwent therapeutic embolization. In total, 32 patients, including 1 patient who was not treated and 10 with persistent or aggravated arteriovenous malformation after previous embolization, underwent mass excision or auricular resection depending on the extent of the lesion. No major postoperative complication was recorded. The postoperative follow-up duration varied from 1 month to 19 years, and only one case of unresectable, residual cervicofacial arteriovenous malformation was recorded. Conclusion: Auricular arteriovenous malformation is a rarely encountered disease, but should be suspected if a patient presents with a swollen ear and pulsatile tinnitus. Appropriate imaging is essential for diagnosis and evaluation of the extent of disease. As embolization affords only relatively poor control, total surgical removal of the vascular mass is recommended.

Resumo Introdução: Malformação Arteriovenosa Auricular da orelha externa é uma doença raramente observada, em particular, na região da aurícula, com hemorragia espontânea, tem sido infrequentemente relatada. Objetivo: No presente estudo, relatamos um caso incomum de malformação arteriovenosa auricular de início tardio originária da artéria auricular posterior confirmada por angiotomografia computadorizada. O caso foi controlado com sucesso por embolização endovascular pré-cirúrgica seguida por excisão completa da lesão. Além disso, nós também apresentamos uma revisão abrangente da literatura. Método: Um homem de 60 anos de idade com malformação arteriovenosa auricular direita tratado em nosso centro de atendimento terciário e 52 pacientes com malformação arteriovenosa auricular descritos em 10 relatos de casos e um estudo de revisão são apresentados. A malformação arteriovenosa auricular pode manifestar-se como inchaço da orelha, zumbido pulsátil, dor e/ou sangramento. Ao exame físico, um edema pulsátil e/ou uma massa sensível é evidente. Quando há suspeita de malformação arteriovenosa, as lesões devem ser visualizadas usando modalidades de imagem que detectam de maneira ideal as lesões vasculares, e tratadas por meio de embolização, excisão total da lesão, ou ressecção auricular. A eficácia dos vários métodos de diagnóstico utilizados e os desfechos do tratamento foram analisados. Resultados: Várias modalidades de imagem, incluindo ultrassonografia Doppler, angiotomografia computadorizada, angiografia por ressonância magnética e/ou angiografia cerebral transfemoral foram usadas para diagnosticar 38 casos relatados na literatura. Em outros 15 casos, nenhuma imagem foi realizada; o tratamento foi determinado unicamente pelo exame físico e ausculta. Do total de 53 casos, 12 não foram tratados (os seus sintomas foram apenas observados), enquanto que 20 foram submetidos a embolização terapêutica. No total, 32 doentes, incluindo um paciente que não foi tratado e 10 com malformação arteriovenosa persistente ou agravada após a embolização anterior, foram submetidos a excisão completa da lesão ou ressecção auricular, dependendo da extensão da lesão. Nenhuma complicação pós-operatória importante foi registrada. O tempo de seguimento pós-operatório variou de 1 mês a 19 anos, e apenas um caso de malformação arteriovenosa cervicofacial irressecável, residual foi registrado. Conclusão: A malformação arteriovenosa auricular é uma doença raramente encontrada, mas deve ser suspeitada se um paciente apresentar orelha inchada e zumbido pulsátil. A imagem apropriada é essencial para o diagnóstico e avaliação da extensão da doença. Como a embolização proporciona apenas um controle relativamente precário, a remoção cirúrgica total da lesão vascular é recomendada.

Arteriovenous malformation of the external ear: a clinical assessment with a scoping review of the literature.

INTRODUCTION: Auricular Arteriovenous Malformation of the external ear is a rarely encountered disease; in particular, arteriovenous malformation arising from the auricle, with spontaneous bleeding, has seldom been reported. OBJECTIVE: In the current study, we report an unusual case of late-onset auricular arteriovenous malformation originating from the posterior auricular artery that was confirmed by computed tomographic angiography. The case was successfully managed by pre-surgical intravascular embolization followed by total lesion excision. Prompted by this case, we also present a scoping review of the literature. METHODS: A case of a 60 year-old man with right auricular arteriovenous malformation treated in our tertiary care center, and 52 patients with auricular arteriovenous malformation described in 10 case reports and a retrospective review are presented. Auricular arteriovenous malformation can manifest as swelling of the ear, pulsatile tinnitus, pain, and/or bleeding. On physical examination, a pulsatile swelling and/or a tender mass is evident. When arteriovenous malformation is suspected, the lesions should be visualized using imaging modalities that optimally detect vascular lesions, and managed via embolization, mass excision, or auricular resection. Effectiveness of the various diagnostic methods used and the treatment outcomes were analyzed. RESULTS: Various imaging modalities including Doppler sonography, computed tomographic angiography, magnetic resonance angiography, and/or transfemoral cerebral angiography were used to diagnose 38 cases reported in the literature. In another 15 cases, no imaging was performed; treatment was determined solely by physical examination and auscultation. Of the total of 53 cases, 12 were not treated (their symptoms were merely observed) whereas 20 underwent therapeutic embolization. In total, 32 patients, including 1 patient who was not treated and 10 with persistent or aggravated arteriovenous malformation after previous embolization, underwent mass excision or auricular resection depending on the extent of the lesion. No major postoperative complication was recorded. The postoperative follow-up duration varied from 1 month to 19 years, and only one case of unresectable, residual cervicofacial arteriovenous malformation was recorded. CONCLUSION: Auricular arteriovenous malformation is a rarely encountered disease, but should be suspected if a patient presents with a swollen ear and pulsatile tinnitus. Appropriate imaging is essential for diagnosis and evaluation of the extent of disease. As embolization affords only relatively poor control, total surgical removal of the vascular mass is recommended.