Recurrent massive haemoperitoneum associated with ruptured corpus luteum in women with congenital afibrinogenemia; case report.
Recurrent massive haemoperitoneum associated with ruptured corpus luteum in women with congenital afibrinogenemia; case report.
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DOI：
10.4274/tjod.04935
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发表时间：
2014-12
影响因子：
1.1
通讯作者：
Atalay CR
中科院分区：
文献类型：
--
作者：
Özdemir Ö;Sarı ME;Kurt A;Şen E;Atalay CR
继发于黄体破裂的大量腹水是一种罕见但严重且危及生命的并发症，对于患有先天性出血疾病的女性来说，可能会导致手术干预，甚至卵巢切除术。先天性无纤维蛋白原血症是一种罕见的遗传性凝血障碍。由于它可以是无症状的，其临床表现从最小的出血倾向到危及生命的出血不等。由于排卵引起的腹内出血在这些患者中非常罕见，仅有少数无纤维蛋白原血症患者发生黄体破裂和腹膜积血。我们报告一位28岁的女性，患有先天性无纤维蛋白原血症，以排卵引起的腹内大出血为主要临床症状。第1组采用新鲜冰冻血浆+冷沉淀+输血；第2组采用剖腹探查切除破裂卵泡；第3组采用新鲜冰冻血浆+冷沉淀+输血；第4组采用剖腹探查+右侧输卵管切除术；第5组+新鲜冰冻血浆+冷沉淀+输血。保守治疗对先天性出血性疾病患者至关重要。这些病例表明，保守治疗可以保留卵巢功能，抑制排卵可以防止复发。
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.