Successful surgical treatment of complete cervical agenesia with duplicate uterus associated with severe DIE

Cervical agenesis is a rare clinical condition presenting with primary amenorrhea and is often associated with other Müllerian duct malformations. Quick and definitive surgical treatment is essential in preventing later, severe complications associating with consequential endometriosis. The unsuccessful series of operations often result in loss of the uterus. The major challenge of the surgical approaches is not to reconstruct the flow of menstrual fluid but to sustain the anatomic situation and prevent later stenosis and obstruction. In our case report we present the surgical steps essential to the successful complex treatment of the malformation with excellent long-term results. In our case a 26-year-old patient with a history of 3 previous unsuccessful operations presented to our clinic with stage IV endometriosis. The patient had severe abdominal pain with each menstruation and MRI scan confirmed complete cervical agenesia with uterus duplex and consequent haematometra, haematosalpinx and endometriotic cysts (DIE). We performed laparotomy with a complete eradication of DIE and simple trachelectomy of the agenetic tissue with metroplasty with the preservation of the uterine arteries and uterus. We would like to present our fully photo and video documented case report with pre- and postoperative imaging documentation of the successful case.The ethical approval registration number at the University Of Debrecen is: DE KK RKEB.IKEB 6278A-2022 Cervical agenesis is a rare clinical condition presenting with primary amenorrhea and is often associated with other Müllerian duct malformations. Quick and definitive surgical treatment is essential in preventing later, severe complications associating with consequential endometriosis. The unsuccessful series of operations often result in loss of the uterus. The major challenge of the surgical approaches is not to reconstruct the flow of menstrual fluid but to sustain the anatomic situation and prevent later stenosis and obstruction. In our case report we present the surgical steps essential to the successful complex treatment of the malformation with excellent long-term results. In our case a 26-year-old patient with a history of 3 previous unsuccessful operations presented to our clinic with stage IV endometriosis. The patient had severe abdominal pain with each menstruation and MRI scan confirmed complete cervical agenesia with uterus duplex and consequent haematometra, haematosalpinx and endometriotic cysts (DIE). We performed laparotomy with a complete eradication of DIE and simple trachelectomy of the agenetic tissue with metroplasty with the preservation of the uterine arteries and uterus. We would like to present our fully photo and video documented case report with pre- and postoperative imaging documentation of the successful case. The ethical approval registration number at the University Of Debrecen is: DE KK RKEB.IKEB 6278A-2022 1. Deffarges JV, Haddad B, Musset R, et al: Utero-vaginal anastomosis in women with uterine cervix atresia: long-term follow-up and reproductive performance. A study of 18 cases. Hum Reprod 2001;16:17222. Fujimoto VY, Miller JH, Klein NA, et al: Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol 1997; 177:14193. Bugmann P, Amaudruz M, Hanquinet S, et al: Uterocervicoplasty with a bladder mucosa layer for the treatment of complete cervical agenesis. Fertil Steril 2002; 77:8314. Niver DH, Barrette G, Jewelewicz R: Congenital atresia of the uterine cervix and vagina: three cases. Fertil Steril 1980; 33:255. Dillon WP, Mudaliar NA, Wingate MB: Congenital atresia of the cervix. Obstet Gynecol 1979; 54:126