Duplicate bladder exstrophy: a unique approach to initial repair

PEDIATRIC UROLOGY

DUPLICATE BLADDER EXSTROPHY: A UNIQUE APPROACH TO INITIAL REPAIR MICHAEL D. FABRIZIO, STEPHEN E. STRUP, R. BRUCE FILMER, JOHN NOSEWORTHY, AND JOHN P. GEARHART

ABSTRACT 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. UROLOGY 53: 401–404, 1999. © 1999, Elsevier Science Inc. All rights reserved.

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xstrophy is an exceedingly rare congenital anomaly. The incidence of exstrophy-epispadias complex ranges from 1/10,000 to 1/50,000 live births and can present as a spectrum of anomalies.1,2 The surgeons’ ability to reconstruct patients with exstrophy has reached the state where both function and cosmesis are readily attainable.3 Duplicate bladder exstrophy associated with an omphalocele is an uncommon variant of exstroFrom the Department of Urology, Thomas Jefferson University, Philadelphia, Pennsylvania; duPont Hospital for Children, Wilmington, Delaware; and Department of Urology, Brady Urologic Institute School of Medicine, The Johns Hopkins University, Baltimore, Maryland Reprint requests: Stephen E. Strup, M.D., Department of Urology, Thomas Jefferson University, 11th Floor College Building, 1025 Walnut Street, Philadelphia, PA 19107 Submitted: March 4, 1998, accepted (with revisions): August 14, 1998 © 1999, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED

phy. Zivkovic4 presented a case of duplicate exstrophy and omphalocele with associated musculoskeletal defects. When Marshall and Muecke5 reported their variations in exstrophy of the bladder, only 5 cases of duplicate exstrophy had been reported. In 1990, Sheldon et al.6 described a newborn with an exstrophic plate with a coloniform projection and underlying bladder. Turner et al.2 were the first to describe a duplicate exstrophy with the exstrophic bladder serving the left renal system and a covered bladder serving the right. According to Kogan et al.,7 true duplicate exstrophy is represented by the presence of bladder mucosa on the anterior abdominal wall without any communication with the intact bladder. The investigators describe another variation of duplicate bladder exstrophy and the unique approach to initial repair of the bladder and penis. Long-term follow-up is reported in detail. 0090-4295/99/$20.00 PII S0090-4295(98)00482-8 401

FIGURE 1. Intravenous urogram showing two normal upper tracts and no obvious lower tract anomaly other than simple exstrophy.

FIGURE 2. Opening at bladder neck cannulated under fluoroscopy revealing a larger duplicate bladder.

MATERIAL AND METHODS A newborn male was born at 30 weeks’ gestation following a normal pregnancy. At birth, it was thought that a bladder exstrophy and a ruptured omphalocele were present. The omphalocele was closed emergently shortly after birth, and bilateral orchiopexies were performed at that time. Because of the large size of the omphalocele and the small dimensions of the exstrophied bladder, the decision was made to delay closure of the bladder until there was interval growth of the child and hopefully the bladder. Intravenous urography revealed two normal upper tracts and normal ureters with no obvious lower tract anomaly other than simple bladder exstrophy (Fig. 1). At 8 months of age, the patient underwent bladder closure, using bilateral supra-acetabular innominate osteotomies and vertical iliac wing osteotomies. At the time of bladder closure, the exstrophic plate was still quite small. The left ureteral orifice was identified and catheterized. The right hemitrigone could not be clearly identified. An opening at the bladder neck was then cannulated under fluoroscopy and revealed a large saccular system consistent with a duplicate bladder exstrophy (Fig. 2). Cystoscopy identified a bladder of reasonable size and a right ureteral orifice, which was cannulated with a 3F catheter. The skin adjacent to the exstrophic plate was incised, and dissection proceeded posteriorly in the midline until the duplicated bladder was identified. It was determined that the exstrophic plate could be used for construction of a neourethra and concomitant epispadias repair (Fig. 3). The left ureter was mobilized, and a flap of exstrophic bladder plate measuring 2.5 3 2.0 cm was isolated (Fig. 3). After mobilizing the prevesical space, the duplicate bladder was opened, and the left ureter was reimplanted into the posterior duplicate bladder. The exstrophic bladder plate was tubularized into a neourethra over a 12F red rubber catheter. Penile lengthening was performed by mobilizing the bladder neck and prostatic urethra. The urethra was transected just distal to 402

FIGURE 3. Mobilized exstrophic plate and shallow urethral groove leading into duplicate bladder neck (noted by metal sound). the level of the prostate, which was actually just distal to the opening for the internal bladder. The urethral plate was dissected free from the corpora. The corpora were separated and dissected free from the midline, and the tubularized bladder plate was brought between the corpora and out to the tip of the glans without tension. The corporal bodies were rotated over the bladder tube (neourethra) to help straighten the penis. Glandular wings were developed to allow placement of the neourethra into the urethral groove (Fig. 3). The corporal bodies were reapproximated medially. Figure 4 illustrates the completed reconstruction of the urethra and phallus as well as the left ureter and bladder. Finally, the pelvic ring was reconstructed, and the abdominal wall musculature and skin were closed.

RESULTS The patient progressed well after initial closure and epispadias repair. There were no postoperative UROLOGY 53 (2), 1999

FIGURE 4. Completed reconstruction. (A) Left reimplanted ureter. (B) Suprapubic tube into covered bladder. (C) Neourethra from exstrophic plate. (D) Reconstructed phallus.

FIGURE 5. Patient at 4.5 years of age.

strictures or fistula formation. However, more than 2 years after his initial reconstruction, he had a bladder capacity of only 50 mL with bilateral grade 2 vesicoureteral reflux and continuous incontinence. The patient underwent a Young-Dees-Leadbetter bladder neck reconstruction, augmentation cystoplasty, appendicovesicostomy, and bilateral ureteral reimplantation at 3.5 years of age. Most recently, the child has required one periurethral injection of collagen for intermittent incontinence and is now dry between catheterizations. His urethra is of adequate length, and the phallic reconstruction is both functional and cosmetically sound (Fig. 5) with straight erections witnessed by the parents on several occasions. COMMENT There have been fewer than 20 cases of duplicate bladder exstrophy described in the literature, emphasizing the operative and embryologic significance of this case. Marshall and Muecke5 have suggested that an overdeveloped cloacal membrane UROLOGY 53 (2), 1999

prevents the lateral mesoderm from migrating medially, preventing its insertion between the ectodermal and endodermal layers. Thus, numerous variations in the exstrophy complex can develop. The superior vesical fissure represents one such variation in which the cloacal membrane opens only in the upper portion.5,8 If the superior vesical fissure fuses, embryologic remnants of bladder may persist externally, leading to duplication of the bladder. Kogan et al.7 refer to duplicate exstrophy as a form of incomplete exstrophy with bladder mucosa on the anterior abdominal wall without any communication with the intact bladder. Similar variations have been described by Kittredge and Bradburn9 who presented a patient with a small communication between the exstrophic bladder and the covered bladder. Sheldon et al.6 believe that duplicate bladder exstrophy can be divided into two categories. The first is associated with a suprapubic exstrophic mucosal plate and a covered bladder. The phallus is relatively well formed in this setting. The second type is true duplication with the classic findings of exstrophy complex. Turner et al.2 describe 3 patients with vesical duplication; 2 were covered by skin but had the associated musculoskeletal anomalies of exstrophy, and 1 patient had an exstrophic bladder associated with the left collecting system and a covered bladder that served the right kidney. In this series, all patients were girls and had at least one damaged renal unit. Finally, Ellis10 described a boy with duplicate bladder exstrophy with the closed bladder serving both renal units. Our patient had several interesting findings that warrant discussion. There was an associated omphalocele that was closed at birth. The penis was epispadic, and bilateral cryptorchism was noted. His bladder was not closed at birth because of the associated omphalocele and small size of the exstrophic bladder plate, although the importance on early closure and subsequent continence is noted.11 Large omphaloceles are rarely associated with the exstrophic complex, and few have been described in association with duplicate bladder exstrophy.4 As noted previously, if the ureteral orifices cannot be clearly identified at the time of operation, duplicate exstrophy must be considered. Although this was clearly a significantly different variant of bladder exstrophy, the investigators believed that concomitant osteotomy would add to the security of the bladder and abdominal wall closure. The importance of this fixation of the pelvic ring is underscored by the higher incidence of incontinence in those patients without fixation.12,13 In this particular case, the exstrophic segment allowed a rather large (2.5 cm by 2 cm) flap to be tubularized for repair of the epispadic urethra. The 403

plate was chosen as a urethral substitute because of its excellent mucosal characteristics and the lack of muscle bulk, thus allowing easy tubularization. Because of the rarity of duplicate exstrophy, longterm results after bladder neck repair remain inconclusive. However, because this bladder remained small, augmentation was required along with bladder neck and continent stoma construction. The reason this duplicate exstrophy bladder failed to grow is unknown. When this duplicate exstrophy bladder was discovered and treated, it was assumed that the bladder would likely grow to sufficient size to allow future bladder neck reconstruction. This assumption is made in the treatment of classic exstrophy as well, even though some exstrophied bladders just do not grow sufficiently. There is evidence that collagen deposition is altered in the exstrophied bladder,14 which may be one factor contributing to lack of bladder growth in some exstrophy patients. It is unlikely that ureteral reimplantation in a transtrigonal fashion would be a contributing factor in this case, especially because the left ureter did not have to be dissected free from the posterior bladder before reimplantation. For those exstrophied bladders that do not grow to an acceptable volume, there is ample evidence that cystoplasty produces excellent results.15 CONCLUSIONS Duplicate bladder exstrophy is an exceedingly rare condition that can require modifications of well-known reconstructive techniques. The investigators present a unique method of reconstructing the urethra by tubularizing the exstrophic plate while sparing the internal covered bladder. This case demonstrates the wide spectrum of anomalies that can occur in the exstrophy-epispadias complex.

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UROLOGY 53 (2), 1999