Arteriovenous fistula for long-term venous access for boys with hemophilia

Hemophilia is a sex-linked condition affecting about 1 of every 5000 males in the United States. The management of children with hemophilia can be improved with regular intravenous infusion of factor VIII or IX, thus preventing crippling and sometimes fatal hemorrhage. Maintaining this vital intravenous access is often hampered by gradual loss of superficial veins or repeated central catheter sepsis and thrombosis. This study reviewed an experience with arteriovenous fistula in selected hemophilia patients with limited venous access.

Consecutive patients operated on between October 2000 and July 2006 for venous access with the creation of an arteriovenous fistula were reviewed. They were selected because of repeated problems with other venous access. Patency, ease of use, duplex scan derived brachial artery diameter, and arm length were assessed.

During a 69-month period, 10 arteriovenous fistulas (five brachial artery-basilic vein fistulas, 5 brachial artery-cephalic vein fistulas) were created for nine patients. The patients were a median age of 5.5 years (range, 1 to 27 years), and all were <13 except the 27-year-old patient. There were no postoperative hematomas requiring evacuation. One arteriovenous fistula failed to mature and was redone in the opposite arm, which subsequently occluded after 13 months. Of the mature fistulas, patency was 100% at 1 year, 80% (4/5) at 3 years, and 75% (3/4) at 4 years, with mean follow-up of 22 months. Brachial artery diameter increased in the involved arm by a ratio of 1.95 (range, 1.51 to 2.5) compared with the opposite arm. Arm length disparity was increased by 0.5 cm (range, 0.8 to 1.5 cm) in the involved arm. All fistulas allowed good access at home by a care provider.

For hemophilia patients with compromised venous access, arteriovenous fistulas provide good early patency. Brachial artery diameter and arm length require continued follow-up.