HIGH-FLOW PRIAPISM ASSOCIATED WITH FABRYS-DISEASE IN A CHILD - A CASE-REPORT AND REVIEW OF THE LITERATURE

We report a case of priapism in an 11-year-old boy known to have Fabry 's disease. High flow was confirmed by color flow Doppler ultrasound a nd intracorporal blood gas measurements. Successful treatment was achi eved by unilateral percutaneous Gelfoam embolization of the left inter nal pudendal artery. Previously reported cases of priapism associated with Fabry's disease were identified through a MEDLINE search of the E nglish literature and review of the publications. Five reported cases of priapism associated with Fabry's disease were found, including thos e of 3 children. A report of a child with high-flow priapism that did not respond to conventional treatment including cavernovenous shunting was noted. Priapism associated with Fabry's disease may be caused by unregulated high arterial inflow. Early recognition of the underlying pathophysiology may identify those cases that would be amenable to per cutaneous embolization therapy and may obviate the need to pursue othe r ineffective conventional treatments. Copyright 1996 by Elsevier Scie nce Inc.