Bhavik B. Shah

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.

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.

Impact of nephrectomy on long-term renal function in non-syndromic children treated for unifocal Wilms tumor

OBJECTIVE The present study is designed to assess the long-term renal function of children who underwent radical nephrectomy for unifocal Wilms tumor. METHODS A single institution retrospective cohort study of non-syndromic children treated with radical nephrectomy for unifocal Wilms tumor between 1995 and 2011 was performed to identify risk factors for decreased glomerular filtration rate (GFR). The primary endpoint was decrease in age-adjusted GFR below normal published ranges. The secondary endpoint was progression to chronic renal insufficiency (CRI). RESULTS A total of 55 patients were identified in the cohort. Eight (15%) patients exhibited decreased age-adjusted GFR during the follow-up period, with 2 (4%) progressing to CRI. Increasing time between surgery and the last known GFR follow-up was associated with decreased GFR, with the normal GFR group having median follow-up of 7.32 years versus 11.47 years (p = 0.019) in the decreased GFR group. CONCLUSIONS A trend toward decline in GFR was detected with longer follow-up. Longer follow-up may reveal that clinically significant decline in renal function occurs years following nephrectomy among a subset of Wilms tumor survivors, even among those who do not progress to end stage renal disease.

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.

Emotional and behavioral functioning in children with bladder exstrophy–epispadias complex: A developmental perspective

OBJECTIVE To evaluate the emotional and behavioral functioning of children with bladder exstrophy-epispadias complex (BEEC), taking into account developmental and gender considerations. This study also sought to overcome methodological limitations of previous studies evaluating psychological well-being of children with BEEC. METHODS Eighty-six children were consecutively evaluated using the parent report version of the Behavior Assessment System for Children during visits to a multidisciplinary urology clinic. RESULTS Results indicated normative emotional and behavioral functioning across the sample. However, there was a significant effect of age, such that older children consistently had worse internalizing symptoms and adaptive functioning. Males tended to have more externalizing problems as they aged, and also tended to have lower levels of adaptive functioning but this was independent of age. The level of psychological impairment was unrelated to the specific type of BEEC, and was also unrelated to whether or not the patient had undergone continence surgery. CONCLUSION Children with BEEC have a greater likelihood of experiencing a wide range of emotional and behavioral problems as they reach adolescence. These findings point to the need to prevent potential psychological distress by intervening with these children before they become clinically impaired.

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.

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.

Robotic Ileal Interposition for Radiation-induced Ureteral Stricture Disease

OBJECTIVE To present a novel surgical concept using preoperative imaging to estimate length needed for ileal segment. This enables robotic ileal interposition to be completed with only 1 position change during surgery. MATERIALS AND METHODS The index patient is a 69-year-old woman with a history of stage IIIB cervical cancer treated with chemotherapy and radiation 5 years prior to presentation. The patient subsequently developed a long segment stricture of the left ureter, which had been managed with ureteral stents. The patient decided to proceed with robotic ileal ureter for management of her ureteral stricture disease. We used preoperative computed tomography scan measurements to estimate length needed for ileal segment. This eliminated the need for multiple position changes during surgery. RESULTS The patient tolerated the procedure well. Estimated blood loss was 50 cc. Kidney ultrasound at 1 month post-op revealed no hydronephrosis. Renal scan at 12 months post-op revealed stable function at 32% and no evidence of obstruction. The patient reports she is doing well and is pain free at this time. CONCLUSION Robotic ileal interposition is a technically feasible procedure with good functional outcomes. By using preoperative imaging to estimate length needed for ileal segment, only 1 position change is needed during surgery.

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.