Duplicate Bladder Exstrophy with Hypospadias: A Rare Exstrophy Variant with Unusual Association.

Sir,

We report a case of duplicate bladder exstrophy that presented with the appearance of a classic bladder exstrophy. The actual condition was recognized only at the time of surgical exploration when he was diagnosed as duplicate bladder exstrophy. It differed from those reported previously in that, not only it grossly appeared as a classic exstrophy-epispadias complex but was also associated with a hypospadiac urethral opening. This urethral opening was draining the underlying normal bladder located posterior to the exstrophic bladder patch.

Our case was 2 months male infant presented with apparent features of a classic bladder exstrophy-epispadias [Figure 1a]. Physical examination revealed an exstrophic bladder plate of 5-7 cc in capacity and an epispadiac phallus with ventral prepucial hood and an opening on the ventral aspect of phallus which was assumed as a blind pit. Diastasis of the pubis and divergence of rectus abdominis muscle were noted, and both the testes were in their normal scrotal position. Serum creatinine level was normal. Abdominal ultrasound revealed bilateral normal kidneys. The infant was taken up for surgery for bladder closure (primary turn-in) with the diagnosis of classic bladder exstrophy. During surgery, it was not possible to locate the ureteric orifice. Hence, a decision was made to go ahead with the primary turn in without intubating the ureter. The exstrophic bladder plate was mobilized with a circumscribing incision. It was then noticed that the underlying ureters were draining into a subjacent covered bladder [Figure 1b]. On pressing the subjacent duplicated bladder, urine coming from an opening on the ventral aspect of the phallus was noticed. Numerous attempts to cannulate this opening failed. On the table, a diagnosis of duplicate bladder exstrophy with hypospadias was made. The exstrophic plate was excised, and the rectus abdominis muscles and fascia were approximated in the midline [Figure 1c]. The hypospadias repair was planned to be assigned at a later age in follow-up.

Figure 1

(a) Preoperative clinical photograph of duplicated bladder exstrophy mimicking classical exstrophy epispadias complex, (b) intraoperative photograph of duplicate bladder exstrophy with a feeding tube in the hypospadiac urethral opening draining the normal bladder(I), the exstrophied bladder mucosa (II) and the underlying covered normal bladder (III)

Exstrophy variants are rare and comprise 10% of all exstrophy-epispadias complex.[1] The major exstrophy variants include superior vesical fissure, covered exstrophy, pseudoexstrophy, and duplicate bladder exstrophy.[2] Duplicate bladder exstrophy refers to the suprapubic exstrophic mucosal plate with a subjacent covered bladder is an extremely rare congenital anomaly.[2] Two forms of duplicate bladder exstrophy have been described by Lowentritt et al., Bouhafs et al. and Sheldon et al.[123] The first type known as the anteroposterior form is associated with a suprapubic exstrophic mucosal plate and a subjacent covered bladder. The ureters attach to the closed bladder rendering the superficial mucosa dry. The surgical approach consists of excision of the exstrophied mucosal patch with the plastic reconstruction of the abdominal wall. The second type of true duplication with exstrophy of bladder is the collateral form. In this type, there is side by side duplication with one ureter traveling to each bladder is less common. The duplicate organ can be either excised or used to augment the remaining one. Only three cases of collateral bladder duplication with exstrophic bladder have been reported.[2] The outcome in terms of continence is better especially for the anteroposterior type and can be obtained in most of these patients. The prognosis in terms of renal function is more of concern requiring long-term follow-ups.[4] An urodynamic study and a vesico cystourethrogram are recommended to eliminate the possibility of postclosure vesicoureteric reflux, especially in patients who had a communication between the exstrophic remnant and the intact bladder. The presence of a fluid filled cavity behind the exstrophied bladder patch along with the inability to identify the ureteric orifice at exploration should raise the possibility of duplicate bladder exstrophy.

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