Uterine Isthmus Agenesis with Inverted Uterine Body and Malrotated Kidney.

Overview

A 21-year-old with a history of cyclic abdominal pain beginning at age 13 years and a previous diagnosis of a complex müllerian anomaly presented with abdominal pain and a finding of a right distended hemiuterus, left hematosalpinx, and cervix separate from the uterine body. After laparoscopic decompression for symptomatic relief at that time, she presented to our center for definitive management. After a diagnostic vaginoscopy and laparoscopy confirmed the diagnosis of uterine isthmus agenesis, an abdominal approach to uterocervical anastomosis was planned and undertaken. At the postsurgical clinical follow-up, the patient reported normal menses and resolution of pain, and imaging confirmed maintenance of anastomotic patency. The diagnosis and classification system of müllerian anomalies are complex, and few reports detail the management of rare müllerian anomalies. In this case report, the successful anastomosis of the uterus and the cervix is reviewed as an approach that can restore normal menses successfully and safely and potentially allow for future fertility in patients with uterine isthmus agenesis.