Background: Hemophilia A is an X-linked recessive disorder caused by a deficiency in factor VIII. Hemophilia A affects 1 in 5,000-10,000 males. Hematuria is frequent in hemophilia. Hematuria in hemophilia is generally considered benign and manageable with conservative therapy; however, severe hematuria requiring surgical therapy has rarely been reported.
Case presentation: A 60-year-old male with hemophilia A presented with persistent gross hematuria of unknown cause. He was treated with recombinant factor VIII products, followed by several conservative therapies as follows: clot evacuation by vesicoclysis, continuous bladder irrigation with normal saline, and intravesical instillation of aluminum hydroxide/magnesium hydroxide (Maalox); however, these failed to resolve the hemorrhaging. The patient was offered and consented to cystectomy with an ileal conduit. Intraoperative clotting was normal with the infusion of adequate recombinant factor VIII products and transfusion of fresh-frozen plasma, and the procedure was performed safely. After surgery, the patient had blood in his stool several times. No bleeding site was demonstrated in the colon by colonoscopy and (99m)Technetium-human serum albumin-diethylenetriaminepenta-acetic acid scintigraphy demonstrated that the extravasation of radioactive isotope was detected at the anal side of terminal ileum but not at the oral side. These findings were suspected to be bleeding from the ileoileal anastomosis. However, the bleeding was managed with recombinant factor VIII products.
Conclusions: Cystectomy in hemophilia may be safe, if monitored appropriately. Urinary diversion using the intestine may be avoided because anastomotic hemorrhage may become a problem.