Report of surgical correction of a cervicovaginal agenesis case: cervicovaginal reconstruction with pudendal thigh flaps

Congenital agenesis of the uterine cervix and vagina in the presence of a functional endometrium is an extremely rare mullerian anomaly [1]. Congenital vaginal agenesis occurs with a prevalence of 1/4000-1/5000 live-born females, while the accurate incidence of cervicovaginal agenesis is controversial [1-3]. Cervicovaginal agenesis is usually not diagnosed until menarche. It is manifested by the collection of menstrual blood in the functional endometrium, Fallopian tubes and peritoneal cavity, leading to primary amenorrhea and cyclic abdominal pain [1, 4]. The primary goal of cervicovaginal reconstruction in cervicovaginal agenesis cases is to preserve reproductive performance [5-8]. In routine clinical practice, hysterectomy was advised in cases with complete cervical aplasia, to control pain and dysmenorrhea and also prevent complications such as peritonitis [4, 9, 10]. But recent studies are more encouraging [3]. However, as different techniques were performed for each individual type of cervical agenesis case, there is great controversy as to the best technique because each has associated advantages and disadvantages. In this case report, a functional reconstruction with pudendal thigh flaps complicated with endometrioma and hematosalpinx in a cervicovaginal agenesis case will be discussed in the light of the literature. An 18-year-old virgin woman with a history of primary amenorrhea and cyclic abdominal pain was referred to our gynecology clinic with normal secondary sex characteristics. Adrenarche and thelarche had occurred by age 13 years. Physical examination revealed no abnormalities. The vagina ended with a 1 cm blind pouch. A pelvic sonographic examination showed an abnormally enlarged uterus due to hematometra and also a left ovarian endometrioma. No cervical tissue was observed in inspection or sonographic imaging, indicating complete cervical agenesis. The intravenous pyelogram showed a normal urinary tract system. Magnetic resonance imaging (MRI) revealed an arcuate uterus and a normal endometrial cavity with complete cervical agenesis (Figure 1). The laboratory work-up showed normal serum levels of follicle-stimulating hormone, thyroid-stimuCorresponding author: Remzi Abali MD Department of Gynecology and Obstetrics Faculty of Medicine Namik Kemal University 100. yil Mah. Barbaros Cad No: 132, Tekirdag, Turkey Phone: +90 282 2620130, +90 532 7315360 Fax: +90 282 2626810 E-mail: [email protected] Letter to the Editor