Recurrent massive hemoperitoneum due to ovulation as a clinical sign in congenital afibrinogenemia

Massive hemoperitoneum due to ovulation is a rare but serious and life-threatening complication for women with coagulation disorders, and may lead to surgical interventions and even oophorectomy. Congenital afibrinogenemia is an uncommon coagulation disorder usually discovered during childhood. 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. In all women, the diagnosis was known since childhood. We report on a 24-year-old woman with congenital afibrinogenemia with recurrent massive intraabdominal bleeding due to ovulation as the presenting clinical sign. Exploratory laparotomy and excision of the ruptured follicle was performed at the first bleeding episode; the second episode was managed with fresh frozen plasma and blood transfusions. Conservative management is crucial for these patients. If surgery cannot be avoided, a conservative surgical approach should be chosen to preserve ovarian function.