A Case Report on Hendren's Technique for Managing Symptomatic Isolated Urogenital Sinus in an Infant.

A 10-month-old baby girl presented with bladder outlet obstruction and pyocolpos. On examination, she had an isolated UGS with extra digits on all four limbs and a vaginostomy in the upper abdomen already done elsewhere at birth. Ultrasonography showed bilateral mild hydroureteronephrosis and pyocolpos. On catheterization, the vaginostomy drained turbid urine; symptoms recurred after accidental dislodgement of the catheter. Micturition-Cysto-Urethrogram (MCUG) showed no reflux, good capacity bladder, and high confluence UGS. Dimercaptosuccinic acid showed right cortical scar with 40% function. Magnetic resonance imaging (MRI) did not reveal any apparent communication of the vagina with bladder or urethra but suggested left ureteric communication with vagina. Cystoscopy revealed high UGS with a common channel >3 cm, the length of the urethra was <1 cm, the vaginal opening could not be cannulated, and only the right ureteric orifice was identified near the midline. Hence, a ureteric reimplantation of the ectopic left ureter was planned. During surgery, both the ureteric orifices were found to be normal in position and caliber. Hence, the bladder was closed with a suprapubic cystostomy and vaginostomy was revised to the lower abdomen to aid vaginal mobilization in future reconstruction. A vaginal reconstruction was planned 3 months later. Cystoscopy using 4Fr composite cystoscope confirmed high confluence UGS, scope negotiated through the vaginal opening snugly, and an attempt to place a 3Fr Fogarty catheter over a guidewire failed. So an alternative access through the vaginostomy was considered; a 1-0 Prolene suture was passed through it and delivered out through the UGS with a DJ-stent removal forceps passed through the cystoscope. An 8Fr Foley was successfully placed into the vagina tied to the Prolene suture guiding it across the UGS. The patient was kept in the lithotomy position in case a laparotomy is required for vaginal mobilization. An inverted U-shaped incision was made anterior to the anus, UGS was cut back to few millimeters, followed by half circumferential incision around the UGS [ Figures 1a and 2a ]. Anal pack was kept in the rectum for aiding dissection. Once the dissection reached the vagina, identified by palpating the already placed Foley’s bulb, the vaginal wall was opened at the most dependent position and vaginal mobilization was done with initial submucosal dissection to separate carefully from the confluence and the posterior wall of the urethra avoiding injury to the bladder neck region [ Figure 2b ]. Neo-urethra was reconstructed by securely closing the opening in the UGS [ Figure 2c ]. The right labial fat pad was developed inferiorly and interposed between the neo-urethral suture line and the mobilized vagina [ Figure 2d ]. Vaginal reconstruction was completed by raising perineal flaps and approximated to the end of the mobilized native vagina [ Figure 2e ]. The reconstructed urethra and vagina were stented with appropriately sized catheters. Preoperative and postoperative pictures. (a) Lines of incision (b) Postoperative follow-up picture showing separate (-) urethral and (=) vaginal orifices Line diagram of surgical procedure. (a) Lines of incision in red color (b) Lateral view - showing the plane of dissection to reach vagina and opening of the vagina at most dependent area (c) separation of UG sinus through the vaginal end (d) Labial fat pad taken from the lateral side to interpose between the sutured ends (e) Lines of skin closure from the lateral and posterior flaps Minimal wound dehiscence was noticed on postoperative day (POD)-5. Vaginal and urethral stents were removed on POD-10. During follow-up, vaginal mucosa was noticed to fully cover the defect and was admitting 12Fr dilator easily. At 6-month follow-up, she remains continent for bowel and bladder [ Figure 1b ].

Urogenital sinus (UGS) is usually associated with cloaca or in females with congenital adrenal hyperplasia (CAH). Isolated UGS is a rare anomaly and has a known association with renal and limb anomalies. We highlight the difficulties encountered while managing an infant with respect to the role of imaging in demonstrating surgical anatomy, catheterization during surgical repair, surgical technique to preserve urinary continence, and postoperative neovaginal stenosis.

Hendren’s procedure is a simple, effective, and relatively less invasive surgical technique with minimal complications for the repair of UGS with high confluence. The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Nil. There are no conflicts of interest.

In UGS, the preferred age for vaginoplasty is during adolescence if there are no urinary symptoms because after menarche under the influence of estrogen, the vagina enlarges and its tissue becomes thick allowing better manipulation during surgery; also patient can actively participate in postoperative vaginal dilatations.[ 1 ] An early repair may be indicated due to recurrent urinary complaints; it may also address psychological stress due to a deformed self-body image and the chance of endometriosis due to retrograde menstruation at adolescence. In our case, the following problems related to vaginostomy were also a major concern – wrongly sited in the upper abdomen, inadequate drainage, and long waiting period till adolescence. Detailed imaging is necessary to discern the surgical anatomy (the length of confluence and the proximal native urethra and vagina) and rule out associated malformations. Interestingly in our case, MRI misled us by suspecting an ectopic left ureter opening into the vagina. Management of UGS with high confluence is more challenging. The surgical options, to choose from, are vaginal pull-through, total UG mobilization, bowel vaginoplasty, and anterior sagittal or posterior sagittal transanorectal approach.[ 2 ] Total UG mobilization may cause neurogenic bladder and urinary incontinence. Bowel vaginoplasty is a major procedure with an increased risk of complications and morbidity due to mucosal discharge from bowel mucosa.[ 3 ] Anterior sagittal (Domini, De Castro) or posterior sagittal (Pena) approach involves a potential risk of damage to the rectum and anal sphincter requiring a diverting colostomy. Although successful in experienced hands, all these procedures may not be reproducible by everyone, and most of them are done in patients with CAH where feminizing genitoplasty is done along with vaginoplasty with the advantage of good availability of local tissues. Hendren described his procedure mainly for androgenital syndrome patients. The patient is placed in a lithotomy position in case a laparotomy is needed for further vaginal mobilization. The vagina is not encircled at the confluence but opened at the most dependent part and UGS is closed from inside the vagina and a labial fat pad is interposed following vaginal separation to prevent a recurrence. Instead of extensively mobilizing the vagina and causing damage to bladder innervation, labial flaps were folded inside to create a neovagina.[ 4 ] This concept was quite appealing and convinced us to do this procedure in our case. A revision vaginoplasty is advisable during adolescence for creating an adequate-sized vaginal orifice for intercourse.