摘要：The Spinal Arterio-Venous Metameric Syndrome (SAMS) is a rare non-hereditary disease that presents itself with spinal metameric Arterio-Venous Malformations (AVMs) and cutaneous lesions in the same dermatome. We present female patient with history of cesarean section at the age of 19 and thoracolumbar AVM, that was treated through endovascular embolization (2006). Patient deteriorates progressively and consults at the age of 24 (2011) with chronic back-pain, difficulty to walk, spastic paraparesia, and severe sphincter dysfunction. Cutaneous phenotypic nevi are observed in the right inferior thoracic area. Radiologic studies show medullary cone edema and multiple bilateral AVMs from T8 to L3 with AVM around medullary cone. Following the interpretation of said studies, SAMS diagnosis is made. Patient was treated twice in 2011 through multiple thoracolumbar supraselective embolizations (T8 to L1). Medullary cone AVM was considered a surgical risk for embolization. After embolizations, patient gets considerably better and remains this way until March, 2018 when follow-up is made (32 years). SAMS is a syndrome with complex radiologic presentation and interpretation and consequently, with a large differential diagnosis. Phenotypic findings include angioma-type macular cutaneous lesions and neurological clinical manifestations. CT and MRI evidence severe vertebral lesions, hyperintense spinal cord in T2W due to edema, hypointense hemorrhagic residues and signal-less para-lumbar, medullary and/or peri-medullary malformations in the same dermatome. Spinal angiography studies are extremely useful in the diagnosis, treatment and follow-up of disease. A multidisciplinary medical team is essential for correct diagnosis and opportune treatment.
关键词：Síndrome de Malformaciones Arterio-Venosas Espinales Metaméricas;Síndrome Cobb;Embolización supraselectiva
