Spinal intradural perimedullary arteriovenous fistula with varix in infant

Abstract A rare occurrence of type IV spinal arteriovenous malformation (intradural perimedullary arteriovenous fistula) is described in an 18-month-old boy initially misdiagnosed with Guillain-Barre syndrome. An intramedullary mixed-intensity mass lesion at Th1 was demonstrated by magnetic resonance imaging together with flow voids over the dorsal aspect of the swollen spinal cord. Angiography demonstrated an intradural perimedullary arteriovenous fistula including an intraparenchymal vascular pocket. After partial embolisation of the posterior spinal arteries through the left intercostal-radicular artery, the arteriovenous fistula was removed completely together with an organised haematoma. The fistula directly opened into a vascular pocket, which was confirmed pathologically to be a varix. The postoperative course was uneventful, and the patient resumed ambulation within 4 months. The case, subclassifiable as a type IVb spinal perimedullary AVF, was unique given its location and the patient’s age at presentation.