Figs. 13.42 and

13.43 Clinical photographs of a male with classic bladder exstrophy. Note the small size of the open urinary bladder and the associated epispadias. Note also the phallus which is short, wide with dorsal chordee

OPEN

URINARY

BLADDER

EPISPADIAS

OPEN

URINARY

BLADDER

EPISPADIAS

Fig. 13.44 A clinical photograph of a female with bladder extrophy. Note the small size of the urinary bladder with granular appearance

Fig. 13.45 A clinical photograph showing bladder exstrophy in a female. Note also the associated vaginal prolapse

13.4.2 Associated Anomalies

•Associated anomalies are common in patients with cloacal extrophy-epispadias complex.

•The ureters in bladder extrophy and cloacal extrophy patients enter the bladder at an abnormal angle leading to vesicoureteral reflux in all patients following bladder closure. The ureters are most commonly

reimplanted into the bladder at the time of augmentation or bladder neck reconstruction in staged repair. They can also be safely reimplanted during primary complete repair.

•Common urological anomalies include ureteropelvic junction obstruction, horseshoe kidney, and ectopic kidney.

•The pelvic deformities may cause the child to ambulate with a waddling gait.

•Magnetic resonance imaging (MRI) studies have shown that the pelvic floor musculature is also significantly different in patients with extrophy-epispadias complex. Cloacal extrophy patients have similar, but more severe, pelvic floor musculature abnormalities than bladder extrophy patients. These deficits contribute to incontinence in these patients and predispose females to vaginal and uterine prolapse.

•Gastrointestinal Abnormalities

–An anteriorly displaced anus and anal sphincter is found in patients with bladder extrophy.

–Bladder extrophy patients occasionally have omphalocele, imperforate anus, rectal stenosis, and rectal prolapse.

–Cloacal extrophy patients almost always have some gastrointestinal defect. These include omphalocele, imperforate anus, rudimentary hindgut, malrotation of the bowel, and short gut syndrome, the last of which is often compounded by multiple bowel surgeries.

•Neurospinal Abnormalities

–Approximately 7 % of bladder extrophy patients will have a spinal abnormality such as spina bifida occulta, scoliosis, and hemivertebrae.

–Spinal dysraphism may cause neurologic dysfunction.

–Nearly all cloacal extrophy patients demonstrate significant neurospinal deficits including:

•Neural tube defects

•Vertebral anomalies

•Spinal myelodysplasia

•Spinal dysraphism

•Tethered cord

–These associated anomalies necessitate prompt neurological evaluation with spinal US and MRI and can further exacerbate urinary and bowel incontinence, lower extremity immobility, and erectile dysfunction.

13.4.3Principles of Surgical Management of Bladder Exstrophy

•The surgical technique of staged repair of classic bladder exstrophy include:

–Initial bladder closure is completed within 72 h of birth.

–If bladder closure is delayed, pelvic osteotomies are required to facilitate successful closure of the abdominal wall and to allow the bladder to lie within a closed and supportive pelvic ring.

–Performance of anterior innominate osteotomy allows approximation of the pubic bones, closure of the symphysis pubis and abdominal wall muscles without tension.

–This is performed in conjunction with pediatric orthopedic surgeons.

–The urinary bladder is drained via a suprapubic tube or a urethral catheter and ureteral stents are also left in place.

–At the end of the first stage procedure, the patient will be left with an epispadias.

–Repair of epispadias is delayed till the age 12–18 months to allow time to improve bladder capacity.

–The epispadias repair is performed using the modified Cantwell-Ransley repair technique.

–The bladder neck is reconstructed at the age of 4–8 years.

–Bladder neck reconstruction is done using the modified Young-Dees-Leadbetter repair. This allows correction of associated vesico-ureteral reflux.

–The procedure is delayed until bladder capacity is adequate.

–It has been reported that the results are much better with a bladder capacity greater than 85 ml.

Fig. 13.46 A clinical photograph showing the end result after staged repair of classic bladder exstrophy. Note the scar of bladder closure. Note also the small phallus with epispadias which needs to be repaired in the second stage

•The surgical technique of complete primary repair for classic bladder exstrophy:

–In this technique, bladder closure, epispadias repair, and genital reconstruction are performed in a single stage in the newborn period.

–Hypospadias is a common outcome in males postoperatively and requires subsequent urethroplasty (Fig. 13.46).

13.4.4 Evaluation and Management

•It is important to tie the umbilical cord immediately after delivery with 2.0 silk as the umbilical clamp causes irritation and trauma to the open urinary bladder.

•The bladder mucosa should be frequently irrigated with warm saline and always covered with a protective clear plastic wrap that is changed at frequent intervals until the time of bladder closure. Avoid using wet gauze which can cause irritation to the delicate bladder mucosa.

•The treatment of bladder exstrophy-epispadias is complex should be performed in specialized

centers and by experienced surgeons. The reconstructive surgery consists of a series of corrective surgeries performed over several years.

•The management of these patients should be in specialized centers and via a multidisciplinary approach which include:

–Neonatologist

–Pediatric gastroenterologist

–Pediatric urologist or pediatric surgeon with special interest and expertise in the management of exstrophy-epispadias complex

–Neurosurgeon

–Pediatric Orthopedic surgeon

–Child psychiatrist

–Stoma therapist

–Social workers

•The parents stress should not be overlooked during the initial and long-term care of these patients.

•A cardiac echo is commonly done to rule out significant cardiopulmonary anomalies.

•A renal ultrasound should be obtained to evaluate the upper urinary tracts.

•A KUB is done to evaluate the pelvis bony anatomy.

•A spinal ultrasound is done to rule out an associated spinal dysraphism.

•Antibiotics should be started immediately after delivery and continued postoperatively.

•Daily prophylactic antibiotic therapy should be given to these patients during postoperative follow-up.

•These patients have a high incidence of latex sensitization and it is important to institute latex precautions.

•The goals of treatment include:

–Closing the defect in the urinary bladder

–Closing the abdominal wall defect while maintaining renal function

–Achieving urinary continence and adequate urinary storage at low pressures with the ability to empty the bladder completely without compromising renal function.

–Maintaining sexual function.

–Achieving cosmetically and functionally acceptable external genitalia.

–Creation of a neo-umbilicus

•In the past, early attempts at bladder exstrophy repair were unsuccessful and for many years the management for bladder exstrophy consisted of excision of the exstrophic bladder and urinary diversion commonly by ureterosigmoidostomy.

•The surgical management of bladder exstrophy has changed over the years and many modifications in surgical techniques have improved the outcome for these patients.

•Currently, most patients are managed either with:

–A (single stage) complete primary repair of bladder exstrophy (Mitchell technique).

–Or modern staged reconstruction of exstrophy.

•There are those who advocate a staged approach while others prefer total reconstruction in one stage.

•Early bladder closure (closure during the first 72 h) is beneficial.

–It decreases inflammation and fibrosis of the bladder

–Improves bladder growth and expansion

–Decreases the need for urinary diversion

–Decreases the risk of precancerous changes in the bladder

•Pelvic osteotomies:

–Pelvic osteotomies may be performed at the time of primary closure.

•This helps deepen the flattened pelvis

•Close the pubic diastasis

•Release tension on the abdominal wall

–Pelvic osteotomies are recommended in patients who undergo repair after 72 h of age as this facilitates closure of the bladder and abdominal wall and also results in deeper placement of the bladder into the pelvis.

–A combination of bilateral anterior transverse innominate and vertical posterior iliac osteotomies has been shown to decrease the rate of abdominal dehiscence and bladder prolapse.

–There are several maneuvers to facilitate this including:

•The use of fixator pins and external fixation devices can be placed and left postoperatively for 4–6 weeks.

•Modified Buck’s traction exerts pull longitudinally on the lower extremities.

•Modified Bryant’s traction, where the hips are placed into 90° of flexion, may be used if there is no osteotomy.

•Spica casts to immobilize the pelvis without the need for external fixators or traction.

•“Mummy wrapping” the child’s legs.

•Complete Primary Repair of Exstrophy (CPRE):

–This technique was pioneered by Dr. Mitchell in 1996.

–The CPRE is done as a one stage procedure with or without pelvic osteotomies and includes:

•Bladder closure with bladder neck remodeling.

•A disassembly technique for epispadias repair.

•This technique allows for bladder cycling and more ‘normal’ growth and development because the bladder experiences an outlet resistance.

•The disassembly technique for epispadias repair also allows for division of the intersymphyseal ligaments and appropriate anatomic placement of the bladder neck and posterior urethra deep into the pelvis in its normal position.

•If the procedure is done within the first 72 h of life, the pelvis is malleable enough to close without osteotomies.

•Postoperatively, the patient is placed in

Bryant’s traction for approximately 1 week and then lower extremity casting for 3 weeks to prevent tension on the pelvis closure.

•A large number of these patients require a formal bladder neck procedure to achieve continence

–Outcome and complications of CPRE:

•The outcome of CPRE is generally good.

•Continence defined as dry intervals longer than 2 h and spontaneous voiding without catheterizations is around 75 %.

•About 30 % of these patients require clean intermittent catheterization (CIC).

•There is a potential risk for renal deterioration related to a high pressure lower urinary system in these patients

•Glans necrosis and penile injury or loss.

•Hydronephrosis, pyelonephritis and renal scarring.

•Approximately 36–68% of patients will be left with a hypospadias that will require urethroplasty (Figs. 13.47, 13.48, and 13.49).

•Modern Staged Reconstruction of Exstrophy (MSRE):

–In this technique, the exstrophic bladder is closed first.

–A pelvic osteotomy will facilitate this closure as well as closure of the abdominal wall. This is especially so if the closure is performed after 72 h of birth.

–The end result is a complete epispadias.

–The epispadias repair is performed between 6 and 12 months of age.

–Penile growth can be stimulated with long acting testosterone.

–The bladder neck repairs for continence is performed between 4 and 5 years of age if they have an adequate bladder capacity.

–This can be delayed until the bladder grows and maturity improves.

–The other alternative is bladder augmentation with a catheterizable channel and bladder neck closure when necessary.

–Outcome and complications:

•Continence rates are reported to be 70 % in males and 74 % in females with dry periods greater than 3 h with spontaneous voiding and dry at night without clean intermittent catheterization.

•A bladder capacity of 100 ml predicts success at the time of bladder neck reconstruction utilizing the modified Young-Dees-Leadbetter repair.

•If the bladder capacity is less than 100 ml, these patients should undergo an augmentation at the time of reconstruction for continence.

•Bladder neck reconstruction:

–This allows continence and correction of vesicoureteral reflux.

–The procedure is delayed until bladder capacity is adequate; better results are reported with a bladder capacity greater than 85 ml.

–A continence procedure such as the Young- Dees-Leadbetter, is delayed until the patient achieves a bladder with adequate capacity and desires continence (usually between 5 and 9 years of age).

–This stage is combined with ureteral reimplantation to repair associated vesicoureteral reflux.

–Children who are not candidates for a continence procedure or who fail to achieve urinary continence after the procedure may require bladder neck transection, augmentation cystoplasty, and continent catheterizable stoma.

–This will make clean intermittent catheterization (CIC) to empty their bladders easier.

•Bladder Augmentation:

–This is indicated for patients with a bladder that is noncompliant or of insufficient capacity.

–Several techniques using segments of bowel, stomach, or redundant ureter have been used to augment the bladder.

–Following augmentation cystoplasty, a continent urinary diversion is performed using a segment of appendix or ileum.

–This segment connect the urinary bladder to the abdominal wall and provide a continent stoma through which to perform clean intermittent catheterization.

–This makes clean intermittent catheterization much easier for these patients and more convenient.

•In females, the external genitalia are fully reconstructed at the time of exstrophy closure.

–This includes reapproximation of the bifid clitoris and anterior labia to make a fourchette.

–A monsplasty is an important part of reconstruction in females using hair bearing skin and fat to cover the midline defect.

–Further reconstruction is done during adolescent years including total urogenital mobilization to correct the location and angle of the vagina in female exstrophy patients.

–These patients usually have normal sexual desire and normal sexual intercourse after reconstruction.

–These patients can subsequently become pregnant and deliver normally.

–There is however an increased risk of vaginal, cervical and uterine prolapse in these patients. This is especially seen after pregnancy and delivery.

–It is recommended that these patients should undergo cesarean sections to eliminate stress on the pelvic floor and to avoid traumatic injury to the urinary sphincter mechanism.

•Male patients with exstrophy have normal sexual function and libido but are at high risk of infertility after reconstruction. These patients can be helped further with an assisted reproduction.

•Currently, 70–75 % of patients with bladder exstrophy are continent following modern reconstruction.