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The various methods of managing bladder exstrophy are described. Bladder reconstruction and ureterosigmoidostomy are discussed and evaluated. The satisfactory results obtained with 21 patients treated by the Heitz Boyer-Hovelacque operation are reported and it is concluded that this is the procedure of choice when urinary diversion is needed for the exstrophic bladder.
Lower hindgut duplications, although very rare, usually present as a cystic mass located posterior to the rectum and confined to the pelvis. Hindgut duplications are often associated with genitourinary or spinal anomalies. We describe a neonate with a previously unreported heterotopic colonic duplication presenting as an exophytic perineal mass associated with classic bladder exstrophy. The embryonic significance of these anomalies is discussed.
The different methods of treatment of bladder exstrophy are described. Ureterosigmoidostomy and its various modifications are discussed and the longterm result critically reviewed. Urinary diversion by transplanting the ureters into an isolated segment of ileum or colon should, in the authors' opinion, only very rarely be necessary. Cutaneous ureterostomy is used only as a last resort in incontinent patients with severe renal damage. The greatest single advance in the management of the malformation has been the two-stage closure of the exstrophic bladder. The authors' experiences in the two-stage closure of 28 patients with ectopia vesicae are described. No selection of patients was practiced. The results obtained, both clinically and by manometry of the operated bladder, are reviewed. The results are moderately satisfactory.
The authors present the case of a 39-year-old male patient, married and assumed to be the father of 4 children, who presented with bladder exstrophy. The patient, living in Mali, never came to consultation for this disease. The review of the literature allows defining the consequences for adults of this condition, which is now operated in childhood in most cases.
Posterior pelvic osteotomy has not been a satisfactory operation to provide penile length in the repair of defects associated with bladder exstrophy. The authors are proposing a new technique based on the movement of the halves of the symphysis and pubic rami (en bloc with the attached corpora cavernosa) to the midline. This is accomplished by osteotomies of the superior and inferior rami and bone grafting of the resulting defects in the superior rami only. The hip joints are not disturbed, risk of complications appears to be reduced, and increased effective penile length is obtained. Cadaver dissections confirmed the practicality of this operation and a successful case is reported. The anatomy and physiology of penile function that is important to surgeons is reviewed.
Bladder exstrophy and cloacal exstrophy present challenging reconstructive problems. Traditionally, bilateral posterior iliac osteotomy has been performed in the majority of patients undergoing primary closure for these abnormalities. Recently, 2 anterior osteotomy approaches have been proposed: 1) incision of the ilium transversely above the acetabulum (transverse osteotomy) and 2) incision of the superior ramus of the pubis (superior ramus osteotomy). We devised a new anterior mid-iliac diagonal osteotomy that provides a more functional pelvic closure. To provide a fair comparison of the procedures we developed a pelvic exstrophy model based on data from a 3-dimensional computerized tomogram of a 3-year-old patient with classical bladder exstrophy. The different osteotomies were then performed on the model and the results were compared. Our new anterior mid-iliac diagonal osteotomy correction allowed the best surgical approach, provided the best correction of the flattened ilia bony deformities, was performed in the thinnest area of marrow bone and resulted in the best pelvic symmetry.
Epispadias, bladder exstrophy and its variants are in the first place usually considered as urological anomalies. Embryological theses and therapeutic approaches are mainly based upon this aspect. We challenge this point of view, in order to bring out a new axis of research about this still misknown pathologic field. A review of 16 cases of bladder exstrophy, 6 epispadias cases, and one cloacal exstrophy case, which had never been described before, revealed that the almost constant bony defect of the pelvic ring was linked with the severity of the visceral features, and with the continence status in epispadias cases. The commonly admitted theories about exstrophy development are based on a primary defect of the cloacal membrane. We suggest that the first anomaly could lie in a lack of rotation in the pelvic ring primordia.
Functional reconstructive surgery is performed in bladder exstrophy at the University Children's Hospital of Zurich since 1971. The surgical procedure is usually done in three stages--bilateral iliac osteotomy--turn-in-operation wih preliminary reconstruction of the bladder neck and antireflux surgery--closure of the epispadia, if necessary combined with continence plasty. Out of 26 patients with bladder exstrophy 23 underwent reconstruction. 3 patients died postoperatively. One was lost for follow-up. The preliminary long-term results in 19 patients are presented. The assessment was based on four criteria: last intravenous pyelography, urinary function, frequency and severity of urinary infections and degree of attained continence. The results were classified as good in 5 patients, fair in another 5 patients and insufficient in 6. 3 patients were too young to be assessed. The results are compared with other series reported in literature.
Classic bladder exstrophy and cloacal exstrophy are grotesque anomalies in the eyes of young parents. An immediate evaluation by an experienced team is required to assess the variations in the condition and to devise a plan of treatment that can reassure the parents as to eventual prognosis and rehabilitation. This plan of treatment must include provisions for renal preservation, urinary control, cosmetic appearance, and sexual function. The newborn is usually normal in respect to nutrition and its cardiopulmonary state, and will tolerate surgery well. Passive parental immunity provides better resistance to intercurrent illness at this time than will be present in the later months of infancy. When applicable, the initial stages of surgery should be undertaken at birth when the pelvic ring can be approximated without osteotomy and the bladder mucosa has not deteriorated from inflammatory changes. Parental attitudes toward the child as well as successful reconstruction may both be best served by immediate surgery to begin reconstruction and reduce the visible defect.
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The bladder exstrophy and epispadias complex (BEEC) is an anterior midline defect with variable expression involving the infraumbilical abdominal wall including the pelvis, urinary tract, and external genitalia. The incidence varies with regard to ethnical background, sex, and phenotypic expression, and an incidence of 1:20,000 to 1:80,000 has been observed in the middle European population. No gene defect has been attributed to BEEC thus far and chromosomal aberrations or genetic syndromes associated with BEEC have only rarely been reported. According to epidemiological data, a complex genetic as well as a multifactorial mode of inheritance could underlie BEEC. However, no single teratogenic agent or environmental factor has been identified, which could play a dominant role in the expression of the BEEC.A risk of recurrence of 0.5-3% has been described in families with one affected subject. These values correspond to an increased recurrence risk estimated to be as high as 200- to 800-fold when compared to the common population. Due to the paucity of affected sib pairs and suitable multiplex families, conventional linkage analysis to identify candidate genes causally related with BEEC appears to be unfeasible. Large association studies and consecutive linkage disequilibrium mapping should therefore lead to the identification of candidate genes. Also new methods including matrix-based comparative genomic hybridization (CGH) are promising and have successfully been used in the past (e.g., CHARGE association). Moreover, the low incidence of the BEEC requires close cooperation between clinicians in the operative and nonoperative specialties as well as geneticists for successful gene search.
Seven patients with bladder-exstrophy-epispadias complex underwent high resolution prostatic ultrasonography in order to establish the presence and appearance of their prostate gland and seminal vesicles. Six patients had been born with classic bladder exstrophy, and one patient with complete epispadias. The size of the prostate gland was in the normal range in three patients. Three patients had small glands, and in one patient no prostatic tissue could be identified. Two patients showed an unusual position or echoappearance of their glands. The seminal vesicles in five patients were relatively large, contained multiple cystic spaces, and/or extended posteriorly and inferiorly to the prostate gland proper. Our study demonstrates that the prostate gland and seminal vesicles are present in patients with bladder exstrophy. The unusual appearance, position, and size in most patients, however, suggests faulty embryologic development or changes secondary to surgery reflecting the complex nature of the condition. Likewise, the enlarged seminal vesicles may indicate impaired drainage of these structures secondary to the initial bladder closure or subsequent bladder neck reconstruction.
Young-Dees' method of bladder neck reconstruction is described. A material of 17 cases with complete or almost complete incontinence on account on a deficient sphincter who were submitted to this operative method is presented. It is concluded that the method can be recommended as the primary operation for incontinence, particularly in patients with exstrophy of the bladder or epispadias as satisfactory continence could be obtained in 60%. No deterioration of renal function was observed and the patients functioned well both socially and sexually.
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OBJECTIVE: To review the long term outcome of children who underwent urinary diversion for various non-malignant conditions and to assess the suitability of urinary diversion as an alternative treatment to primary bladder closure in cases of bladder exstrophy epispadias complex. DESIGN: A retrospective study. SETTING: Institute of Urology, Moshi, Tanzania. SUBJECTS: Fifteen children who underwent urinary diversion for various non-malignant conditions between 1985 and 1997. RESULTS: Fifteen children underwent urinary diversion for: exstrophy epispadias complex (n = 12) neurogenic bladder (n = 2) and trauma (n = 1). Seven children underwent Mainz pouch II diversion, six had classical ureterosigmoidostomy and two had appendicovesicostomy. One patient with a solitary kidney developed ureteral stenosis at the implantation site and was undiverted. Metabolic acidosis was well compensated with none of the patients requiring sodium bicarbonate supplements. All the patients except one were continent. CONCLUSION: We conclude that continent urinary diversion in children offers a viable alternative method for children with bladder exstrophy epispadias complex.