Complete primary closure of bladder exstrophy. Epispadias and bladder exstrophy repair

Bladder exstrophy remains one of the most challenging problems in pediatric urology. Recent efforts have focused on primary reconstruction rather than urinary diversion to treat exstrophy. Complete primary closure appears to offer improved continence and decreases the number of surgical procedures required to treat exstrophy.     

Failed bladder exstrophy repair. Evaluation and management

Early successful closure, careful management of the incontinent interval, and a good technical bladder neck reconstruction can result in a patient with normal control and upper tracts. However, failure at any step of a staged functional reconstruction does not doom the child to failure in the eventual achievement of the above goals. Nonetheless, taking care of the exstrophy failure is still a formidable task for the reconstructive surgeon. Currently, surgical techniques exist that can salvage the vast majority of these children. Careful evaluation of the failure, waiting an appropriate interval before further reconstructive surgery is attempted, and dedication to the patient most often can produce a successful result.     

Complete primary repair of exstrophy.

PURPOSE: The surgical correction of bladder exstrophy to achieve continence with voiding remains a challenging problem for the urologist. Since 1989 we have performed complete primary repair for exstrophy based on the concept that the primary defect of bladder and cloacal exstrophy is anterior herniation. Thus, the bladder and urethra must be treated as a single unit to move them posteriorly into the pelvis. We present this technique. MATERIALS AND METHODS: From 1989 to 1997, 18 patients with bladder exstrophy and 6 with cloacal exstrophy underwent complete primary repair of exstrophy. This procedure was done on day 1 of life in 18 patients. Mean followup is 44 months (range 4 months to 8 years). RESULTS: At a median followup of 48 months 4 boys and 4 girls have volitional voiding after complete primary repair of bladder exstrophy, 21 patients have continent intervals and 2 boys void with continent intervals after complete primary repair of cloacal exstrophy. No patient has had a loss of renal function in this series. Postoperative complications included urethrocutaneous fistula formation in 2 cases. No patient had primary closure dehiscence. CONCLUSIONS: The rate of urinary continence achieved with complete primary repair compares favorably to that of staged repair for exstrophy. Complete primary repair also minimizes the number of surgical procedures required to achieve urinary continence and potentiates bladder neck function that permits bladder cycling in year 1 of life. The complication rates of these techniques are significantly lower than those reported in previous series of primary closure of exstrophy.     

Resultant hypospadias after epispadias repair in bladder exstrophy patients: a difficult surgical task with high complication rate

Background/Purpose

The aim of this study was to analyze the complication rate in male bladder exstrophy (BE) patients undergoing flap or graft urethroplasty for the repair of resultant hypospadias after epispadias repair.

Methods

We retrospectively reviewed the charts of 22 male BE patients who underwent 24 urethroplasties for resultant hypospadias between 2000 and 2009. Median patient age was 4.2 (range, 1.5-26.5) years, and median follow-up was 7.5 (range, 0.8-10.3) years. Meatal location after epispadias repair was midshaft in 6 cases and proximal shaft in 15. Complications were compared in relation to meatal position, type of urethroplasty (no graft vs graft), use of second-layer coverage of the urethroplasty, and use of suprapubic diversion.

Results

Overall, complications developed in 12 (50%) patients, including 10 urethrocutaneous fistulas and 2 urethroplasty dehiscence. Univariate analysis failed to show any differences between complicated and uncomplicated cases in all the variables. Only the 3 cases undergoing a 2-stage repair had fully successful outcomes.

Conclusions

Urethroplasty in patients with BE has a high complication rate. Quality of local tissue and presence of scarring are possibly the 2 major determinants of a poor outcome. A staged repair seems the safest, although this commits the patient to 2 procedures.     

Complete primary repair of exstrophy. Surgical technique

The single-stage reconstructive approach to exstrophy evolved out of changes in the management of exstrophy. The success of Jeffs and others that functionally reconstructed the bladder of patients with exstrophy demonstrated that the approach was feasible and acceptable. Increased understanding of the anatomic pathology associated with exstrophy and epispadias resulted in the development of complete penile disassembly for epispadias and the extension of this technique to exstrophy as the complete primary repair technique described herein. The results using this technique are encouraging, leading to the recommendation for the procedure by other surgeons committed to the care of patients with exstrophy.     

Complete reconstruction of bladder exstrophy. Experimental program.

Diabetes mellitus is a prevalent disorder, well controlled in many persons with prolongation of life. Several radiologic manifestations are sufficiently specific to suggest a diagnosis in the unidentified patient, but even more important is an awareness of the sometimes life-threatening complications of diabetes which can be diagnosed from uroradiologic studies. We review the following urinary tract manifestations and complications of diabetes: pyelonephritis, perinephric abscess, renal papillary necrosis, emphysematous pyelonephritis, emphysematous cystitis, fungus infections, calcification of the vas deferens, seminal vesicle, and intrarenal branches of the renal artery, neuropathic bladder, and renal failure.     

Anterior innominate osteotomy in repair of bladder exstrophy

BACKGROUND: Classic bladder exstrophy is a developmental defect presenting at birth with a wide pubic separation and an exposed bladder; cloacal exstrophy involves, in addition, intestinal prolapse. Reconstruction requires several surgical procedures. The use of anterior iliac osteotomies in this process has not been reviewed in a large series. METHODS: We reviewed the results of eighty-six anterior innominate osteotomies performed in conjunction with genitourinary repair of classic and cloacal bladder exstrophy in eighty-two patients. Clinical outcome measures were successful bladder closure, achievement of continence, and maintenance of a normal gait. Radiographs of the pelvis were reviewed, and the pubic intersymphyseal diastasis (a measure of the reduction in tension on the anterior closure) was measured preoperatively and at three time-points postoperatively. Children with classic exstrophy who had undergone osteotomy and bladder neck reconstruction but not bladder augmentation were divided into four groups on the basis of the degree of continence. In addition, children with classic exstrophy were stratified according to age at the time of the osteotomy. The mean postoperative percent reduction in the amount of the original diastasis was determined for all groups. RESULTS: Children with classic exstrophy and those with cloacal exstrophy had correction of the diastasis after the osteotomy, with greater correction in those with classic exstrophy, presumably because of better bone quality. Daytime continence was achieved with anterior osteotomy and bladder neck reconstruction in 74% of the children for whom continence was a goal. However, no difference in the symphyseal diastasis or in the percentage of pubic reduction was detected among the four continence groups. Children who were older at the time of the osteotomy maintained better correction over time. Wound dehiscence or bladder prolapse occurred in 4% of the patients who had osteotomy and primary closure, and the only important complication of the osteotomies was transient palsy of the left femoral nerve in seven children. CONCLUSIONS: Anterior innominate osteotomy is an effective part of reconstructive repair of bladder exstrophy. The primary goals of the osteotomy are to reduce the tension in the closed bladder and the lower abdominal wall and to promote continence by restoring the sling of the pelvic floor muscles. These goals can be achieved in the majority of patients.     

Initial management of complex urological disorders: bladder exstrophy

Bladder exstrophy is a rare condition associated with lifelong issues including infection,renal insufficiency, incontinence, and fertility. Despite its seriousness, many affected children lead normal lives. With new operative techniques, children require fewer surgeries and have more hope for continence.With close follow-up, no child should develop renal insufficiency. Families should be reassured that a normal life and parenthood with normal sexual function are possible.     

Spontaneous perforation of augmented bladder after exstrophy repair.

A patient with spontaneous perforation of an augmented bladder following exstrophy repair is presented. Apart from the clinical presentation the various causes for augmented bladder perforation are discussed and the literature reviewed.     

Primary surgical repair of combined gastroschisis and bladder exstrophy

The combination of bladder exstrophy and gastroschisis has not been reported previously. The authors describe this rare combination of congenital anomalies, as well as the successful multidisciplinary management of this unusual problem.     

Surgical management of incontinence in bladder exstrophy.

Between 1978 and 1990, 86 patients with previously closed classical bladder exstrophy and 10 patients seeking undiversion have presented for continence management and have undergone selective reconstruction designed for voiding and/or intermittent urethral catheterisation. The reconstruction in these 96 patients has been reviewed. Eight of the 10 patients undergoing undiversion achieved a satisfactory state of continence but 4 required Mitrofanoff procedures to enable catheterisation. Of the other 86 patients, 2 reached a satisfactory state of continence without further surgery; 79 underwent bladder neck surgery for continence either without augmentation (n = 32) or with augmentation (n = 47). Twenty of the 32 patients who were treated by bladder neck reconstruction alone were later found to require augmentation. Five patients had very early augmentation either to facilitate neonatal closure or on account of severe upper tract dilatation. Of these, 1 became continent without further surgery and 4 demonstrated the need for bladder neck reconstruction. Thus 12 children achieved successful continence (n = 6) or are evolving satisfactorily with potential success (n = 6) as a result of bladder neck reconstruction. Of the 71 patients requiring bladder neck reconstruction and augmentation, 68 have completed their surgery. The current status of these patients is: satisfactory in 57 (80%) (42 void/urethral clean intermittent catheterisation (CIC), 7 waiting to learn CIC, 5 Mitrofanoff, 3 artificial urinary sphincter (AUS]. Of the remaining 11 patients (20%), 8 are unsatisfactory to varying degrees and the status of the other 3 is unknown.     

Plastic repair of the isolated bladder in exstrophy in children

Sigmoid colon segment was used to pass the urine through the intestine in 14 children with exstrophy of the bladder. Preoperative management of large intestine helped to reduce the number of operative stages from two to one. 12 patients were followed up from 1 to 3 yrs. Right ureterohydronephrosis of the upper urinary tract was observed in 1 patient. Urodynamic investigation of isolated sigmoid colon segment evidenced of its adequate contractile and peristaltic activity preventing the contact of feces with entero-ureteral anastomoses. To improve the closing ability of rectal sphincter in the above patients anal electrostimulation with diadynamic current can be recommended.