Patent urachus with double urethra and testicular teratoma: A rare association.

In this paper, we aim to highlight a case report of a rare association of anomalies comprising patent urachus, double urethra and development of testicular teratoma.

INTRODUCTION

Congenital patent urachus associated with urethral duplication is a very rare association. There are only two such cases in the English literature. We are reporting one such case, in which the patient also developed testicular teratoma during follow up.

CASE REPORT

A seven day old male baby born at 39 weeks of gestation to a primi mother with a birth weight 2.45 Kg was brought for not passing urine since birth. After the umbilical cord stump had fallen, on the 5th day the mother noticed soakage of clothes and flow of urine from umbilical region for two days. On examination, an intermittent stream of urine was coming from the inferior margin of the umbilicus suggestive of patent urachus. On palpation, abdomen was soft with no mass palpable. Genital examination showed normal size penis and scrotum with descended testes. There were two urethral openings - one at the tip of penis and another at the penoscrotal junction without associated chordee. The urethral meatus at the tip of penis (dorsal) was admitting a 3F ureteric catheter which could be passed only upto 5 cm length whereas the meatus at the penoscrotal junction (ventral and hypospadiac) was admitting a 6 size infant feeding tube and giving access to the bladder as shown in Figure 1. An improvement in the urinary stream from the hypospadiac ventral opening and a reduction in urine leak from patent urachus were noticed after the urethral calibration.

Figure 1

Catheters passed into the bladder through two urethral openings and patent urachus. (The urethral meatus at the tip of penis (dorsal) was admitting a 3F ureteric catheter whereas the meatus at the penoscrotal junction (ventral and hypospadiac) was admitting a 6 size infant feeding tube giving access to the bladder)

An ultrasonogram of abdomen confirmed a patent urachal tract from the umbilicus to the bladder with normal kidneys. A retrograde urethrogram (RGU) was done by injecting contrast (Urograffin) through the two urethral openings. The two tracts for urinary outflow were made out in the RGU as shown in Figure 2. The dorsal urethra ending at the tip of penis was very narrow, irregular and stenosed at few sites with some dilatation near the bladder neck. The ventral urethra opening at the penoscrotal junction was of good caliber and relatively smooth. Both the urethrae were having separate origin from the bladder, which was confirmed by an isolated imaging study for individual urethra. Thus, diagnosis of a patent urachus associated with a complete and hypospadiac urethral duplication with normal kidneys and ureters was made. Cystoscopy could not be done as we were lacking such a small sized cystoscope. A good urinary stream through the hypospadiac ventral urethra, confirmed by saline infusion via the patent urachus, prompted us to close the patent urachus.

Figure 2

Retrograde urethrogram showing two separate urethra

The abdomen was opened under general anesthesia through a small Pfannenstiel incision. The patent urachus was identified with the guidance of in situ feeding tube, transfixed and excised. Post-operatively, the bladder was drained through the hypospadiac opening for 7 days. There were no postoperative complications. Urethral reconstruction was planned for the baby at the age of 2 years. At follow up, the baby was passing few drops of urine from the tip of penis and a good stream from ventral urethra.

Three months after the surgery, he developed left side hard testicular mass measuring 1.5 cm × 1.5 cm arising from the lower pole. An ultrasonogram showed mixed echogenic heterogeneous mass. Serum Alpha feto protein and beta human chorionic gonadotrophic (HCG) hormone were normal. Left side high orchiectomy and testicular prosthesis insertion was done. The biopsy of the testis showed features of teratoma.

In two years follow-up he was doing well and hence submitted for urethral reconstruction. Cystoscopy done via hyposapadiac ventral urethra showed normal caliber urethra with no communication with the other urethra. The verumontanum was not present. The dorsal urethra was admitting only 3 F ureteric stent up to the penoscrotal junction and the 9.5 F cystoscope could not be passed even after dilatation. Since the dorsal urethra was good caliber at least from penoscrotal junction to tip of penis and its course towards bladder was irregular and stenotic, we planned for end to side urethro-urethrostomy of the external end of ventral hypospadiac urethra to the side of the ventral urethra. The ventral urethra was mobilized and the corpus spongiosum and dorsal urethra was slit opened in the midline at penoscrotal junction. The end of ventral urethra was anastomosed to the side of dorsal urethra by interrupted stitches with 6-0 PDS suture with a 3 F ureteric stent in situ across the anastomosis. Thus a ‘Y’ shaped urethra was created which will be having good urine flow from the bladder via the ventral urethra and exiting via the dorsal urethral meatus at the tip of penis. The stent was removed on 7th post operative day and there was no fistula. He is passing urine in good stream and continent.

DISCUSSION

Urethral duplication is a rare congenital anomaly.[1] Patent urachus is a communication between the dome of the bladder and the umbilicus. It results when there is a persistence of allantoic remnant which normally gets obliterated during embryological development. It is often associated with lower urinary tract obstruction like posterior urethral valve, urethral atresia etc.[2] There are only two case reports of urethral duplication with an associated patent urachus in the English literature. There is no case report of such a case developing testicular teratoma.

In urethral duplication, one urethra will have a normal course and the other remains as an accessory. Embryology of urethral duplication is unclear and many hypotheses have been proposed which include ischemia, abnormal Mullerian duct termination, growth failure of the urogenital sinus etc.[13] Widely accepted classification for male urethral duplication is by Effman.[4] Blind incomplete urethral duplication is encountered more frequently than other types of urethral duplication in clinical practice. A similar case of urethral duplication with posterior urethral valve involving the ventral urethra is described by Ramanujam et al.[5] The dilated part or abnormal angulation at the origin of the urethrae from the bladder neck may theoretically cause urinary outflow obstruction which can lead to patent urachus.

The embryological background for the coexistence of urethral duplication with patent urachus is difficult to explain. We believe the hypotheses of Vaos[6] that both anomalies are due to early insult to cloaca development needs further confirmation. Patent urachus may be due to lower urinary obstruction,[7] and the double urethra may be a separate embryological error. Differential diagnosis includes acquired fistulous tracts (post infective and post-instrumentation), urethral diverticula and dilated Cowper's gland. Indications for surgery in double urethra include annoying symptoms such as a double stream, urinary incontinence, urinary obstruction or associated genitourinary anomalies. Treatment for urethral duplication should be individually tailored. An endoscopic urethrotomy and a distal septotomy are advised. For Y-type duplication with normal and continent dorsal urethra, perineal excision alone can be successful,[389] and if the dominant urethra is ventral, progressive augmentation by dilatation of the urethra can be done; and, if still unsuccessful a multistage urethral reconstruction is necessary like posterior hypospadias. Double urethra cases with one urethra ending in seminal vesicles or vas deference can develop epididymo-orchitis which can mimic testicular tumor.

To conclude, double urethra is a very rare anomaly with varied presentation and management. An associated anomaly needs to be addressed and treatment is tailored to the individual. Double urethra with patent urachus may be isolated anomalies. Its association with testicular teratoma is not yet described.