I would like to begin by thanking the scientific committee for allowing us to present a report entitled A Multidisciplinary Approach to Diagnosis and Management of Avira Syndrome and Rectal Prolapse. Our objective is to describe a complex female genital urinary tract anomaly with associated rectal prolapse and the utilization of MRI and a multidisciplinary surgical team necessary for successful diagnosis and management. Failure of the malaria ducts to fuse at approximately 9 weeks gestation leads to a variety of congenital anomalies ranging from septate uterus to complete uterine didelphys. The true incidence is unknown as most patients are asymptomatic but is estimated to range from 0.1 to 3.8%. Uterine didelphys represents 11% of malaria anomalies manifesting as two complete hemi uteri with two cervices fused at the lower uterine segment and often a duplicated vagina with either a partial or complete longitudinal septum. A variant of uterine didelphys is associated with a transverse vaginal septum and an ipsilateral renal anomaly. This is termed Avira Syndrome. The most commonly described anomaly is renal agenesis, however, ureteral and renal ectopy and renal dysplasia have also been described in the literature. Many patients remain asymptomatic but obstruction of menstrual flow by the vaginal septum can lead to cyclic or non-cyclic pain or an abdominal mass on exam. Ureteral ectopy occurs when the ureteral butt arises off an abnormal location from the Wolfian duct. This occurs more frequently in females than males with a ratio of 3 to 1. The most common presenting complaint is persistent urinary incontinence after toilet training. Many patients, however, remain asymptomatic and the true incidence of ectopic ureter is unknown. Avira Syndrome can be correctly diagnosed with a combination of a good history and physical as well as appropriate imaging. The challenge lies in diagnosing the renal anomaly. This can be accomplished using intravenous pylography, MRI, or ultrasound. While ultrasound has been described in the correct identification of renal anomalies, MRI often provides better anatomic resolution. Our patient is a 25-year-old G0 presenting with a history of activity associated urinary incontinence with incomplete bladder emptying, recurrent UTIs, and malodorous vaginal discharge. The patient was also complaining of fecal incontinence. Her only significant medical history was hyperthyroidism. She reported regular non-painful menses and denied sexual activity. Two years prior to coming to our institution, the patient had an outside evaluation which demonstrated uterine didelphys, rectal prolapse, and right renal agenesis. On our physical exam, she had an intact hymen, a suburethral fullness measuring 3 by 3 centimeters, and a palpation of only a single cervix. Her history and physical were otherwise unremarkable. Evaluation at our institution included examinations by a urogynecologist, colorectal surgeon, and a urologist. Multi-channel urodynamic studies demonstrated normal voiding function and normal bladder capacity. Colonoscopy and a sitzmarker study revealed no abnormalities. Pelvic MRI again showed uterine didelphys, but a hypoplastic right kidney with an ectopic ureter inserting into the right hemivaginal wall was demonstrated. A nephrogram was performed and demonstrated only 15% remaining function within the right kidney. This is a coronal view of an MRI through the patient's pelvis demonstrating a pelvic kidney at the right lateral border of the L5 S1 vertebra with an ectopic ureter extending into the obstructed right hemivagina, which is distended with fluid. Uterine didelphys is clearly demonstrated. A 3D reconstruction view of the same patient demonstrates better the uterine didelphys with a communication between the right hemivagina and ectopic ureter. The final diagnosis was of ovarian syndrome with renal dysplasia and ectopic ureter insertion into the right hemivagina with rectal prolapse. This constellation of findings has not been previously described in the literature. After appropriate counseling, the patient underwent a robotic laparoscopic rectopaxy with robotic right nephro-ureterectomy and resection of the vaginal septum. We begin with identification of both hemi-uteri and the bilateral with NEXA. These appear normal. The left ureter is easily identified. The right ureter is not clearly identified. Numerous anomalous vessels are seen in the right pelvis and in the midline. We begin by dissection of the mesorectum in order to mobilize the rectum. Once mobilization is complete, we review the MRI to identify the location of the right pelvic kidney. We begin retroperitoneal dissection at the border of the L5-S1 vertebra on the right. Kidney dissection reveals numerous anomalous vessels in the retroperitoneal space. These are in close proximity to the right common iliac vessels. These vessels are carefully ligated using thermal energy and surgical clips. The dysplastic kidney is identified by following the anomalous vessels proximally. Once identified, it is dissected from the retroperitoneal fascia and lifted out of the retroperitoneal space. The right proximal ureter is identified. More anomalous vessels are seen and are ligated. We continue with dissection of the ureter to its distal-most insertion point in the right hemivaginal wall. We then turn our attention to completion of the rectopexy suturing the mesorectum to the anterior longitudinal ligament with 2-O-Tychron. We then return to completion of the ureteroectomy. The ureter is transected from its distal insertion into the right vaginal wall. The surgical site is closed using 3-O-Vicrol. Attention is then turned to resection of the vaginal septum. This is done by incising the septum using a scalpel and then completing the dissection with metambalm scissors. This is the post-surgical appearance demonstrating 2-Patent Cervical Ossus. The surgical site is closed using 2-O-Vicrol suture. At her 4-month follow-up visit, the patient reported complete resolution of her symptoms. In conclusion, we demonstrate a previously undescribed association between a virus syndrome with ectopic ureter and rectal prolapse and the success of a multidisciplinary approach for diagnosis and management. I thank you for your attention and welcome any questions you may have.

Avira Syndrome

diagnosis

management

rectal prolapse

female genital urinary tract anomaly