Simultaneous Endoscopic Management of Urethral Duplication and Postposterior Sagittal Anorectoplasty Urethral Diverticulum in a Boy with Anorectal Malformation.

We report a case of urethral duplication with high anorectal malformation (ARM). A 12-year-old boy who had undergone transverse colostomy on day 2 of life and posterior sagittal anorectoplasty (PSARP) for ARM (rectobulbar fistula) at 8 years and also had urinary dribbling from a midscrotal opening in addition to normal voiding, presented with recurrent urinary tract infections and dysuria. Midscrotal dribbling had continued postoperatively. Colostomy had been closed 3 months after PSARP. Investigations revealed a large urethral diverticulum at the site of rectourethral fistula due to a segment of the gut being left behind during fistula closure. In addition, he had a sagittal urethral duplication originating at bulbar urethra and opening externally at midscrotum. The highlight of this report is successful endoscopic management of both the urethral duplication and the diverticulum. Copyright:

INTRODUCTION

“Y-”shaped urethral duplication is generally defined as two urethrae originating from a common bladder neck with the ventral urethra opening in the perineum, anterior to rectum, and the dorsal urethra being orthotropic but hypoplastic.[1] Ventral channel is the better functional channel. However, there are instances where ventral urethra is less functional than the dorsal orthotopic urethra.[2] Wagner et al.[3] have suggested the term “congenital urethroperineal fistula” (CUPF) for such malformations mainly due to the presence of transitional epithelial lining of the “fistula,” hence suggesting its origin from the urinary tract. Bello[4] has suggested modifying the Effmann classification to include CUPF as a “Type II A2, Y-hypoplastic ventral urethral defect.” Association of urethral duplication with midline defects and VACTERL complex as has also been described.[5]

We report a case of urethral duplication with high anorectal malformation (ARM). Besides the preexisting duplication, he had also developed as a complication of posterior sagittal anorectoplasty (PSARP), a urethral diverticulum at the site of rectourethral (bulbar) fistula. The highlight of this report is the successful endoscopic management of both the urethral duplication and the diverticulum.

CASE REPORT

A 12-year-old boy, who had been born with imperforate anus and had undergone colostomy on day 2 of life followed by PSARP at 8 years of age elsewhere, presented with recurrent urinary tract infection (UTI) and dribbling of urine from an opening in the midscrotum. Detailed history revealed that he was born at term through normal vaginal delivery and had no anal orifice. He had a history of meconuria. He had undergone a transverse loop colostomy at birth. Thereafter, he did not receive any treatment till 8 years of age. No distal washes were given. Since early infancy, the parents had also noticed him passing few drops of urine from midscrotal opening while passing urine through normal meatus at glans. Evaluation at the age of 8 years had revealed a large fecaloma in the rectum that led to closure of transverse colostomy, removal of the fecaloma, and divided sigmoid colostomy. Subsequent cologram revealed a rectourethral fistula, and he underwent PSARP at 8 years of age. Colostomy was closed after 3 months. Subsequently, he developed recurrent UTI and dysuria. The dribbling from midscrotum while micturating continued. He presented to us at 12 years of age with these complaints. Evaluation revealed satisfactory neoanus with adequate caliber, good anocutaneous reflex, and no prolapse. The scrotum was partially bifid with a well-defined opening in midline raphe through which he passed few drops of urine while voiding. There was no scarring in the scrotum [Figure 1a]. His phallus was normal with external meatus of normal caliber at glans. Testes were scrotal. The abdomen was soft, no fecaloma. Scars were seen in the right upper abdomen and left iliac fossa. Ultrasound revealed normal bilateral kidneys. His blood profile was normal. Urine showed 10–15 pus cells/HPF and grew Escherichia coli on culture. Following treatment of UTI, Micturating cystourethrogram (MCUG) was done [Figure 1b], and he underwent Examination under anaesthesia (EUA) and cystoscopy. Through the midscrotal opening, a guidewire was passed which was seen to enter the midbulbar urethra on urethroscopy [Figure 2]. In the proximal bulb, there was a large diverticulum in the sagittal plane. Its opening was small but enough to allow the scope to enter the large cavity of the diverticulum [Figure 3a]. There were a few pus flakes, which were washed. The mucosal lining of the diverticulum appeared like intestinal mucosa with folds, indicative of left over intestinal mucosa during fistula closure, and PSARP. Using a Bugabee electrode, the intestinal mucosa of the diverticulum was ablated using coagulation current. The mouth of the diverticulum was widened [Figure 3b]. The procedure was repeated 3 months and 5 months later. In the third setting, the diverticulum had significantly shrunken, and no gut mucosa was visible. In the same sitting (third), the midscrotal duplication was mobilized to corpus spongiosum through a vertical scrotal incision. A Venflon (No. 16) was passed through it and seen to enter the bulbous urethra on urethroscopy. As soon as the tip of the Venflon was visible in the bulbar urethra, the plastic part of the cannula was removed leaving the metallic needle in the duplication. The mucosa of the duplication traversing the corpus spongiosum was burnt using cautery. The procedure was guided through the scope in urethra to ensure no damage to mainstream urethra. A silicone catheter was passed per urethra. Scrotal midline incision was closed after correcting bifid scrotum. The catheter was retained for 3 weeks. Following catheter removal, he passed urine in a single stream. He has remained well and infection free in 5-year follow-up.

Figure 1

(a) Clinical photograph on table showing ureteric catheter in midscrotal opening with no scarring around it suggestive of congenital origin. (b) MCUG with representative sketch diagram. 1: Posterior urethra, 2: Impression of the diverticulum on the urethra, 3. Duplication

Figure 2

Endoscopic view with illustrative sketch 1: Veru 2: Opening of the diverticulum (site of fistula) 3: Internal opening of the duplication 4: External opening of the duplication. A cannula has been passed through the duplication into the diverticulum

Figure 3

(a) Endoscopic view showing opening of the duplication (3), narrow opening (fistula site) of the diverticulum (2) and veru (1). (b) The diverticulum has been marsupialized (2) and the internal mucosa cauterized

DISCUSSION

Association of urethral duplication and ARM is uncommon. Haleblian et al.[6] described ten cases of “Y” urethral duplication, of which seven had ARM – three with high anomalies, one anteriorly placed anus, and three with anorectal stenosis.

Effmann et al.[1] have given the most commonly used classification of urethral duplication. A “Y-”shaped urethral duplication in the sagittal plane – two urethrae originating from a common bladder neck are the most common. The ventral urethra which frequently open in the rectum is the better functioning urethra with the dorsal urethra being rudimentary or hypoplastic, although it is located in the normal location on the glans. In our case, however, the anomaly appears to be Type IIA 2 “Y” type with the dorsal urethra normal. The site of internal opening has varied from the bladder neck to posterior urethra.[134] In our case, it was at the proximal bulbar urethra. The term “congenital urethroperineal fistula” has also been used to describe duplications with a normal dorsal urethra.[3] However, most authors have preferred a more unified classification, i.e., Effmann et al. classification for such description as was found in our patients.[347]

When it comes to treatment, it is important to correctly identify the more normal urethra so that it can be saved, and only the less dominant urethra is excised. Therefore, excision of the ventral duplication is the treatment in cases like ours.[34] Sakaguchi et al.[8] managed CUPF by giving circular incision around the opening, and the dissection was performed along the fistula. The fistula was ligated in the vicinity of the prostatic urethra. Bedir et al.[9] reported an alternative endourological intervention, injection of a bulking agent around the internal orifice of a fistula, for the treatment of isolated CUPF if the tract was hypoplastic.

The urethral diverticulum at more proximal level in our case seems to be related to left over intestinal mucosa at the site of fistula during PSARP. Such a complication is well reported.[10] Possibly, it was a difficult pull through, and the operating surgeon closed the fistula much proximal to the urethral opening of the fistula, thus leaving a significant portion of the intestinal mucosa attached to the urethra.

Different approaches to deal with symptomatic posterior urethral diverticulum have been described including excision through posterior sagittal approach[10] and laparoscopic/robotic excision.[11]

Our case represents a unique aspect in that the urethral diverticulum and the urethral duplication were treated together endoscopically. It took us three sittings of endoscopic cautery ablation of the diverticular mucosa because it was a large diverticulum. Further, we had to be very careful, especially at the posterior wall, which was very close to the pull-through rectum. Any overdoing in that region could have led to a rectourinary fistula.

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Conflicts of interest

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