Conservative Treatment of Didelphyc Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis

Study Objective: To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina.Design: Stepwise demonstration of the technique with narrated video footage.Setting: In the context of obstructive congenital mullerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperfo-rate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenor-rhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adoles-cent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hema-tocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis.Interventions: A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal sep-tum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemi-uterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemiva-gina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disap-pearance of the hematocolpos can be clearly seen.Conclusion: Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction. Journal of Minimally Invasive Gynecology (2022) 29, 929. (c) 2022 AAGL. All rights reserved.