PubMed · 9138712
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P G Duffy. 1996. Bladder exstrophy.. https://pubmed.ncbi.nlm.nih.gov/9138712/
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PURPOSE: We assessed the impact of posterior urethral obstruction after primary bladder exstrophy closure. MATERIALS AND METHODS: A review of the records of patients with classic bladder exstrophy at our institution identified 29 boys and 12 girls with a mean age of 11.75 years who had had posterior urethral outlet obstruction after closure was done in the neonatal period. RESULTS: Of these 41 patients 75% underwent closure elsewhere. At closure osteotomies were done in 13 patients and 23 were younger than 72 hours. Paraexstrophy skin flaps were used at primary closure in 27 cases (66%). Obstruction presented as recurrent urinary tract infection, upper tract deterioration with or without renal failure, bladder stones, difficult catheterization, urethral stitch erosion, a full bladder on ultrasound, a prolonged dry interval, urinary retention, inability to catheterize, bladder rupture, rectal prolapse and epididymitis or prostatitis. Usually the initial obstructive episode developed within 60 days of closure and it was recurrent. Therapy included suprapubic catheter placement, vesicostomy, ureterostomy, nephrostomy and multiple urethral manipulations, such as dilation with or without steroid injection, internal urethrotomy, urethral stitch removal, clean intermittent catheterization or open urethroplasty. All 6 patients who underwent long-term diversion via vesicostomy, ureterostomy or a conduit for greater than 6 months required permanent bowel segments for reconstruction, while in 5 of the 6 who underwent short-term diversion via nephrostomy or suprapubic tube placement for less than 6 months reconstruction was bowel-free. Of the 36 children in whom functional reconstruction was performed 9 are undergoing staged reconstruction, reconstruction failed in 14, 4 are socially dry and 9 are continent. CONCLUSIONS: Posterior urethral obstruction after exstrophy closure markedly decreases the success of staged bladder reconstruction, presents a significant risk to the upper urinary tract and should be detected early.
OBJECTIVES: To describe a unique approach to the management of duplicate bladder exstrophy combining initial bladder closure and epispadias repair. Bladder exstrophy has been successfully managed by staged surgical repair with early bladder closure, subsequent epispadias repair, and, finally, bladder neck reconstruction. Duplicate bladder exstrophy is a rare variant of the exstrophy complex with fewer than 20 cases reported. METHODS: A male newborn presented with the appearance of both bladder exstrophy and a ruptured omphalocele. Repair of the omphalocele and bilateral orchiopexy was performed shortly after birth, but bladder closure was delayed until there was complete healing of the omphalocele defect, and the investigators believed the infant was ready for abdominal wall and bladder exstrophy closure. At age 8 months, bladder closure was performed with the intraoperative finding of a duplicate bladder lying posterior to the exstrophied bladder. The left ureter drained on the exstrophic bladder plate, and the right ureter drained into the posterior (internalized) duplicate bladder. After anterior innominate and vertical iliac osteotomies, the left ureter was reimplanted into the posterior bladder. A portion of the exstrophied bladder was then tubularized to construct a neourethra of the epispadic penis. RESULTS: The child has done well with an excellent cosmetic appearance of the abdominal wall and a straight phallus. The bladder subsequently required a Mitrofanoff-type continent stoma along with bladder augmentation, which was performed at the time of his Young-Dees-Leadbetter bladder neck reconstruction (age 3.5). The child is currently continent but requires intermittent clean catheterization through his appendicovesicostomy. CONCLUSIONS: The investigators report a unique and unexpected variant of bladder exstrophy and its successful management.
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.