The management of bladder exstrophy: Indian scenario.

Till almost half a century ago, it was believed that the bladder could not be functionally restored in patients with bladder exstrophy. Since then, the surgical treatment for this condition has made tremendous advances. Today, it is possible to not only close the bladder and repair the abdominal and pelvic defect but also restore the storage capability of the bladder and achieve functional voiding. Permanent urinary diversions, e.g. ureterosigmoidostomy, are now considered as a thing of the past.

The recognition of the spectrum and extent of the deformity led to the concept of staged repair of bladder exstrophy. This has many variations but revolve around the closure of the bladder, bladder neck repair and epispadias repair in different stages with or without pelvic osteotomy. The timing of surgery has also undergone a sea change. During the early times when surgical repair was being standardized, the bladder was not closed until one year of age and was often preceded by bilateral posterior iliac osteotomy. Now it is common to repair bladder exstrophy in the newborn period.

India is a vast country with an exploding population. Although there is no dearth of clinical material, the progress and expansion of pediatric surgery and pediatric surgical centers have not kept pace with other surgical specialties. Consequently, advanced centers and training centers are limited in numbers. Thus complicated surgical problems and those with unpredictable results tend to gravitate to specific centers. Possibly, this is the reason why the expertise for surgical management of bladder exstrophy is restricted to a few major centers, or so it seems. A search of the indexed English literature has shown up very few publications. From recent presentations in National Conferences of the Indian Association of Pediatric Surgeons and other meetings of Pediatric Surgeons, it is estimated that comprehensive management of bladder exstrophy is being provided in Chennai, Vellore, Bangalore, Mumbai, Pune, Udaipur, Chandigarh, Lucknow, Varanasi and Delhi. This is not to say that surgery for bladder exstrophy is not being done elsewhere; it is neither being presented nor reported! Outstanding contributions for the comprehensive management of bladder exstrophy have been reported from Delhi, Vellore, Mumbai, Pune and Lucknow. Different approaches have been and are being practiced.

An early report (1974-1985) from AIIMS, New Delhi[1] suggested that the surgical repair should be carried out early but after the neonatal period. The repair was invariably staged. Posterior iliac osteotomies were used for wide gaps and paraexstrophy flaps were used for urethral lengthening. Permanent diversion (employing ureterosigmoidostomy, ileal conduit and colonic conduit) was a very viable option in cases with failure of primary closure. Subsequent years have seen the evolution of approaches and techniques. Pelvic osteotomy has been given up and a technique for abdominal wall closure was developed by detaching the recti and re-anchoring them to the pubic rami after approximation in the midline.[2] Simultaneously, an effective technique for bladder neck repair was reported[3] and bladder augmentation was added to options for improving continence and preserving the upper tracts.[4] Paraexstrophy flaps have also been largely given up because they create problems for self catheterization. Currently, a one stage total repair in the new born period is also being practiced.[5]

A similar evolution of approach and technique has been reported from Vellore.[6] The practice of urinary diversion has been given up and the repair is effected by a staged functional reconstruction. However, augmentation cystoplasty and a catheterisable stoma with or without bladder neck closure are considered to be the preferential options because it is believed that the native bladder is not a very useful storage organ and upper tract deterioration is common after staged reconstruction.

However, ureterosigmoidostomy is still being practiced in some centers along with bladder preserving exstrophy repair and bladder augmented exstrophy repair as the other options.[7] A better quality of life has been reported in patients undergoing ureterosigmoidostomy because they are dry. Total cystectomy and permanent diversion have also been reported to be the better options in patients with failed repair.[8] Elsewhere, a technique for transverse closure of the bladder along with anterior iliac osteotomies was developed and reported to have improved results.[9]

A new hypothesis for the embryogenesis of bladder exstrophy has been proposed from Mumbai and based on this concept a new technique has been described.[10-12] This technique transfers skin between the penis and scrotum in males and between the clitoris and labia majora in females into a superior position for a better repair of the perineum. Another major input in the reconstruction of bladder exstrophy has been reported from Lucknow. New techniques for abdominal wall closure without pelvic osteotomy and for the construction of an umbilicus have been described.[1314] A complete one stage repair of the exstrophy with use of the muscle stimulator for identification of muscles of the pelvic diaphragm has also been described from this center with good results.[15] Reliable anterior pubic fixation has been reported to be of great importance in the successful urological outcome in exstrophic anomalies. For this, a new three-loop technique for anterior pubic fixation has been reported from Mumbai.[16]

A number of live operative workshops featuring repair of bladder exstrophy and epispadias have been organized in Delhi, Lucknow, Kochi, Hyderabad etc. Experts from India and overseas have demonstrated their skills and innovative techniques. In addition, there have been numerous case reports on rare varieties of bladder exstrophy and exstrophy variants and the innovative methods of treatment of such condition (these are all available on the internet-based literature searches). Thus, pediatric surgeons in India have demonstrated exceptional skills in the surgical repair of the exstrophy–epispadias complex. However, there is a paucity of data on what happens to these patients after the initial repair. The reasons for this may be many and this may not be the appropriate forum to discuss this. From the available reports in literature, it can be concluded that the postoperative period is complicated by numerous and variable events – infection, dehiscence, upper tract dilatation with deterioration, fistulas, stone formation and incontinence to name a few of the major complications. Redo surgery for bladder closure, bladder neck reconstruction, epispadias repair and closure of fistulas are frequently required.

Although a number of authors/centers are practicing the surgery for bladder exstrophy, very few have reported long-term follow up. The plight of these patients forces them to visit hospitals repeatedly; if long-term follow-ups are not being reported then it suggests that either there are flaws in documentation or the results are not worth reporting. Publications and presentations on aspects beyond the operative techniques have come from very few centers.[6717-21]