The Curious Case of the Unknown Müllerian Anomaly скачать в хорошем качестве

The Curious Case of the Unknown Müllerian Anomaly

Here we present the Curious Case of the Unknown Müllerian Anomaly and resection of an isolated ureteric remnant. First we review our case and the OHVIRA syndrome, then we present an approach to complex Müllerian anomalies and to surgical resection of a symptomatic ureteric remnant. Our patient was a 36-year-old G0 female with a known solitary left kidney and a history of unknown uterine reconstructive procedure. She had multiple emergency visits for purulent, copious vaginal discharge, abdominal pain, and fever. This was treated as PID with antibiotics without improvement, though the discharge resolved with oral contraceptive treatment. On exam she had one cervix draining copious purulent discharge and no vaginal septum. A pre-reconstructive MRI showed a didelphys uterus with a short upper vaginal septum. The distal aspect of the right ureteric remnant, ipsilateral to the renal agenesis, connected to the upper right endocervical canal. Post-reconstructive MRI showed interval resection of the muscular uterine midline division and findings suggestive of OHVIRA syndrome, including an obstructed right hemivagina, absent right kidney, and a persistent ectopic right ureteric remnant. Haemorrhagic contents filled the remnant, possibly explaining the discharge. CT urogram showed a serpiginous cystic tubular structure terminating in the cervix. The left kidney and ureter were normal. Given these findings we considered two diagnoses: OHVIRA syndrome after unknown uterine reconstruction with a ureteric remnant draining into the endocervix or hemivagina, or a previously complete septic uterus after partial resection with an isolated ureteric remnant draining into the endocervix or vagina. OHVIRA stands for Obstructed Hemivagina and Ipsilateral Renal Agenesis and presents with a didelphys uterus, unilateral obstructed hemivagina, and ipsilateral renal agenesis. When faced with complex Müllerian anomalies, first perform uterine and renal imaging to assess for agenesis, uterine morphology, and number of cervices. A thorough vaginal exam is needed to assess the introitus, septa, and number of cervices. In the operating room, vaginoscopy and hysteroscopy help evaluate the cervix and uterine cavity. Here we observed one cervical canal leading to one uterine cavity with both tubal ostia visualised and a remaining longitudinal uterine septum at the fundus, making OHVIRA less likely. Laparoscopy allows assessment of uterine fundal contour and tubal patency. In this case the fundus was normal and bilateral tubal spill was noted with hysteroscopic distension fluid. These findings ruled out OHVIRA and confirmed a previously resected uterine septum. The right ureteric remnant was visible in the retroperitoneum and did not vermiculate. For resection of the isolated ureteric remnant, the retroperitoneum was opened to access the perirectal and perivesical spaces. Ureterolysis was performed starting at the pelvic brim and carried caudally. Because the right ureter appeared normal, intraoperative urology consultation confirmed right renal agenesis and agreed with resection of the ureteric remnant. The ectopic ureter was dissected above the pelvic brim by extending the peritoneal incision cephalad. Its serpiginous course was followed 5 to 7 cm above the pelvic rim. To delineate insertion of the remnant, a ureterostomy was performed and purulent material noted. A guidewire and ureteric stent were placed, then ureterolysis was completed caudally. The ureteric remnant coursed through the parametria. The stent could be palpated vaginally but was not seen by hysteroscopy or vaginoscopy. The uterine artery was identified and isolated from the ureteric remnant, which was then resected to its base at its insertion to the vagina. This case reminds us that Müllerian anomalies do not always follow the textbook. Examination under anaesthesia is useful when history, imaging, and physical findings disagree. Clinicians must remain alert to discrepancies and consider a broad differential. A multidisciplinary approach is strongly recommended. The patient’s persistent purulent vaginal discharge resolved after the procedure.