Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: Case report

OBJECTIVE AND IMPORTANCE: Type IVc arteriovenous malformations (AVMs) of th e spinal cord consist of multiple high-now feeding vessels, and they often present a challenging management situation. Their location is intradural an d extramedullary, and they are rare malformations that are difficult to tre at owing to the risk of thrombosis of the anterior spinal artery, The autho rs report a case of Type IVc spinal AVM in a patient with a family history of three siblings with pulmonary AVMs. Spinal AVMs have been reported to be associated with inherited syndromes such as familial cutaneous hemangiomas and Kartagener's syndrome, but an association with pulmonary AVMs has not previously been described. CLINICAL PRESENTATION: A 27-year-old man presented with sudden onset of occ ipital headache with cervical radiation while weightlifting. Results of com puted tomography of the brain were normal, but lumbar puncture revealed a s ubarachnoid hemorrhage, The patient had a 1-year history of a neurogenic bl adder and exhibited marked left calf muscle wasting. INTERVENTION: The patient underwent spinal magnetic resonance imaging, whic h revealed the AVM in the conus region. Selective spinal angiography was pe rformed for diagnostic purposes. A laminectomy was performed, and the vesse ls feeding the AVM were clipped, as was the fistula. CONCLUSION: The patient remained neurologically stable, and angiography con firmed obliteration of the AVM. This is the first case report of a patient with a spinal AVM who had multiple siblings with pulmonary malformations or AVMs.