Novel Anatomical Observations of the Prostate, Prostatic Vasculature and Penile Vasculature in Classic Bladder Exstrophy Using Magnetic Resonance Imaging.

PURPOSE: We used magnetic resonance imaging to define the innate pelvic neurovascular course and prostatic anatomy in infants with classic bladder exstrophy before the pelvis was altered by surgery. MATERIALS AND METHODS: Pelvic magnetic resonance imaging was performed in male infants with classic bladder exstrophy and compared to a group of age matched controls. Data collected included prostatic dimensions as well as course of the prostatic artery, periprostatic vessels and pudendal neurovasculature. RESULTS: The prostate was larger in the transverse (p <0.001) and anteroposterior (p <0.001) dimensions in patients with classic bladder exstrophy compared to those with normal prostates but was smaller in the craniocaudal dimension (p <0.001). This finding resulted in a larger calculated prostate volume in patients with classic bladder exstrophy compared to controls (p = 0.015). The pelvic vasculature and prostatic artery followed a similar course in patients with classic bladder exstrophy and controls. Relative to each other, the lateral to medial course of the prostatic arteries in males with classic bladder exstrophy was less pronounced than in normal males. A similar externally rotated pattern was seen when both sides of the pudendal vasculature were compared in males with classic bladder exstrophy. CONCLUSIONS: The prostate in infants with classic bladder exstrophy has a consistent configuration and dimensions that differ from those in normal infants. When both sides are compared, the periprostatic vasculature and penile sensory neurovascular bundles are externally rotated in infants with classic bladder exstrophy. However, these components course along the same landmarks as in normal patients.

Novel Observations of Female Genital Anatomy in Classic Bladder Exstrophy Using 3-Dimensional Magnetic Resonance Imaging Reconstruction.

PURPOSE: Understanding the distinct female anatomy in classic bladder exstrophy is crucial for optimal reconstructive and functional outcomes. We present novel quantitative anatomical data in females with classic bladder exstrophy before primary closure. MATERIALS AND METHODS: 3-Dimensional reconstruction was performed in patients undergoing pelvic magnetic resonance imaging, and pelvic anatomy was characterized, including measurements of the vagina, cervix and erectile bodies. RESULTS: We examined magnetic resonance imaging of 5 females (mean age 5.5 months) with classic bladder exstrophy and 4 age matched controls (mean age 5.8 months). Mean distance between the anal verge and vaginal introitus was greater in patients with classic bladder exstrophy (2.43 cm) than in controls (1.62 cm). Mean total vaginal length in patients with classic bladder exstrophy was half that of controls (1.64 cm vs 3.39 cm). All 4 controls had posterior facing cervical ora, while 4 of 5 females with exstrophy had anterior facing cervical ora located in the anterior vaginal wall. Lateral deviation of the cervical ora was also seen in all 5 patients with classic bladder exstrophy but in only 1 control. Clitoral body length was comparable in both groups (26.2 mm and 28.0 mm). However, the anterior cavernosa-to-posterior (pelvic rami associated) cavernosa ratio was much greater in patients with classic bladder exstrophy (6.4) compared to controls (2.5). CONCLUSIONS: This study uncovers the uniquely novel finding that contrary to their male counterparts, females with classic bladder exstrophy have the majority of the clitoral body anterior to the pelvic attachment. This discovery has surgical and embryological implications.

The Role of Human Acellular Dermis in Preventing Fistulas After Bladder Neck Transection in the Exstrophy-epispadias Complex.

OBJECTIVE: To evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition. METHODS: A prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas. RESULTS: In total, 147 EEC patients underwent a BNT with a mean follow-up time of 6.9 years (range 0.52-23.35 years). There were 124 (84.4%) classic exstrophy patients, 22 (15.0%) cloacal exstrophy patients, and 1 (0.7%) penopubic epispadias patient. A total of 12 (8.2%) BNTs resulted in fistulization, including 4 vesicoperineal fistulas, 7 vesicourethral fistulas, and 1 vesicovaginal fistula. There were 5 (22.7%) fistulas in the cloacal exstrophy cohort and 7 (5.6%) fistulas in the classic bladder exstrophy cohort (P = .019). Using either HAD or native tissue flaps resulted in a lower fistulization rate than using no interposed layers (5.8% vs 20.8%; P = .039). Of those with HAD, the use of a fibrin sealant did not decrease fistulization rates when compared to HAD alone (6.5% vs 8.8%, P = .695). There was no statistical difference in surgical complications between the use of HAD and native flaps (8.6% vs 5%, P = .716). CONCLUSION: Use of soft tissue flaps and HAD is associated with decreased fistulization rates after BNT. HAD is a simple option and an effective adjunct that does not require harvesting of tissues in patients where a native flap is not feasible.

Bladder Re-augmentation in Classic Bladder Exstrophy: Risk Factors and Prevention.

OBJECTIVE: To characterize the causes of re-augmentation in patients with classic bladder exstrophy (CBE). METHODS: A prospectively maintained institutional database of 1327 exstrophy-epispadias complex patients was reviewed for patients with CBE who underwent more than 1 augmentation cystoplasty (AC) procedure. Data regarding bladder capacities, complications following AC, and reasons for re-augmentation were evaluated. RESULTS: A total of 166 patients with CBE underwent AC. Of these, 67 (40.4%) were included in the control group and 17 (10%) patients underwent a re-augmentation. There were several indications for re-augmentation including continued small bladder capacity (17 of 17), inadequate bladder necks (8 of 17), failed rattail augmentation (2 of 17), stomal incontinence (1 of 17), a urethrocutaneous fistula (1 of 17), and an hourglass augmentation (1 of 17). Of note, 5 of the 17 patients (29%) had a re-augmentation procedure with a ureteral reimplantation. The sigmoid colon was the most commonly used bowel segment in the failed initial AC (8 patients), whereas the ileum was the most commonly used segment during re-augmentation (12 patients). In the re-augmentation cohort, the mean amount of bowel used during the first AC procedure was 12 cm (standard deviation [SD] 3.6) compared with 19 cm (SD 5.0) during re-augmentation. The mean amount of bowel used for control group augmentations was 20.8 cm (SD 4). The mean re-augmentation preoperative bladder capacity of 100 mL (SD 60) immediately increased after re-augmentation to 180.8 mL (SD 56.4) (P = .0001). CONCLUSION: Bladder re-augmentation is most commonly required in the setting of a small bladder capacity after an initial AC, when an insufficient amount of bowel is used during the first AC procedure.

Pelvic and lower extremity immobilization for cloacal exstrophy bladder and abdominal closure in neonates and older children.

INTRODUCTION: Successful bladder closure in cloacal exstrophy (CE) is best accomplished through a multidisciplinary team and attention to pre- and postoperative technique. This study from a high volume exstrophy center investigates outcomes and complications of primary and reoperative bladder closures in patients immobilized with spica cast or patients with external fixation (EF) and skin traction. METHODS: The authors reviewed an institutionally approved and daily updated database of 1311 patients with exstrophy-epispadias complex and identified patients with cloacal exstrophy born between 1975 and 2015 who had undergone primary or reoperative bladder closures. Only the closures that used spica casting or external fixation were included for analysis. Demographic, operative, and outcomes data were compared between patients with spica cast only and patients with external fixation and skin traction. RESULTS: Out of 140 patients with CE or a CE variant, a total of 71 patients with 94 bladder closures (66 primary and 28 reoperative) met inclusion criteria. Median follow-up time was 8.8 years (range 1.5-29.1). There were 37 closures performed at the authors' institution and 58 from outside hospitals. Pelvic osteotomy was undertaken in 66 (70.2%) of all closures, and in 36 (97.3%) of closures at the authors' institution. Postoperative immobilization was achieved with spica cast alone in 46 (48.9%) closures, external fixation and skin traction in 43 (45.7%), and spica cast and external fixation in 5 (5.3%) closures. For all closures, there were 33 failures (71.7%) among those immobilized with spica cast alone versus 4 failures (9.3%) for those immobilized with external fixation and skin traction (p<0.001). When restricted to closures performed with osteotomy, the failure rates were 50.0% and 9.3% respectively (p=0.002). There was minimal differences in complication rates between spica and external fixation groups (8.7% versus 23.3%, p=0.059). CONCLUSION: Failure of CE closure can occur with any form of pelvic and lower extremity immobilization. This study, however, provides continued evidence that external fixation with skin traction is an optimal, secure technique (3.8% failure rate) for postoperative management in an older child (1-2 years). LEVEL OF EVIDENCE: Level III, Retrospective comparative study STUDY TYPE: Therapeutic study.