Abstract
Massive hemoperitoneum secondary to ruptured corpus luteum is a rare but serious and life-threatening complication for women with congenital bleeding disorders and may lead to surgical interventions and even oophorectomy. Congenital afibrinogenemia is a rare inherited coagulation disorder. As it can be asymptomatic, its clinical manifestations vary from minimal tendency of bleeding to life-threatening bleedings. Intraabdominal bleeding due to ovulation is very rare in these patients and only a few cases of corpus luteum rupture and hemoperitoneum in afibrinogenemic patients have been described. We report on a 28-year-old woman with congenital afibrinogenemia with recurrent massive intraabdominal bleeding due to ovulation as the presenting clinical sign. The first episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions; exploratory laparotomy and excision of the ruptured follicle was performed at the second episode; the third episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions; exploratory laparotomy and right salphingooopherectomy was performed at the fourth episode; fifth episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions. Conservative management is crucial for patients with congenital bleeding disorders. These case demonstrate that preservation of ovarian function is possible with a conservative approach and recurrent episodes may be prevented by suppression of ovulation.