Patients with Ehlers-Danlos syndrome (EDS), especially types IV, VI, and VIII, are at increased risk of bleeding, and most do not have specific hemostatic deficiencies that would be amenable to replacement therapy. We have investigated the ability of DDAVP (desmopressin acetate) to control bleeding in EDS.
Two children with EDS, types VIII and VI, presented with hemorrhagic symptoms and scheduled surgical procedures. Ivy bleeding times (BTs) were measured before and after intravenous (i.v.) DDAVP challenge, and i.v. DDAVP was used prophylactically for their procedures. Laboratory testing was performed to rule out other hemostatic disorders.
Both patients had prolonged BTs that corrected following i.v. DDAVP therapy; all other laboratory values were normal. Both patients had excellent clinical hemostasis with surgery, and one has continued to use intranasal DDAVP to control epistaxis and gingival bleeding.
The bleeding time in both patients was corrected with DDAVP, and the patients did not have any postoperative bleeding. DDAVP should be considered in other patients who have EDS with bleeding tendencies.
From the Department of Pediatrics, Section of Hematology/Oncology, Comprehensive Hemophilia Center, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, AR 72202, U.S.A.
Submitted for publication August 21, 1996. Accepted November 13, 1996.
Address correspondence and reprint requests to Dr. Kimo C. Stine, Department of Pediatrics, Section of Hematology/Oncology, Comprehensive Hemophilia Center, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, 800 Marshall Street, Little Rock, AR 72202, U.S.A.