Repeat pelvic osteotomy in patients with failed closure of bladder exstrophy: applications and outcomes

Background/Purpose

In patients with failed primary or secondary closure of bladder exstrophy, repeat osteotomy is useful in facilitating reconstruction. The clinical consequences of repeated surgical disruption of the pelvic ring have not been carefully described, however.

Methods

We reviewed our experience with exstrophy patients who had undergone repeat pelvic osteotomy (RPO) and analyzed patient history, complications, and orthopedic outcomes.

Results

Fifty-six patients who underwent RPO were identified. All had previously failed at least one attempted bladder closure. The patients underwent RPO at a mean age of 23.2 months. The mean time from initial osteotomy to RPO was 20.5 months. Anterior innominate or combined iliac/innominate approaches comprised 80% of RPO procedures. Of the patients, 95% had a normal gait after RPO; all 3 patients with an abnormal gait had osteotomy site nonunion, which was treated with bone grafting. Five patients had local fixator pin site infections, which were managed with local care and oral antibiotics, and 1 patient had late osteomyelitis requiring incision and drainage. No patient had femoral or sciatic nerve palsy after RPO at our institution.

Conclusions

Orthopedic complications after RPO are uncommon, and most patients have a normal gait postoperatively. Repeat pelvic osteotomy is useful in the complex reconstruction of failed exstrophy closures, and few cases fail reclosure when the reconstruction is combined with RPO.     

Lateral-based flap: a single stage urethral reconstruction for proximal hypospadias

Purpose

The aim of this study is to describe and report the outcome of a single stage repair—the lateral-based (LB) flap—for proximal hypospadias with chordee.

Materials and Methods

Between January 1995 and January 2007, 107 boys with proximal hypospadias underwent repair using the LB flap technique. The boys' age ranged from 1 to 5 years. The technique employs the use of lateral penile skin and extends into the outer preputial skin. The LB flap enjoys double blood supply from the base of the meatus as well as the preputial vessels. The Y-shaped incision in the glans gives a terminal slit-like meatus, and the technique allows for correction of the chordee. Follow-up period ranged from 1 year to 13 years (mean, 72 months).

Results and Complications

Satisfactory results were obtained in 98 (91%) patients. Three patients developed fistulae. Two patients had proximal stenosis that required temporary urethrotomy for 3 months. Four patients developed urethral diverticulum.

Conclusions

The LB flap is a robust flap with a reliable dual blood supply, which produces good results (91%) and is suitable for proximal forms of hypospadias with chordee.     

会阴型尿道下裂的矫形和尿道重建

目的 探讨采用分期手术治疗会阴型尿道下裂,行阴茎、阴囊矫形、重建缺损尿道的临床效果.方法 22例会阴型尿道下裂采用分期手术：一期手术将阴茎海绵体完全伸直,阴茎包皮内板和背侧皮肤预置于阴茎腹侧和阴囊裂缝凹陷处;二期手术重建阴茎尿道采用半环状阴茎皮岛＋半环状膀胱黏膜丛行侧面缝合形成阴茎尿道,阴囊尿道采用一期预置的组织丛行卷曲侧侧缝合重建阴囊尿道,在两尿道的接合点端端吻合,同时施行阴囊矫形.结果 22例会阴型尿道下裂矫形后几乎接近正常状态,重建阴茎尿道长度为4～9 cm,平均7 cm.手术的成功率为68%（15/22）,尿瘘发生率为32%（7/22）,5例（5/22）发生阴茎阴囊尿道交界处狭窄,经尿道扩张治疗后痊愈.结论 会阴型尿道下裂行分期手术治疗可以修复超过10 cm长的缺损尿道,而且完成手术以后外形形态较好.     

An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy

Classic bladder exstrophy is characterized by displaced pelvic floor musculature and significant skeletal and genitourinary defects. A paucity of data exist evaluating long-term pelvic floor function in exstrophy patients after ureterosigmoidostomy. This study is an initial attempt to evaluate the prevalence of urofecal incontinence, pelvic organ prolapse, and overall quality of life in patients who have had ureterosigmoidostomies. Fifty-two individuals who underwent ureterosigmoidostomy between 1937 and 1990 were identified through the Ureterosigmoidostomy Association and the Johns Hopkins bladder exstrophy database and mailed questionnaires approved by the Institutional Review Board (Johns Hopkins). Data were analyzed with SigmaStat 3.0 (SPSS, Inc., Chicago, IL). Eighty-three percent of the subjects responded, with a mean age of 44.4 years (range, 14–73 years) and mean of 40.9 years (range, 14–65 years) after ureterosigmoidostomy. Prevalence of daily urinary and fecal incontinence was 48% (n = 20) and 26% (n = 11), respectively, whereas the prevalence of weekly combined urofecal incontinence was 63% (n = 27). The incidence of pelvic organ prolapse in this cohort was 48% (n = 20). In these patients, a significant risk of urofecal incontinence and pelvic organ prolapse exists. Long-term follow-up studies are needed to understand the role of pelvic floor musculature in this complex birth defect. Presented at the Forty-Sixth Annual Meeting of The Society for Surgery of the Alimentary Tract, Chicago, Illinois, May 14–19, 2005 (poster presentation).     

Bone health in patients with cloacal exstrophy and persistent cloaca after bladder augmentation

Purpose

In this study, the prevalence of osteoporosis was evaluated in patients operated on for cloacal exstrophy or persistent cloaca and who had undergone bladder augmentation.

Methods

Seven patients operated on because of cloacal exstrophy and 3 patients operated on because of persistent cloaca were evaluated. Patients were clinically assessed. Areal bone mineral density for the lumbar spine (L1-L4), femoral neck, and whole body was measured with dual-energy x-ray absorptiometry. Acid-base balance, plasma 25-hydroxyvitamin D (vitamin D), and gonadal function were determined and correlated with dual-energy x-ray absorptiometry results.

Results

The patients were small in height (median, −2.2 SD). According to areal bone mineral density measurements, 40% of the patients had significantly reduced BMD. When the values were adjusted for patient size, only 1 patient had slightly reduced bone mass. Half of the patients had low vitamin D values. The height-corrected bone mineral content values showed a positive correlation with vitamin D values (P = .0072) but were not different in patients with and without hypogonadism (P = .33).

Conclusions

Patients with cloacal exstrophy or complicated cloaca have many risk factors for osteoporosis. However, this study showed good overall bone health in affected adolescents.     

小儿尿道下裂术后尿瘘的成因及修复(附33例报告)

目的 研究小儿先天性尿道下裂手术后尿瘘发生的原因,探讨小儿尿道下裂术后尿瘘的修复方法,总结手术经验,提高尿瘘一次修补成功率.方法 收集2001年1月-2011年2月33例小儿尿道下裂尿道成形术后尿瘘的临床资料,平均年龄6.2岁(3～13岁).共38个瘘口,痿口直径小于3 mm的有17个,瘘口直径为3～10mm的有16个...     

Overview of bladder exstrophy: a third world perspective

Introduction

Bladder exstrophy is an uncommon condition, and the ramifications for patients living in a Third World environment are extensive.

Patients and Methods

This was a retrospective study of 57 patients, managed between 1983 and 2006. The review looked at the home environment, clinical findings, treatment provided, and short-term outcome.

Results

Of the 57 patients, 19 were born in metropolitan and 38 in rural hospitals. Twenty-four patients (42%) were referred on the first day of life, 21 (37%) within the first week, and 12 patients (21%) were older than 1 week of life. Associated congenital anomalies were seen in 18 patients (32%). Twelve patients died in hospital, the remaining 45 were discharged. Thirty three patients returned for review; their ages ranged from 2 to 17 years (median, 3 years). The overall mortality was 42%. On follow-up, urinary incontinence was the main complaint, which greatly affected the child's schooling and social life.

Conclusion

Bladder exstrophy is an uncommon condition. The associated congenital abnormalities together with the impoverished environment result in a poor prognosis. Antenatal screening, early referral, and establishing urinary continence are factors that will improve the outcome in children with this condition in a Third World environment.     

Recurrent pelvic organ prolapse in a woman with bladder exstrophy: a case report of surgical management and review of the literature

Management of gynecologic issues in women with bladder exstrophy is challenging. Congenital pelvic organ prolapse and prolapse-associated pregnancy and delivery are common. The management of prolapse is complicated by the anatomic changes involving the bony pelvis, connective tissue support, pelvic floor, and the length and axis of the vagina. A case of recurrent pelvic organ prolapse successfully managed with a sacral colpopexy and a review of the literature are presented.     

Augmentation ureterocystoplasty in bladder exstrophy: 5-year follow-up in two cases

OBJECTIVE: To report two cases of bladder exstrophy managed successfully by augmentation ureterocystoplasty (UCP) together with bladder neck surgery and continent diversion. PATIENTS: Two boys, age 5 and 1 years respectively, had augmentation UCP. The left refluxing megaureter was used in the first case together with bladder neck reconstruction. In the second patient, bilateral obstructed magaureters were used in tandem together with bladder neck division. Both patients had appendicovesicostomy according to Mitrofanoff. RESULTS: In follow-up for over 5 years, both patients are continent with improved upper urinary tracts and normal or stable renal function. They are managed by clean intermittent catheterization (CIC) via the continent stoma with bladder capacities of 220 cc and 150 cc, respectively. Cystogram showed no vesicoureteric reflux (VUR). The procedures were considered to be successful although one patient was on oxybutinin because urodynamics showed high intravesical pressures. CONCLUSIONS: Augmentation UCP should be considered in patients with bladder exstrophy when a suitable megaureter is available. This may be combined with simultaneous bladder neck reconstruction together with a continent diversion.     

The failed bladder closure in cloacal exstrophy: Management and outcomes

PurposeCloacal exstrophy (CE) is the most severe presentation of the Exstrophy-Epispadias Complex (EEC) and is associated with an omphalocele, making the bladder and abdominal wall closure difficult. If the bladder closure fails, a secondary closure is necessary. The objective of this study is to identify patient or surgical factors associated with a successful secondary closure.MethodsThe institution's EEC database was reviewed for CE patients between 1975 and 2015. Inclusion criteria included a failed primary bladder closure with a secondary closure. Patient demographics, surgical factors and outcomes of the secondary bladder closure were reviewed.ResultsTwenty-four patients met inclusion criteria. 8/8 patients had a successful two-staged closure at the author's institution (100%); 2/16 patients had a successful closure at an outside institution (12.5%). Older median age at secondary closure was associated with outcome, p = 0.045. Pelvic osteotomy was associated with successful secondary closure, p = 0.013. Using Buck's immobilization with external fixation was associated with a higher proportion of successful secondary closures compared to Spica cast, p = 0.012.ConclusionSuccessful reclosure in CE patients is associated with the use of osteotomy as well as Buck's immobilization with external fixation. While successful reclosure can be achieved, it is often at the cost of multiple procedures and, therefore, all efforts should be expended to achieve a successful primary closure.Type of Study: Prognostic.Level of Evidence: III.     

Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias

In the paediatric section this month several important issues are addressed. The authors from Lyon describe the results of bladder neck reconstruction for incontinence in children with bladder exstrophy and incontinent epispadias. They indicate the unpredictability of bladder neck reconstruction and discuss the results of the other techniques used for urinary continence.

OBJECTIVE

To review the long‐term results of bladder neck reconstruction (BNR) in patients with classical bladder exstrophy or epispadias, and to review the concept of continence surgery in these two groups, stressing the difficulty in finding an adequate balance between urine storage (which implies high outlet resistance and low storage pressure) and complete bladder emptying (which implies low outlet resistance and a transient increase in bladder pressure); surgery cannot achieve ‘continence’ (which implies active mechanisms) but only ‘dryness’ (which implies passive mechanisms).

PATIENTS AND METHODS

Eighty patients with classical bladder exstrophy (52 male, 28 female) and 25 with incontinent epispadias (17 male, 18 female) had their bladder neck reconstructed after a Young‐Dees‐Leadbetter procedure, subsequently modified by Mollard. The treatment is detailed and results reviewed after a mean follow‐up of 11 years. All patients were treated and followed in the same institution.

RESULTS

In the exstrophy group, 36 (45%) patients presented with a dry interval of > 3 h, with urethral emptying after one BNR; 52 (65%) presented with recurrent urinary tract infections, 19 (24%) with urinary stones, 21 (26%) with dilated upper urinary tracts, 13 (16%) with bladder perforations and one with an adenocarcinoma of the bladder. Thirty‐eight patients (48%) required further surgery; 51% of all patients required an endoscopic procedure within 3 months after the BNR and 26% had endoscopic procedures for late (> 3 months) urine retention. In the epispadias group, 13 (52%) patients presented with a dry interval of > 3 h with urethral emptying after one BNR; 12 (48%) had recurrent urinary tract infections, five (20%) upper tract dilatation, two (8%) bladder stones, one (4%) bladder perforation and one an adenocarcinoma of the bowels after a ureterosigmoidostomy. Ten (40%) children required further surgery.

CONCLUSION

We compared the present results for continence with those in other published series; most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behaviour caused by BNR. We consider that BNR is unpredictable and the roles of the other factors in urinary continence are discussed. Alternative procedures are detailed. The concept of continence surgery in exstrophy and incontinent epispadias is reviewed, stressing the importance of favouring bladder development and limiting obstructive patterns of bladder emptying that cause severe and recurrent complications.

     

游离包皮内板或膀胱粘膜治疗尿道下裂(附56例报告)

目的：介绍采用游离包皮内板或膀胱粘膜治疗尿道下裂的方法及效果。方法：对56例尿道下裂患儿根据尿道缺损长度不同，42例采用包皮内板，14例采用膀胱粘膜行尿道成形术。结果：56例中一次手术成功49例（87.5%），7例尿瘘（12.5%），其中1例小尿瘘自行愈合，另6例分别于术后3个月行尿瘘修补术治愈，随访6个月－3年，疗效满意。结论：游离包皮内板或膀胱粘膜可以治疗各种类型尿道下裂，效果良好。     

Predictors of a successful primary bladder closure in cloacal exstrophy: A multivariable analysis

Purpose

To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure.

Colorectal anomalies in patients with classic bladder exstrophy

Aim

This study aims to determine the proportion and type of colorectal anomalies that occur in children born with classic bladder exstrophy (CBE).

Methods

All patients in a database of 1044 patients with the bladder exstrophy-epispadias-cloacal exstrophy complex were reviewed. Those with CBE had their complete medical records reviewed. Children noted to have gastrointestinal malformations were identified, and all aspects of their history were extracted.

Results

A total of 676 patients were identified with CBE, of whom 12 patients were identified who had a concomitant colorectal anomaly. In this population, the proportion of colorectal anomalies is 1.8%. The most common gastrointestinal anomaly was imperforate anus in 8 patients. Two patients had severe rectal stenosis requiring serial dilations, and 2 patients were born with congenital rectal prolapse.

Conclusions

Excluding all variants and cloacal patients with exstrophy, isolated colorectal anomalies occur at a rate of 1.8% in children born with CBE, a 72-fold increase compared with the general population. Although uncommon in this rare birth defect, the morbidity is significant and warrants prompt evaluation and treatment of both the genitourinary and colorectal anomalies.     

Analysis of factors associated with postoperative complications after primary hypospadias repair: a retrospective study

BackgroundTo determine the risk factors for postoperative complications after primary hypospadias repair. Hypospadias has a high postoperative complication rate, and the risk factors of postoperative complications have attracted extensive attention.MethodsA total of 857 children who received primary surgical repair for hypospadias in our center between 3 January 2017 and 29 January 2021 were retrospectively analyzed. The collected data included age at time of surgery, type of hypospadias, body mass index (BMI), surgeon, operation time, length of reconstructed urethra, method of anesthesia (general anesthesia or general anesthesia combined with caudal anesthesia), and postoperative constipation. The risk factors for postoperative complications were analyzed by multivariate analysis.ResultsThe follow-up time in this study was 6–54 months, with a mean follow-up time of 29 months. A total of 96 (11.2%) of the 857 pediatric patients had postoperative complications, including 44 (45.8%) cases of urethral fistula, 14 (14.6%) cases of urethral stricture, 5 (5.2%) cases of urethral diverticula, 5 (5.2%) cases of distal dehiscence, 3 (3.1%) cases of poor exposure, 2 (2.1%) cases of residual curvature, 1 (1.0%) case of penoscrotal transposition, 6 (6.3%) cases of urethral stricture and diverticulum, 6 (6.3%) cases of urethral fistula and diverticulum, 3 (3.1%) cases of urethral fistula and postoperative residual curvature, 2 (2.1%) cases of urethral fistula and distal dehiscence, and 1 (1.0%) case each of urethral fistula and transposition, urethral diverticulum and poor exposure, urethral stricture and poor exposure, distal dehiscence and transposition, and residual curvature and transposition. After univariate analysis, type of hypospadias (P=0.038), operation time (P<0.001), length of reconstructed urethra (P=0.007), and postoperative constipation (P=0.019) were included in the multivariate logistic regression analysis. The results showed that postoperative constipation was an independent risk factor for complications [P=0.027, odds ratio (OR) =1.793, confidence interval (CI): 1.067 to 3.012].ConclusionsPostoperative constipation is an important influencing factor for postoperative complications following primary hypospadias repair. Therefore, defecation management should be strengthened for hypospadias patients during the perioperative period.     

Treatment of incontinence in children with bladder exstrophy after rectal urinary diversion: the anal sling procedure

PURPOSE: An original technique for reinforcement of the anal sphincter using a sling prosthesis with a flexible fixing point that prevents incontinence and rectal prolapse is presented. MATERIALS AND METHODS: Of 43 patients with exstrophy who have undergone urinary rectal diversion between 1988 and 1997 incontinence was observed in 9 boys and 5 girls. From January 1993 to December 1998 these children were treated with an original method, the anal sling procedure. RESULTS: There were no postoperative complications. Investigations revealed no urinary or fecal incontinence after a mean followup of 3.2 years (range 6 months to 61/2 years). CONCLUSIONS: A short and simple operation eliminates incontinence after rectal urinary diversion in patients with bladder exstrophy. It can be used as an alternative to the formation of an isolated urinary reservoir.