Bernard-Soulier Syndrome (BSS) is a disorder which manifests prolonged bleeding time, thrombocytopenia, and giant platelets. The prevalence of this disease has been reported to be less than 1 in 1,000,000. Bleeding disorders patients can be among the most difficult surgical patients to manage and deal with. Intra-operative and post-operative hemorrhage can be a life-threatening complication in even the most routine surgical procedures. There are no standards methods to deal with management of intra operative bleeding associated with BSS, since this disorder is very rare. Management of intraoperative hemorrhage with pre-operative or during operation HLA-matched platelets, and topical hemostatic agents (gel foam, thrombin), systemic aminocaproic acid, transfused rVIIa, and DDAVP (desmopressin), resulted in good hemostasis and a long-lasting healing response. For these rare disease, we present case report in which a combination of systemic and topical methods may be contributing in achieving good hemostasis during intra-operative hemorrhage.
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