Simultaneous second-stage Fowler-Stephens Orchiopexy and microvascular testicular autotransplantation in a patient with prune belly syndrome with cryptorchidism.

We present a case of simultaneous second-stage Fowler-Stephens Orchiopexy (FSO) with microvascular testicular autotransplantation for cryptorchidism and in a patient with prune belly syndrome. At 5 months old, the patient underwent laparoscopic bilateral first-stage FSO with the right testicle located 1 cm from the liver and the left slightly more caudal. An ultrasound on postoperative Day 72 following second-stage FSO and microvascular autotransplantation showed patent testicular vasculature. Our experience shows that this combination technique is safe and effective to supercharge the testicle and augment collateral vessels if clinical suspicion for monotherapy failure is high.

3-Dimensional Magnetic Resonance Imaging Guided Pelvic Floor Dissection for Bladder Exstrophy: A Single Arm Trial.

PURPOSE: We determined the safety and efficacy of intraoperative magnetic resonance imaging guided surgical reconstruction of bladder exstrophy for the identification of the urogenital diaphragm fibers and the thickened muscular attachments between the posterior urethra, bladder plate and pubic rami. MATERIALS AND METHODS: Institutional review board and U.S. Food and Drug Administration approval was obtained for the use of Brainlab (Munich, Germany) intraoperative magnetic resonance imaging guided navigation of the pelvic floor anatomy during closure of classic bladder exstrophy and cloacal exstrophy at our institution. Preoperative pelvic 3-dimensional magnetic resonance imaging was obtained 1 day before closure in patients undergoing pelvic osteotomies. Intraoperative registration was performed after preoperative planning with a pediatric radiologist using 5 anatomical landmarks immediately before initiation of surgery. Accuracy of pelvic anatomy identification was assessed by 2 pediatric urological surgeons and 1 pediatric radiologist. RESULTS: In 43 patients with classic bladder exstrophy and 4 patients with cloacal exstrophy closed at our institution, Brainlab technology was used successfully to navigate and guide the dissection of the pelvic floor intraoperatively. In all patients there was 100% accuracy in the correlation of gross anatomical landmarks with 3-dimensional magnetic resonance imaging identified landmarks intraoperatively, and all patients had successful closure without any major complications. CONCLUSIONS: Brainlab intraoperative 3-dimensional magnetic resonance imaging guided pelvic floor navigation and dissection is an effective way to accurately identify pelvic anatomy during classic bladder exstrophy and cloacal exstrophy closure. This technology offers a unique opportunity for surgical skill education in this complex reconstructive operation.

Perioperative Analgesic Management of Newborn Bladder Exstrophy Repair Using a Directly Placed Tunneled Epidural Catheter with 0.1% Ropivacaine.

Effective multimodal analgesia and sedation reduce the postoperative morbidity and mortality associated with newborn bladder exstrophy repair. Epidural analgesia is safe and effective for major surgery in neonates and infants, reducing the need for muscle relaxants, opioids, and ventilator support postoperatively. The risk of epidural catheter colonization typically dictates removal after 3 to 5 days. Tunneling the catheter subcutaneously reduces the risk of colonization, providing prolonged analgesia for patients requiring an extended immobilization to prevent compromise of the repair. In this report, we describe the postoperative analgesic management of an infant undergoing bladder exstrophy repair using a directly placed tunneled epidural catheter with ropivacaine 0.1% infusion. Because of the prolonged infusion, we also monitored plasma ropivacaine levels to preclude systemic toxicity from local anesthetic overdose.

Pediatric surgical complications of major genitourinary reconstruction in the exstrophy-epispadias complex.

PURPOSE: Urinary continence is the goal of exstrophy-epispadias complex (EEC) reconstruction. Patients may require a continent urinary diversion (CUD) if they are a poor candidate for bladder neck reconstruction or are receiving an augmentation cystoplasty (AC) or neobladder (NB). This study was designed to identify the incidence of surgical complications among various bowel segments typically used for CUD. METHODS: A prospectively kept database of 1078 patients with EEC at a tertiary referral center from 1980 to 2012 was reviewed for major genitourinary reconstruction. Patient demographics, surgical indications, perioperative complications, and outcomes were recorded. RESULTS: Among reviewed EEC patients, 134 underwent CUD (81 male, 53 female). Concomitant AC was performed in 106 patients and NB in 11. Median follow up time after initial diversion was 5 years. The most common CUD bowel segments were appendix and ileum. The most common surgical complications after CUD were small bowel obstruction, post-operative ileus, and intraabdominal abscess. There was a significantly increased risk in the occurrence of pelvic or abdominal abscess when colon was used as a conduit compared to all other bowel segments (OR=16.7, 95% CI: 1.16-239) and following NB creation compared to AC (OR=39.4, 95% CI: 3.66-423). At postoperative follow-up, 98% of patients were continent of urine via their stoma. CONCLUSION: We report the largest series to date examining CUD in the EEC population. The increased risk of abdominal and pelvic abscesses in patients who receive a colon CUD and undergo NB compared to AC indicates that while surgical complications following major genitourinary reconstruction are rare, they do occur. Practitioners must be wary of potential complications that are best managed by a multi-disciplinary team approach.

Duplicated pelvic floor musculature and diastematomyelia in a cloacal exstrophy patient.

Cloacal exstrophy is the most severe and rare form of the exstrophy-epispadias complex, presenting with exposed bladder halves extruding through an abdominal wall defect and variable genitourinary, gastrointestinal, musculoskeletal, and neurological defects. The authors report magnetic resonance imaging findings of a neurologically-intact, 24-month-old female with cloacal exstrophy who presented with anterior spinal dysraphism and diastematomyelia and duplicate pelvic floor musculature. The constellation of defects suggests a common genetic, biochemical, and embryological origin for duplication of the bladder, spinal cord, and pelvic floor muscles occurring in the fourth week of gestation.

Urologic complications of major genitourinary reconstruction in the exstrophy-epispadias complex.

OBJECTIVE: To present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy-epispadias complex (EEC) population. METHODS: Retrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed. RESULTS: Among the EEC patients reviewed, 133 underwent CS (80 male, 53 female). Mean follow-up time after initial continent stoma was 5.31 years (range: 6 months to 20 years). Appendix and tapered ileum were the primary bowel segments used for the continent channel and stoma in the EEC population. The most common stomal complications in this population were stenosis, incontinence, and prolapse. Seventy-nine percent of EEC CS patients underwent AC primarily done with sigmoid colon or ileum. Eleven patients (8%) underwent neobladder creation with either colon or a combination of colon and ileum. Bladder calculi, vesicocutaneous fistula, and pyelonephritis were the most common non-stomal complications. Stomal ischemia was significantly increased in Monti ileovesicostomy compared to Mitrofanoff appendicovesicostomy in classic bladder exstrophy patients (p = 0.036). Furthermore, pyelonephritis was more than twice as likely in colonic neobladder than all other reservoir tissue types in the same cohort (OR = 2.53, 95% CI: 1.762-3.301, p < 0.001). CONCLUSIONS: To the best of the authors' knowledge, this is the largest study examining catheterizable stomas in the exstrophy population. While Mitrofanoff appendicovesicostomy is preferred to Monti ileovesicostomy because it is technically less challenging, it may also confer a lower rate of stomal ischemia. Furthermore, even though ileum or colon can be used in AC with equally low complication rates, practitioners must be wary of potential urologic complications that should be primarily managed by an experienced reconstructive surgeon.

Initial bladder closure of the cloacal exstrophy complex: outcome related risk factors and keys to success.

PURPOSE: This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes. METHODS: One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients. Twenty-six patients with a history of failed initial bladder closure were compared to 34 with a history of successful initial bladder closure. Univariate logistic regression analysis was used to compare the two groups. RESULTS: Median follow up time after initial closure was 9years (range: 13months-29years). A 1cm increase in pre-closure diastasis resulted in a 2.64 increase in the odds of initial closure failure (p=0.004). Protective strategies against failure included delaying closure (per month) (OR=0.894, p=0.009), employing pelvic osteotomies (OR=0.095, p<0.001), and applying external fixation (OR=0.024; p=0.001). Among patients who underwent osteotomy (31% of patients in the failed group, 82% in the successful group), a longer delay between osteotomy and closure (OR=0.033; p=0.005) was also protective against failure. CONCLUSION: Patients with a large diastasis are more likely to fail initial closure. Delaying initial closure for at least 3months, performing pelvic osteotomy, and using an external fixation device post-operatively are strategies that improve closure success.

Role of urethral bulking agents in epispadias-exstrophy complex patients.

OBJECTIVE: Injection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population. MATERIALS AND METHODS: A review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy-epispadias complex who underwent injection of UBA between 1980 and 2008. RESULTS: Among 66 patients with a median follow-up of 8 years, 41 underwent injections prior to BNR, and 25 had injections after BNR. Only 24% of patients who underwent injections prior to BNR were continent on last follow-up. Among 25 patients who underwent BNR prior to injection(s), 16 were partially continent and nine were incontinent prior to first injection. Patients who were partially continent attained social continence (dry interval greater than 3 h) at a significantly higher rate than those who were incontinent (63% vs. 13%, p = 0.047). No patient with cloacal exstrophy in either group attained urethral continence. CONCLUSION: UBAs do not appear to have a role prior to BNR. However, they may provide benefit when given adjunctively following BNR in patients who are partially continent.

Bladder capacity as a predictor of voided continence after failed exstrophy closure.

OBJECTIVE: To evaluate potential predictors of voided continence among bladder exstrophy patients with a history of a failed closure. PATIENTS AND METHODS: The authors reviewed all patients who underwent a bladder neck reconstruction (BNR) with a history of one or more failed exstrophy closures between 1979 and 2007. The following data were collected for each patient: number of failures, site of surgery, mode of failure, presence of osteotomy, bladder capacity, need for additional procedures, and continence status. RESULTS: Among patients who underwent successful reclosure following one or more failed closures, 52 patients underwent BNR, and 24 (46%) were continent at last follow-up. Bladder capacity was the only variable predictive of voided continence. The median bladder capacity at the time of BNR differed between those who achieved continence (100 mL) and those who did not (65 mL) (p = 0.005). ROC analysis showed an optimal pre-BNR bladder capacity cutoff for predicting future BNR success of between 80 and 100 mL. CONCLUSION: As previously shown in patients with successful primary closure of exstrophy, these data suggest that bladder capacity also has predictive value in the success of BNR after failed exstrophy closure.

Utility of screening ultrasound after first febrile UTI among patients with clinically significant vesicoureteral reflux.

OBJECTIVE: To assess the sensitivity and negative predictive value (NPV) of screening renal and bladder ultrasound (RBUS) after initial febrile urinary tract infection (UTI) among patients with clinically significant vesicoureteral reflux (VUR). METHODS: A retrospective review was performed of all children <2 years of age who presented with a febrile UTI between 2004 and 2011. The sensitivity and NPV of initial RBUS was calculated among patients who were found to have high-grade (IV-V) VUR. Additionally, initial RBUS among patients with evidence of photopenia on dimercaptosuccinic acid (DMSA) scan or who underwent surgical intervention were reviewed. RESULTS: One hundred forty-four patients with febrile UTI were identified; available RBUS, voiding cystourethrogram (VCUG), and DMSA results for each kidney were reviewed. One hundred fifty-eight kidneys had evidence of VUR on VCUG, and initial RBUS demonstrated abnormality in 25 (sensitivity 0.17). Forty-five kidneys had high-grade VUR and RBUS revealed abnormality in 16 (sensitivity 0.36). One hundred seventy-eight kidneys had no evidence of abnormality on initial RBUS, and 136 (76%) were found to have VUR (NPV 0.24), of which 31 had high-grade VUR (NPV 0.83). Seven kidneys had scarring on DMSA and initial RBUS was normal in 4 (57%). Twelve of 19 patients (63%) who eventually underwent surgical intervention had a normal initial RBUS. CONCLUSION: RBUS has poor sensitivity and NPV for detecting high-grade VUR in patients <2 years who present with a febrile UTI. A significant number of patients who were diagnosed with high-grade VUR, renal scarring, or underwent surgical correction of VUR had a negative screening RBUS.

Modern management of bladder exstrophy repair.

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy-epispadias complex, the modern management of bladder exstrophy, and potential surgical complications.

Novel mutation among two sisters with 17ß hydroxysteroid dehydrogenase type 3 deficiency.

The clinical presentations of 17ß hydroxysteroid dehydrogenase type 3 (17ß-HSD3) deficiency, 5α-reductase type 2 deficiency, and complete androgen insensitivity syndrome can be similar. However, those with 17ß-HSD3 deficiency and 5α-reductase type 2 deficiency will develop virilization and should undergo gonadectomy after genetic testing before the age of puberty if reared in the female sex. Two sisters were initially diagnosed with complete androgen insensitivity syndrome as young children after testes were discovered during hernia surgery. Virilization occurred in both sisters during puberty, and a diagnosis of 17ß-HSD3 deficiency was suspected. Confirmatory diagnosis through gene sequencing identified a heterozygous mutation for both a known splicing mutation and a previously unreported amplification mutation of the HSD17B3 gene.

Review and management of 46,XY disorders of sex development.

Disorders of sex development (DSD) among 46,XY individuals are rare and challenging conditions. Abnormalities of karyotype, gonadal formation, androgen synthesis, and androgen action are responsible for the multiple disorders that result in undervirilization during development. Phenotypic appearance and timing of presentation are quite variable. The focus of treatment has shifted from early gender assignment and corrective surgery to careful diagnosis, proper education of patients and their families, and individualized treatment by a multi-disciplinary team. The modern management of these patients is difficult and controversial. Conflicting data on long-term outcomes of these individuals have been reported in the literature. The various etiologies of 46,XY DSD, current approaches to diagnosis and treatment, and reported long-term results are reviewed.

Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy.

PURPOSE: Male patients with bladder and cloacal exstrophy are born with demure genitalia and often desire phalloplasty during late adolescence or early adulthood. Radial forearm free flap phalloplasty was used successfully for congenital aphallia in a few small series. We present a series of patients treated with phalloplasty using a radial forearm free flap after the repair of bladder and cloacal exstrophy. MATERIALS AND METHODS: We reviewed the records of 10 patients who underwent radial forearm free flap phalloplasty between 2007 and 2012. Indications for phalloplasty were classic bladder exstrophy in 8 cases and cloacal exstrophy in 2. Nine patients underwent prior urinary diversion and 1 underwent urethroplasty at phalloplasty. In each case we reviewed the details of prior exstrophy repair, flap size, donor and recipient vessels, complications, need for subsequent surgeries and self-reported sensation. RESULTS: The graft survived in all 10 patients. Short-term complications requiring surgical intervention developed in 2 patients. Five patients subsequently underwent placement of a penile prosthesis and 2 devices were removed secondary to erosion. All 10 patients reported protective sensation and erogenous sensation with ability to achieve orgasm at last followup. CONCLUSIONS: Radial forearm free flap phalloplasty results in a sensate, cosmetic neophallus. No patient had long-term complications related to phalloplasty but complications related to penile prostheses continue to be a challenging aspect of phalloplasty. The long-term results of the forearm free flap are encouraging in this series of patients with bladder and cloacal exstrophy who desired phalloplasty.

Persistent vesicocutaneous fistula after repair of classic bladder exstrophy: a sign of failure?

OBJECTIVE: To describe the potential predisposing clinical characteristics at initial closure, the treatments of and outcomes associated with vesicocutaneous fistula (VCF) after primary bladder closure. MATERIALS AND METHODS: Eighteen patients were referred for VCF after primary bladder exstrophy closure. Aspects from the primary closure such as gender, timing of diagnosis and repair, osteotomies, pelvic immobilization, layers of closure, use of tissue adjuncts, and complications in addition to details from their required treatment of VCF were retrospectively extracted from the medical record. RESULTS: A diagnosis of failed closure was made at the time of evaluation in 13 of 18 patients who presented with VCF. All 13 patients underwent delayed single-layer closures and had a widened pubic diastasis. Five patients with secure closures who underwent successful simple fistula repairs were all closed early in life, had multi-layered closures with pelvic osteotomies, and had minimal change in pubic diastasis. CONCLUSION: VCF may represent a failed bladder closure. Factors which may suggest failure are a fistula tract in the lower abdominal midline between the pubic rami, a pubic diastasis increased from pre-closure measurement, and cystoscopic evidence of an anteriorly positioned bladder.

Perioperative management of classic bladder exstrophy.

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall muscles, and pelvic structures. Modern surgical repairs focus on reconstruction of the bladder and its adjacent structures, with the goal of achieving urinary continence, a satisfactory cosmetic result, and a high quality of life. Complex surgery in neonates and young children, as well as a prolonged postoperative course require close collaboration between surgeons, anesthesiologists, intensivists, pediatricians, and an experienced nursing staff. This article will review the spectrum of bladder exstrophy anomalies, the surgical repair, and the perioperative interdisciplinary management.

Technique for preservation of penile skin in genital reconstruction: free graft to the scrotum.

OBJECTIVE: To describe a new technique to preserve the penile epithelium with good cosmetic results when additional surgery is a significant possibility. Complex genital reconstruction can require multiple procedures. METHODS: Seven patients underwent grafting of the penile epithelium to the scrotum during genital reconstruction to bank the tissue for potential future use. The graft size was 8-40 mm in the maximal length. RESULTS: The median age at surgery was 8.1 months. The median interval to the last follow-up examination after surgery was 13.5 months. All 7 patients demonstrated excellent wound healing with good cosmetic results. CONCLUSION: In patients with complex genital anomalies, nonhair-bearing epithelium is a valuable asset. At times, the paucity of available local tissue necessitates autografting with nongenital epithelium. The need for nongenital tissue can be reduced if excess penile skin is banked for potential use at a later date. We describe a technique in which the penile skin is preserved for potential future reconstruction using free grafting to the scrotum.

Sexual function after male radical cystectomy in a sexually active population.

OBJECTIVES: To assess sexual dysfunction in sexually active men after radical cystectomy (RC) and to determine whether sildenafil citrate can improve erectile dysfunction after surgery. METHODS: The baseline and follow-up data from 49 sexually active male patients (mean age 57.8 +/- 9.1 years) undergoing RC (1995 to 2002) were obtained. Of the 49 patients, 16 (33%) had undergone nerve-sparing RC; 38 (78%) had undergone orthotopic diversion; 8 (16%) had undergone ileal conduit diversion; and 3 (6%) had undergone cutaneous continent diversion. The data were assessed using the abridged 5-item International Index of Erectile Function questionnaire, referred to as the Sexual Health Inventory for Men (SHIM). RESULTS: At a mean follow-up of 47.6 +/- 22.7 months, the total mean SHIM score decreased from 22.08 +/- 3.96 to 4.33 +/- 5.72 after RC (P <0.05). Of the 49 patients, 42 (86%) did not have erections sufficient for vaginal penetration. Of these 42 patients, 22 (52%) tried sildenafil citrate. Of these 22 patients, only 2 (9%) responded positively, with a total mean SHIM score of 23.50 +/- 2.12. Although the mean SHIM score after orthotopic substitution (5.24 +/- 6.21) was statistically significant compared with that after ileal conduit (1.13 +/- 0.33) and cutaneous continent (1.33 +/- 0.58) diversions, this was not clinically significant. CONCLUSIONS: Male erectile dysfunction after RC is a prevalent problem. In our series, only 9 (14%) of 49 sexually active men were potent after surgery. Of these 9 potent patients, 8 (89%) had undergone nerve-sparing RC. Of concern, only 52% of the patients with erectile dysfunction sought treatment after RC.

Female sexual dysfunction after radical cystectomy: a new outcome measure.

OBJECTIVES: To assess, in our contemporary radical cystectomy (RC) series, female sexual dysfunction and whether the type of diversion affected the occurrence of sexual dysfunction. Outcome data after RC with and without orthotopic diversion has focused primarily on cure, urethral recurrence, and continence. METHODS: The baseline and follow-up data from 27 sexually active female patients (mean age 54.79 +/- 12.7 years) who underwent RC from 1997 to 2002 for transitional cell carcinoma of the bladder (16 with Stage T1-T2 and 11 with Stage T3-T4) were obtained. Thirteen patients were premenopausal before RC. Of the 27 patients, 10 (37%) underwent Studor orthotopic diversion, 7 (26%) Indiana cutaneous diversion, and 10 (37%) ileal conduit diversion. A 10-item version of the self-administered Index of Female Sexual Function questionnaire was used to assess sexual dysfunction. The specific domains analyzed in the Index of Female Sexual Function include the degree of vaginal lubrication, ability to achieve orgasm, degree of pain during intercourse, overall sexual desire and interest, and overall sexual satisfaction, with responses graded on a scale of 1 (almost never, never) to 5 (almost always, always). RESULTS: With a mean follow-up of 24.2 months (range 15 to 65.1), the total mean baseline Index of Female Sexual Function score decreased from 17.4 +/- 7.23 to 10.6 +/- 6.62 after RC (P < or =0.05). The most common symptoms reported by the patients included diminished ability or inability to achieve orgasm in 12 (45%), decreased lubrication in 11 (41%), decreased sexual desire in 10 (37%), and dyspareunia in 6 patients (22%). Only 13 (48%) of the 27 patients were able to have successful vaginal intercourse, with 14 (52%) reporting decreased satisfaction in overall sexual life after RC. Eight partners (30%) had a decrease in desire for sexual activity owing to apprehension after cancer diagnosis and treatment. Although the numbers were small, the preliminary data suggested no differences in sexual function between patients undergoing Studor orthotopic diversions and those undergoing Indiana cutaneous diversions. CONCLUSIONS: Sexual dysfunction is a prevalent problem after female RC. The nature of the dysfunction involves multiple domains, including decreased orgasm, decreased lubrication, lack of sexual desire, and dyspareunia. Our early results suggest that the type of continent diversion does not affect sexual function. Surgical modifications such as urethral and vaginal sparing, neurovascular preservation, and tubular vaginal reconstruction sparing may improve female sexual function after RC.