Exstrophy and Epispadias Treatment & Management

Updated: Jul 26, 2016
  • Author: Elizabeth B Yerkes, MD; Chief Editor: Marc Cendron, MD  more...
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Treatment

Medical Care

For neonates with exstrophy and epispadias, initiate general supportive care appropriate for the overall condition and associated anomalies. For patients with cloacal exstrophy, institute parenteral nutrition early.

Place clean plastic wrap over the bladder plate (see the image below). Avoid moistened or impregnated gauze, which is irritating to the delicate bladder mucosa. Mist tents may be used to protect exposed tissue.

Use of plastic wrap to protect the delicate bladde Use of plastic wrap to protect the delicate bladder mucosa in a newborn with bladder exstrophy. Courtesy of Richard Rink, MD.

Start antibiotic therapy after delivery, and continue through the early postoperative period. Daily administration of prophylactic antibiotics may be continued in the weeks after bladder closure. Surgeons' philosophies on this matter vary. Infections may be related to poor emptying and are to be prevented in light of the high incidence of vesicoureteral reflux.

Institute latex precautions; there is a high incidence of latex sensitization in patients with exstrophy-epispadias complex.

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Surgical Care

Reconstruction of exstrophy-epispadias complex remains one of the greatest challenges facing the pediatric urologist. Many modifications in surgical procedures have improved outcomes, but the optimal approach remains uncertain. Longitudinal prospective assessment of the two main current surgical approaches (staged procedure and total reconstruction) is critical for optimizing functional and cosmetic outcomes.

Complete primary reconstruction is now more than 20 years old; however, each approach is in a constant state of minor modification. Data on the newer approach continue to mature and are updated almost yearly. [16, 17, 18, 19, 20, 21] Analysis of each experience focuses on daytime continence with volitional voiding, need for further surgical procedures, and complication rates. In experienced hands, the safety and efficacy of the different approaches are comparable.

Goals of therapy include provision of urinary continence with preservation of renal function and reconstruction of functional and cosmetically acceptable genitalia. Creation of a neoumbilicus is also important to many of these patients.

Surgical techniques used in the treatment of exstrophy-epispadias complex include the following:

  • Staged functional closure for classic bladder exstrophy (ie, modern staged repair of exstrophy [MSRE]) [22]
  • Complete primary repair for classic bladder exstrophy (CPRE)
  • Urinary diversion for classic bladder exstrophy
  • Closure for cloacal exstrophy
  • Gender reassignment

Staged functional closure for classic bladder exstrophy

MSRE, which represents the traditional surgical approach, comprises a series of operations. Initial bladder closure is completed within 72 hours of birth. If this 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.

Epispadias repair with urethroplasty is performed at age 12-18 months. This allows enough increase in bladder outlet resistance to improve the bladder capacity.

Bladder neck reconstruction (typically a modified Young-Dees-Leadbetter repair) is performed at age 4 years. This allows continence and correction of vesicoureteral reflux (VUR). Multiple modifications have been proposed. The procedure is delayed until bladder capacity is adequate; better results are reported with a capacity greater than 85 mL.

The radical soft-tissue mobilization (RSTM) procedure, also referred to as the Kelly repair, has been suggested as an alternative approach to staged reconstruction of bladder exstrophy. [23, 24]

Complete primary repair for classic bladder exstrophy

Compared with MSRE, CPRE is a newer approach to exstrophy closure. Primary bladder closure, urethroplasty, and genital reconstruction are performed in a single stage in newborns. This procedure involves complete penile disassembly in males and mobilization of the urogenital complex in females. Hypospadias is a common outcome in males and requires subsequent reconstruction.

The goal is early bladder cycling. A subset of patients have achieved continence without bladder neck reconstruction.

In a study of 34 boys treated with a modified penile disassembly technique (15 with bladder exstrophy who underwent CPRE, 11 with penopupic epispadias after previous closure of bladder exstrophy, and eight with isolated complete epispadias), Anwar et al found the modified technique to yield excellent cosmetic results. [25] Preservation of the distal urethral plate along with both hemiglans avoided shortening and prevented occurrence of hypospadias.

Urinary diversion for classic bladder exstrophy

Urinary diversion was the original surgical treatment of choice. Diversion may be performed in a patient with an extremely small bladder plate not suitable for functional closure. In Europe, early diversion has been widely used, with success for most exstrophy patients.

Closure for cloacal exstrophy

Treatment of myelodysplasia and gastrointestinal (GI) anomalies has priority over management of urinary and genital anomalies.

Closure can be either staged or performed in a single stage, depending on the overall condition of the child and the severity of the abdominal wall defect. If a large omphalocele is present, successful closure of the abdomen and the bladder in one stage may be difficult to accomplish.

The first stage involves separation of the GI and genitourinary tracts, closure of the colon, creation of a colostomy, and closure of the omphalocele. The bladder plates are brought together in the midline.

Because virtually all of these patients have some element of short-gut syndrome, the hindgut should be incorporated into the GI tract to maximize absorptive surface area. Ileostomy should be avoided because of the high incidence of recurrent hospitalizations for dehydration and severe electrolyte abnormalities. The decision between rectal pull-through and permanent colostomy is based on the surgeon's preference and the projected potential for social fecal continence. [26]

Subsequent bladder closure is carried out as in surgical management of classic bladder exstrophy. The principles of complete primary repair have been applied at this point as well. Consideration may be given to continent diversion as the second stage, on the basis of poor potential for volitional voiding and continence.

Because of more severe pubic diastasis, pelvic osteotomies are required. Staged pelvic osteotomy (SPO) with gradual closure of the pelvis may be needed in severe cases. [27] In a study comparing SPO before bladder closure with combined pelvic osteotomy (CPO) at the time of closure in cloacal exstrophy patients, Inouye et al found that SPO reduced preoperative diastasis more than CPO did, without appearing to incur increased rates of complication, closure failure, or incontinence. [28]

Gender reassignment

Historically, all males with cloacal exstrophy underwent early gender conversion because of inadequate male genitalia. Testicular histology is normal despite frequent cryptorchidism.

Evidence suggesting that testosterone in utero has a significant impact on the developing brain has led to a change in surgical philosophy, as has anecdotal evidence suggesting that raising a 46,XY cloacal exstrophy patient as female can result in significant gender dysphoria. Cloacal exstrophy is now included as a subset of disorders of sex development. [29] Multidisciplinary evaluation and both early and long-term counseling should be offered.

Intraoperative concerns

Multiple or lengthy surgical procedures with exposure to latex antigens increase the risk of latex sensitization or allergy. [20] Approximately 30% of patients with bladder exstrophy have demonstrated symptoms of latex allergy, and 70% reveal sensitization (elevation of specific immunoglobulin E [IgE] antibody) to latex antigens. For practical purposes, all patients with exstrophy-epispadias complex should be considered to be latex-sensitive.

Full latex precautions are recommended in the operating room, beginning with preparation for the first operative procedure. Potential latex-containing materials in the operating room include gloves, catheters, drains, masks, anesthesia materials, bandages, and thromboembolic stockings. Polyvinyl chloride and silicone are acceptable alternatives. Latex allergy should be considered seriously in the event of intraoperative anaphylaxis. The offending agent should be removed and the surgical procedure aborted if necessary.

Treatment includes cardiopulmonary resuscitation with fluids, epinephrine, steroids, and histamine blockade. In those with a known latex allergy, premedication with steroids and histamine H1 and H2 blockers should be considered.

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Postoperative Care

Postoperatively, patients with exstrophy remain in the hospital in modified Bryant traction (legs adducted and pelvis slightly elevated) for 3 weeks after bladder closure. Alternative techniques of immobilization may be used, based on osteotomies or institutional protocol.

Bladder and kidneys are drained fully with multiple catheters during the first few weeks after closure.

Nutritional support is mandatory for patients with cloacal exstrophy. Patients with classic bladder exstrophy may also have early difficulties feeding because of the body position in traction.

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Complications

In the treatment of complex congenital anomalies, the distinction between technical complications and problems inherent to the anomaly is not always obvious.

Failure of closure may occur. If the bladder plate is adequate, reclosure with pelvic osteotomies is recommended. In this instance, bladder closure and epispadias repair are performed in one stage. Urinary diversion is the alternative therapy.

A vesicocutaneous fistula or urethrocutaneous fistula may form after primary closure or urethral reconstruction. If spontaneous closure does not occur, surgical repair is required.

Loss of the hemiglans or corporal body has been reported as a result of complete primary repair. [30, 31]

Minor orthopedic complications may occur after osteotomy or immobilization.

Upper urinary tract deterioration is a potential complication. Causes include excessive outlet resistance and high pressure in a small-capacity reservoir and persistent VUR.

Abnormal bladder function may result in poor emptying. Clinical problems related to poor emptying include recurrent febrile infections, epididymitis, bladder stones, acute urinary retention, and rupture of the native bladder.

Bladder prolapse is a potential complication. Posterior bladder wall may prolapse through the patulous bladder neck after primary closure (see the image below). Recurrent prolapse, congestion, ischemia of bladder mucosa, or failure of ureteral drainage warrants early surgical correction.

Subtotal prolapse of bladder wall through patulous Subtotal prolapse of bladder wall through patulous bladder neck after bladder closure in female with cloacal exstrophy. The bladder mucosa appears healthy, and urine is draining freely. Courtesy of Richard Rink, MD.

Malignancy is a rare late complication of bladder exstrophy and is more common in untreated patients whose bladders are left exstrophic for many years. Adenocarcinoma is the most common of these malignancies, from the precursor cystitis glandularis, which is caused by chronic irritation and inflammation of exposed mucosa of the exstrophic bladder. Squamous cell carcinoma and rhabdomyosarcoma have also been reported.

Adenocarcinoma may develop adjacent to the ureterointestinal anastomosis in patients with urinary diversions that mix the urinary and fecal streams. This malignancy was reported in more than 10% of patients in one series. [32]  Patients younger than 25 years with ureterosigmoidostomy have a 7000-fold greater risk of adenocarcinoma of the colon than the general population (mean latency, 10 years).

Complications of short-gut syndrome are as follows:

  • Paucity of hindgut and, in many cases, limited small intestine can result in electrolyte abnormalities in patients with cloacal exstrophy
  • Dehydration is particularly a concern during an acute GI illness with diarrhea
  • Nutritional supplementation may be required
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Diet

Some young patients with cloacal exstrophy are seriously affected by short-gut syndrome and may depend on long-term supplemental parenteral nutrition for growth and development.

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Activity

The patient is kept in modified Bryant traction for 3 weeks after exstrophy closure. Alternatively, spica casts, lower-extremity wraps, and external pelvic fixators may be used, depending on the performance of osteotomies and on institutional protocols.

In childhood, the patient may have a waddling gait because of abnormal pelvic rotation, but activity is unrestricted, and no long-term orthopedic sequelae occur.

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Consultations

The following consultations may be appropriate:

  • Neonatologist for the special needs of a premature newborn and the care of a child with multiple anomalies
  • Pediatric gastroenterologist if needed for the patient with cloacal exstrophy to manage the short-gut physiology with parenteral and modified external feeding
  • Pediatric urologist or surgeon with special interest and expertise in the management of exstrophy-epispadias complex
  • Neurosurgeon for patients with cloacal exstrophy with myelodysplasia
  • Orthopedic surgeon if lower-extremity anomalies are present and if pelvic osteotomies are required for bladder closure
  • Child psychiatrist if gender conversion is considered; counseling should be offered to children struggling with incontinence and with concerns regarding how their bodies look or function differently from peers' bodies; counseling is recommended before major reconstruction for continence; teen and young adult males appear to be at increased risk for suicidal behaviors and other psychopathology when compared with unaffected peers [33]
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Long-Term Monitoring

Remaining urinary drainage catheters are removed as part of outpatient follow-up care. Prophylactic antibiotics are continued.

Lifelong follow-up care with a urologist is important to monitor for urinary tract infections, deterioration in bladder function, or damage to the kidneys and to direct appropriate surgical or medical therapy. Patients with cloacal exstrophy require long-term follow-up care with a neurosurgeon, orthopedic surgeon, and general surgeon.

Counseling for school-age children may be offered preemptively or as concerns arise. Because of the potential long-term implications of exstrophy, patient education is critical. Exstrophy support groups are established at several major medical centers.

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