Multimodality Treatment of a Complex Cervicocerebral Arteriovenous Shunt in a Patient With CHARGE Syndrome

Abstract

We present our management of a unique case of complex arteriovenous shunt with vascular steal in the left-sided head and neck vessels in a child with CHARGE (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness) syndrome. A 10-year-old girl presented with high-output heart failure. Cerebral angiography revealed high-flow abnormal fistulous connections between the left common carotid artery and innominate vein as well as between the vertebral artery and innominate vein. There was significant collateral blood flow to the fistulae from the left external carotid artery and left thyrocervical and costocervical trunks. The left vertebral artery-to-innominate vein fistula was occluded by endovascular means during temporary balloon occlusion. The left common carotid artery-to-innominate vein fistula was occluded through neck dissection with surgical clipping. Combined neurosurgical and endovascular techniques were used successfully to manage a complex arteriovenous fistula in a patient with CHARGE syndrome. Challenges in therapeutic decision making are discussed.