Improved treatment feasibility in children with hemophilia using arteriovenous fistulae: the results after seven years of follow-up.

BACKGROUND: An easy and stable venous access is essential in hemophilic children who receive regular prophylaxis or immune tolerance induction treatment. Central venous access devices improve treatment feasibility, but their use is complicated by infection and/or thrombosis. Arteriovenous fistula (AVF) has been evaluated as an alternative to central venous access devices in hemophilic children since 1999. DESIGN AND METHODS: This study provides results obtained in a large series after seven years of follow-up. RESULTS: From 1999 to 2008, 43 procedures were performed in 38 children (median age: 2.7 years). Thirty-five AVFs (81%) achieved maturation after a median of 58 days and were used for a median of five years (range: 0.4-8.5). A brachial artery caliber larger than 1.2 mm was associated with successful maturation (p<0.05). Complications with some impact on arteriovenous fistula use or duration were observed in 14/43 procedures (32%) and in 13/38 children (34%). Age at arteriovenous fistula creation was younger in children who lost arteriovenous fistula patency (p<0.05) and aneurysms were more frequent in children who were on daily treatment regimen and thus had a greater cumulative number of arteriovenous fistula accesses (p<0.05). At the end of the follow-up period, 22 AVFs were still in use and 9 had been surgically dismantled. Arteriovenous fistula use allowed long-term prophylaxis (up to 8.5 years) in 11 children and the completion of immune tolerance induction without interruptions in 18 children. CONCLUSIONS: This study confirms the feasibility of arteriovenous fistula with an acceptable rate of complications and suggests that its use is particularly favorable in children with inhibitors in whom it should be considered as first-choice venous access.

Four-year survival after trans-jugular intrahepatic porto-systemic shunt for veno-occlusive disease following autologous bone marrow transplantation.

Severe hepatic veno-occlusive disease is still a potentially lethal complication after bone marrow transplantation. We here report the case of a patient who developed liver veno-occlusive disease with severe hemodynamic dysfunction and was successfully treated by means of a trans-jugular intrahepatic portosystemic shunt. After three years, he is still disease-free with a functioning shunt and a normal laboratory liver profile. A trans-jugular intrahepatic portosystemic shunt is a treatment option that has been used in very few patients affected by hepatic veno-occlusive disease; its indications and timing remain to be established.