Journal Archives - Volume 3
Absract: Collagen, smooth muscle, elastin and neural elements are the most important cellular and histological elements responsible for bladder function. Types I and III collagen provide tensile strength within the bladder. Type I collagen provide accounts for 90% of the baby's collagen and is the major collagen in the intermuscular bundle space. Type III is present in the smooth muscle of the detrusor and within the muscular bundle between individual muscle fibers. Also, during organogenesis the exstrophy bladder does not store urine and thus its requirements for contraction are limited. The innervations of these exstrophy bladders should reflect this difference in function compared to fetal bladders, which when filled and emptied may stimulate neural growth in utero.
Only 4 decades ago, H.J. Hewett proclaimed that it "was impossible to restore the bladder" in exstrophy patients. Now, as the century comes to an end, modern adaptation of classic staged reconstruction offers acceptable outcomes for appropriately selected patients when carried out by experienced surgeons. Implementation of the current approach frequently yields urinary continence with renal preservation and functional, as well as cosmetically pleasing, external genitalia. Newer techniques, such as combined bladder closure and epispadias repair, have shown some promise but await the test of time before a realistic comparison can be made with the thoroughly investigated modern staged approach. Additionally, experts in the field will face new challenges, such as the role of pre-natal diagnosis and the difficulties of dealing with a complex problem under the auspices of managed health care. Clearly, answers to theses questions will require the shared input of clinicians and researchers across different disciplines at a number of specialty centres.
Abstract: The closure of the abdominal wall remains a challenging aspect of the management of cloacal exstrophy. Improvements in cosmesis and function have been based on understanding the anatomy of the abdominal wall and pelvis as well as development of novel surgical techniques. Newer techniques hold promise for improvement in the quality of life for our patients.
Abstract: The surgical management of epispadias has gone through great advances in the last century. Significant improvements have come from better understanding of the anatomy of the exstrophy and the availability of better surgical materials (such as sutures and urethral catheters) and magnification systems. Surgeons of the last century who have been dedicated to the reconstruction of this birth defect (such as Dr. Robert Jeffs) have given those born with epispadias the chance to live much better lives than they would have had 100 years ago. The technical improvements in epispadias repair that will come in the next century will surely build on the ingenuity and experience reported to date and add to the legacy of Dr. Jeff's contributions.
Abstract: Adequate longitudinal psychosocial and psychosexual outcome study data on people with bladder exstrophy is largely lacking. Preliminary studies indicate that sexual function and psychosexual development is problematic, especially in males. Fertility potential often requires a surgical or medical catalyst. Exstrophy impacts psychosocial developmental behaviors in the parents and accentuates typical developmental hurdles in the children. A child psychiatrist as a member of the initial and ongoing treatment team adds valuable expertise in developmental, behavioral, adaptive, and family dynamics of bladder exstrophy.
Abstarct: The staged approach to bladder exstrophy reconstruction has enjoyed increasing success during the last two decades. Currently, the staged exstrophy reconstruction usually includes; bladder exstrophy closure soon after birth, the Cantwell–Ransley epispadias repair usually is done between 1–2 years of age and Young-Dees-Leadbetter bladder neck reconstruction at age of 3–5 years. Unfortunately, in significant number of patients failure do occur. Failures occur at the initial exstrophy closure, at the epispadias and penile reconstruction, and failure to achieve continence; all of which eventually affect the sexuality, fertility, self-esteem, sexual function and social integration. When primary exstrophy closure failed, one should accept the complication and let the wound and scar to mature for at least 4–6 months before re-closure is performed, since early intervention is simply meddlesome and prone to failure.
Abstract: Bladder exstrophy (BE) has remained a surgical challenge to paediatric urologists worldwide. Despite the wide use of urodynamics for neurogenic bladder dysfunction, it is surprising that very little work has been done to assess lower urinary tract function in BE patients. The surgical reconstruction of BE is bases on the premise that the bladder and reconstructed urethra are normal and by increasing outlet resistance urinary continence would be achieved. However, this presumption is an over simplification. The exposed detrusor is definitely abnormal at birth and may remain so after closure. The present article highlights the pitfalls and the advantages of urodynamic studies in Bladder Exstrophy.
Abstract: The complexity of the surgical challenge of repair of exstrophy is highlighted by the spirited debate as to whether repair should even be considered, as opposed to urinary diversion. The severity of the surgical obstacles to overcome is further highlighted by the fact that progress with the surgical management of exstrophy has only occurred in the last 50 years. Rather than presenting may bias as to the "State of the art" surgical technique, the authors have focused on the multitude of clinical factors which contribute to the delicate balance of continence, voiding and renal function in exstrophy. These propose is not to present neat and simple algorithms to the management of such a complex multivariate problem, but to outline evidence based outcomes when possible when possible while commenting on current limitations, so that future researchers efforts may be stimulated.
Abstract: In this article the authors have attempted to analyse the results of treatment of Bladder Exstrophy with or without the use of adjunctive methods. The results of staged reconstruction alone must be viewed separately from the results achieved by the use of additional procedures. In the 7 series reviewed it is evident that continence after staged reconstruction above, without the use of adjunctive procedures is difficult to achieve. The adjunctive procedures described play a vital role in achieving continence but are themselves with significant complications.
Abstract: In order to assess the success of the modern approach to staged reconstruction of bladder exstrophy and its effect on the upper urinary tract the author has analyzed the renal function of a select series of referred bladder exstrophy patients. These patients had their entire surgery performed by one of two senior surgeons including bladder and abdominal wall repair, posterior urethral closure, epispadias repair and bladder neck reconstruction.
Children born with the Epispadias/Bladder Exstrophy range of anomalies can be surgically corrected, the results of surgery being dependent not only on the original pathology but also on the subsequent surgery and the complications of surgery encountered. In children with the most common anomaly, Classic Exstrophy, the results of staged surgery using extensive soft tissue mobilization would indicate that over half will achieve normal or socially acceptable urinary continence in childhood. The long term outcome of this surgical program will need to be assessed.