TOTAL ENDOVASCULAR OCCLUSION OF A GIANT DIRECT ARTERIOVENOUS-FISTULA IN THE POSTERIOR-FOSSA IN A CASE OF RENDU-OSLER-WEBER DISEASE

We report a case of giant single-hole direct arteriovenous fistula (AV F) located in the posterior fossa of a child with Rendu-Osler-Weber di sease (ROW). There is neither a clinical nor an angioarchitectural dif ference between AVFs associated with ROW and sporadic AVFs, but ROW sh ould be suspected in children with multifocal pial AVFs. Transarterial embolization was performed to obliterate the AVF in a unique procedur e using simultaneous bifemoral catheterization. At follow up 4 years l ater, the clinical examination was normal. Control MR scans and angiog raphy showed the total occlusion of the fistula, permanent thrombosis of the venous pouch, and disappearance of the abnormal venous drainage . In this case, the endovascular procedure was available and we estima ted that it represented a lesser risk than the surgical approach (risk of hemorrhage and risk of thrombosis involving the feeding arteries a nd the brain stem venous drainage). Furthermore, it avoids craniotomy and reduces the duration of hospitalization.