Collagen and bleeding diathesis in Ehlers-Danlos syndrome.

There are at least 7 variants of Ehlers-Danlos syndrome (EDS). One family with type 1, one with type 3 and one isolated case with an undefined type of EDS are described. They all had bleeding symptoms, but surgery had been performed without complications. Collagen is believed to be defective in EDS. However, in our cases the platelet aggregating power of the collagen was normal. The bleeding time (Ivy) was slightly prolonged. Extensive examination of the haemostatic process revealed a decreased platelet adhesiveness in only 2 of the patients, a finding believed to be coincidental. The history of former operations on the patients and on affected relatives is considered to be the best guide in determining the risk of an operation, but preoperative estimation of the bleeding time is recommended.