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Bladder exstrophy repair timeline and principals

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quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"The technique includes early closure of the bladder, posterior urethra, and abdominal wall, usually with pelvic osteotomy in the newborn period, subsequently followed by an early epispadias repair at 6 months to 1 year of age after testosterone stimulation by intramuscular injection. Around age 4–5 years, when adequate bladder capacity is reached and the child is ready to participate in a very structured preoperative and postoperative voiding program, a competent bladder neck is reconstructed, with bilateral ureteral reimplantation."

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quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"Achieving urinary continence with a sufficient bladder capacity is strongly dependent on initial successful closure of the bladder and the posterior urethra as well as the size of the bladder template. Therefore, the first step of the reconstruction is conversion of bladder exstrophy into a penile epispadias with incontinence with a balanced posterior outlet resistance that preserves renal function but stimulates bladder growth."

  • admin changed the title to Bladder exstrophy repair timeline and principals
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quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"pelvic osteotomies are performed if the patient is older than 72 h, for a symphyseal diastasis of more than 4 cm or if a tension-free closure cannot be achieved. In those cases, osteotomies are crucial to ensure tension-free approximation of the bladder, posterior urethra, and abdominal wall, preventing dehiscence or bladder prolapse. Furthermore, it places the urethra deep within the pelvic ring, enhances bladder outlet resistance, and ensures alignment of the large pelvic floor muscles to support the bladder neck."

  • Author

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"Combined exstrophy and epispadias repair: In a few selected cases, newborn exstrophy closure can be combined with epispadias repair. This approach requires good phallic length, a deep urethral groove, and an adequate amount of penile skin, however, and it should be attempted only by experienced exstrophy surgeons, as the complications can be severe. Combined exstrophy and epispadias repair is best applied in the patient undergoing delayed primary or reoperative exstrophy closure. The preoperative use of intramuscular testosterone in reoperative exstrophy patients will allow for improved vascularity and more penile skin for the reconstruction."

  • Author

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"The operations are performed with general anaesthesia, with the patient in a supine position even for the osteotomies. A tunneled epidural catheter is placed when possible to reduce the intraoperative amount of anaesthetic agents and for postoperative pain control. It is typically left in place for several weeks after the closure. Care must be taken to create a latex-free environment in the operation room, as many children with bladder exstrophy are prone to latex allergies. Perioperative broad-spectrum antibiotics are administered and continued throughout the first postoperative week."

  • Author

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"The ureteral stents are left in place for 10–14 days, and the suprapubic tube is removed 4 weeks postoperatively, after calibrating the bladder outlet to warrant free drainage. Note that the urethra is not stented at the end of the operation to avoid pressure necrosis, infection, and secretion accumulation. The pelvis is approximated in the midline by gently applying pressure over the greater trochanters bilaterally. Horizontal mattress sutures of #2 nylon are placed in the pubis. It is important to tie the knot away from the neourethra to avoid material migration into the posterior urethra. A second stitch of #2 nylon is used at the most caudal insertion of the rectus fascia onto the pubic bone for added security, if it can be easily done and does not compromise the first stitch."

  • Author

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"Before the operation, the bladder capacity is measured annually by gravity cystograms with the child in anaesthesia. A bladder capacity of 100 mL or more is necessary to undergo bladder neck reconstruction. All children undergo an intense voiding training program, along with urodynamic evaluation, prior to the reconstruction."

  • Author

quote from "Pediatric Surgery (Springer Surgery Atlas)" by Prem Puri, Michael E. Höllwarth -

"Successful initial closure of the bladder and posterior urethra is the most important factor for achieving urinary continence and sufficient bladder capacity.

The fragile mucosa and the detrusor function are best preserved by closing the bladder in the newborn period, but the size and the functional capacity of the detrusor muscle are important considerations for the outcome. Therefore, in the rare presence of a small, fibrotic bladder patch without elasticity or contractility, the operation should be deferred until adequate growth of the bladder template occurs. The risk of bladder neck failure is higher for the group with smaller bladder capacities (<100 mL). If sufficient size is not reached 4–6 months after birth, alternative options like creation of a colon conduit or ureterosigmoidostomy must be employed. Later in life, the former urinary diversion can be converted into a continent catheterizable pouch bladder or augmented bladder if the template is still intact."

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