Repair Of Exstrophy Research Articles

Economic Impact of Failed or Delayed Primary Repair of Bladder Exstrophy: Differences in Cost of Hospitalization

Patients with bladder exstrophy and failed primary newborn closure or who undergo delayed primary repair have suboptimal functional outcomes. We sought to determine whether these patients also have costlier, more resource intensive hospitalizations compared to patients who undergo neonatal primary closure. We reviewed hospital coding records to identify patients who underwent surgical repair of classic bladder exstrophy at The Johns Hopkins Hospital between 1997 and 2006, and obtained charge records for each hospitalization. Total hospital charges (excluding professional fees) were inflation adjusted to year 2005 dollars. Cases were identified as newborn primary repair, delayed primary repair or reclosure of failed prior repair. Results of classic exstrophy repair were analyzed in 80 patients. A total of 34 procedures were newborn primary repairs, 15 were delayed primary repairs and 31 were reclosures of failed prior repair. All of the patients undergoing delayed primary repairs and reclosures underwent osteotomy, compared to only 21% of those undergoing newborn primary repair. Overall mean inflation adjusted hospitalization charge was $66,348 +/- $26,625 (range $29,689 to $179,403). Newborn closures were significantly less costly (mean charge $53,188 +/- $15,086) than either reclosure ($71,621 +/- $19,366) or delayed primary closure ($85,278 +/- $42,354, p <0.0001). Controlling for multiple variables in a regression model showed that the primary factors associated with charges were operative time, days in intensive care unit and length of stay. Length of stay and operative times were significantly shorter in the newborn surgical group, likely accounting for the lower costs in this group (despite higher intensive care unit use). Mean hospital charges and mean length of stay increased during the study period. Primary newborn exstrophy repair is associated with lower surgical hospitalization costs compared to delayed primary repair and reclosure. Combined with previous data on clinical outcomes, these data reiterate the paramount importance of achieving a successful initial newborn closure whenever possible.

Salvage Procedures to Achieve Continence After Failed Bladder Exstrophy Repair

We assessed the results from a single exstrophy center of salvage continence surgery after failed staged reconstruction for bladder exstrophy. A total of 32 patients with bladder exstrophy had undergone salvage continence procedures. Indications for surgery included incontinence due to poor bladder capacity or failed bladder neck repair, and upper tract deterioration. Continence was defined according to the International Children's Continence Society as continent, intermittently incontinent and continuously incontinent. A total of 29 patients (91%) are continent, 3 (9%) are intermittently incontinent and none is continuously incontinent. One patient is continent after bladder augmentation and urethral clean intermittent catheterization. Two patients are continent and 1 is intermittently incontinent after bladder augmentation and modified Young-Dees bladder neck repair using urethral clean intermittent catheterization. One patient is continent using clean intermittent catheterization through a continent cutaneous diversion into a bladder substitution. A total of 19 patients are continent after bladder neck closure, bladder augmentation and continent cutaneous diversion using clean intermittent catheterization. Four patients are continent after cutaneous urinary diversion. Two are continent and 2 are intermittently incontinent after a Mainz II pouch. The majority of patients can still achieve continence following failed staged repair. Patients who have a low bladder leak pressure and who tolerate urethral catheterization can be consistently dry with bladder augmentation and bladder neck repair, which is a viable alternative to bladder neck closure, bladder augmentation and continent cutaneous diversion. Cutaneous urinary diversion has a role in selected patients. Mainz II pouch has not yielded consistent results. With better patient selection and increasing experience within specialist exstrophy centers fewer patients should require salvage continent surgery in the future.

Scrotal Reconstruction Using Rapid Intraoperative Tissue Expansion: A Preliminary Report

Extensive scrotal skin loss can pose a difficult management problem. In most cases delayed scrotal approximation is feasible due to the redundancy of scrotal skin. However, large defects usually require reconstruction using flaps or grafts. We present a novel technique of scrotal closure using rapid intraoperative tissue expansion to manage select extensive partial scrotal defects. Between January 1996 and April 2006, 3 patients underwent delayed closure using rapid intraoperative tissue expansion for approximately 75% scrotal skin loss. Intraoperatively the residual lateral scrotal and medial thigh skin, and subcutaneous tissues were dissected, creating bilateral cavities for the placement of round 300 cc tissue expanders. Gradual inflation for a total of 20 minutes allowed adequate tissue expansion of the 2 thigh flaps, enabling tension-free scrotal closure. Each patient was followed for 12 months. The resulting reconstruction provided a tension-free, cosmetically appealing scrotum with complete testicular coverage. There were no perioperative or postoperative complications. Although rapid intraoperative tissue expansion has been successfully used for the closure of facial and extremity defects, to our knowledge it has not been described for genital skin loss. Our experience suggests that this relatively simple method is a viable addition to the reconstructive armamentarium in a select group of patients with extensive scrotal skin defects that are not amenable to scrotal approximation alone.

Geschichte der Blasenekstrophiebehandlung in Deutschland

While repair of exstrophy has a long tradition in Germany, a comprehensive review of the medical history of exstrophy-epispadias repair and its frequent inconsistencies and setbacks in Germany has not previously been published. Numerous resources were used to collect the information described in this review. Older textbooks from the library of German Society of Urology (DGU) in Düsseldorf and from the School of Medicine Library at the Friedrich-Alexander University in Erlangen were used to collect information on the important steps in the treatment of bladder exstrophy and epispadias from the first report of it onward. A Medline search was done to collect appropriate information from after 1969. Schenck von Grafenberg was the first to describe exstrophy of the bladder, in his report published in 1597, while the term 'exstrophy' was first used by Chaussier in 1780. Isolated epispadias remained unknown and untreated until it was described by Morgagni in 1761. The initial attempts to treat this anomaly were restricted to controlling the incontinence. In 1869 Karl Thiersch described the etiology and anatomy of epispadias and reported on a case of epispadias reconstruction with a long-term follow-up of 11 years. As long ago as in 1897, Mickulicz enlarged a small exstrophic bladder by adding an ilial segment to make it possible to close the bladder. After the first really successful reconstruction of bladder exstrophy in 1942 by Young in the USA, various multiple-stage and single-stage reconstruction approaches emerged in Germany. From the start, urinary diversion was seen as an alternative to reconstruction in the treatment of bladder exstrophy. The first ureterosigmoidostomy was done in an exstrophy patient by Simon in Heidelberg in 1852, after which Maydl described a new technique based on transtrigonal sigmoidostomy for urinary diversion in 1894. There are now three approaches in current use in Germany for the management of bladder exstrophy-epispadias: reconstruction in various staged approaches, complete reconstruction in a single session, and the primary rectal reservoir (Mainz pouch II).

The modern staged repair of bladder exstrophy in the female: A contemporary series

Objective Many changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken. Patients and methods From an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988–2005, at a mean age of 2 months (range 4 h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39–65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80–179 cc). Results Thirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3 h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1 h day and night. The mean time to daytime continence was 12 months (4–16 months) and to night-time continence was 19 months (10–28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure. Conclusions Female classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.

Results of Bladder Neck Reconstruction After Newborn Complete Primary Repair of Exstrophy

We describe the results of modified Young-Dees-Leadbetter bladder neck reconstruction to achieve continence in patients who underwent complete primary repair of bladder exstrophy as newborns. Using the Johns Hopkins Exstrophy Database we identified patients who underwent bladder neck reconstruction after having undergone complete primary repair of bladder exstrophy as newborns. We determined patient characteristics and surgical outcomes. A total of 30 males and 3 females were referred after complete primary repair of bladder exstrophy, of whom 26 underwent bladder neck reconstruction. Results of complete primary repair of bladder exstrophy were strongly associated with osteotomy use. Of the patients 19 (58%), including 16 males and 3 females, who underwent complete primary repair of bladder exstrophy without osteotomy had complications (dehiscence and bladder prolapse), while none of 14 male patients who underwent complete primary repair of bladder exstrophy with osteotomy had complications, although none were subsequently continent. Of the 19 patients who had complications after complete primary repair of bladder exstrophy 12 underwent bladder neck reconstruction and total continence was achieved in only 3 (25%). Of the 14 patients with successful complete primary repair of bladder exstrophy 8 (57%) are dry day and night, 4 (28%) are dry during the day and wet at night, and 2 (14%) are completely incontinent. Continent children underwent successful complete primary repair of bladder exstrophy with pelvic osteotomy, all underwent hypospadias repair before age 1 year and none required ureteral reimplantation before bladder neck reconstruction. Many patients who undergo newborn complete primary repair of bladder exstrophy will require bladder neck reconstruction. Bladder neck reconstruction is more successful in those in whom complete primary repair of bladder exstrophy was successful. As in all types of repair, failed initial closure usually results in a bladder that is unsuitable for bladder neck reconstruction. These patients often require bladder augmentation and a continent stoma to be dry.

Ureterorectostomy as a continent urinary diversion for complicated bladder exstrophy in children by using a modified Duhamel procedure: A case series

Introduction Whatever the method and timing of surgery, a high proportion of children with bladder exstrophy will continue to suffer from urinary incontinence. They face the options of urinary diversion to an external stoma or construction of a neobladder from bowel. This study describes a modified Duhamel's rectal pouch with a ureterorectostomy was carried out on 11 children who had a failed repair of bladder exstrophy. Materials and methods Ten boys and one girl, aged from 4 to 7 years (mean 5.5), had several unsuccessful operations for bladder exstrophy. All selected to have good renal function and no other anomalies, but were incontinent of urine and had a small contracted or prolapsed bladder. They underwent urinary diversion to the rectum using the Duhamel pullthrough technique, where the sigmoid colon was opened into the back of the anal canal above the dentate line, creating a rectal bladder and making use of the anal sphincter to control urine and stool. All were followed up for 24 months (18–27 months). Results In this selected group of patients there were no major operative or postoperative complications. Follow-up for 2 years revealed no deterioration in renal function, or electrolytes disturbance. They can hold up to 300 ml of urine and all patients are continent during the daytime with an emptying frequency of 3–5 times. Nocturnal wetting occur some 4–8 times per month with significant decrease with time. Two cases developed pyelonephritis but this was controlled with medical treatment. Conclusion Eleven children achieved effective urinary continence by ureteric diversion to the rectum using a modified Duhamel pullthrough technique. Two years follow up showed no complications, except bed wetting, but long term assessment is warranted.

Hypothermia-induced bradycardia in a neonate receiving dexmedetomidine

Dexmedetomidine, an α 2-adrenergic agonist, decreases lipolysis and thus prevents nonshivering thermogenesis in infants. We report the case of a two-day-old neonate who received dexmedetomidine for sedation and analgesia after undergoing primary repair of bladder exstrophy. After 9 hours, the patient developed bradycardia and profound hypothermia. The bradycardia was unresponsive to anticholinergics but resolved approximately two hours after radiant heat was applied. This report highlights the potential profound impact of dexmedetomidine on thermoregulation in neonates. Dexmedetomidine impacts thermoregulation in neonates and its use warrants careful attention to the control of temperature and to the routine use of exogenous heat.

374: Economic Impact of Failed or Delayed Primary Repair of Bladder Exstrophy: Differences in cost of Hospitalization

You have accessJournal of UrologyModerated Poster 11, Sunday, May 20, 2007 1:00 - 3:00 pm1 Apr 2007374: Economic Impact of Failed or Delayed Primary Repair of Bladder Exstrophy: Differences in cost of Hospitalization Caleb P. Nelson, Amanda North, Maryann K. Ward, and John P. Gearhart Caleb P. NelsonCaleb P. Nelson More articles by this author , Amanda NorthAmanda North More articles by this author , Maryann K. WardMaryann K. Ward More articles by this author , and John P. GearhartJohn P. Gearhart More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(18)30627-XAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "374: Economic Impact of Failed or Delayed Primary Repair of Bladder Exstrophy: Differences in cost of Hospitalization." The Journal of Urology, 177(4S), p. 124 © 2016 by American Urological AssociationFiguresReferencesRelatedDetails Volume 177Issue 4SApril 2007Page: 124 Advertisement Copyright & Permissions© 2016 by American Urological AssociationMetricsAuthor Information Caleb P. Nelson More articles by this author Amanda North More articles by this author Maryann K. Ward More articles by this author John P. Gearhart More articles by this author Expand All Advertisement Loading ...

Single-stage repair of bladder exstrophy in older children and children with failed previous repair

Aim Evaluation of cosmetic and functional outcome of single-stage exstrophy–epispadias complex repair in older children and those with previously failed repair. Materials and methods This study comprised 15 children (12 boys and 3 girls) with classic bladder exstrophy and a mean age at repair of 8.6 months (range 2–24 months). Eight children had a previously failed repair. All children underwent complete primary repair using the single-stage Mitchell technique. Half of the boys had complete penile disassembly, while in the others a modified Cantwell–Ransley technique for epispadias repair was used. Anterior iliac osteotomy was performed and hip spica used for immobilization in all children. Results One child had urethral stricture treated by endoscopic visual urethrotomy. Three children had penopubic fistulae that closed spontaneously. No bladder dehiscence or prolapse was encountered. Vesicoureteral reflux was present in 20 renal units but ureteral reimplantation was not performed. Average bladder capacity after closure was 134 cm 3 (range 110–160 cm 3) with only two partially continent and six incontinent children. Mean follow-up period is 2 years (range 1–3 years). Conclusions Single-stage repair was performed in children with previously failed repair and those presenting at an older age with satisfactory results. Acceptable bladder and genital anatomy and function were achieved together with preservation of renal function. The impact of this technique on continence is not encouraging, but needs to be determined in a longer follow-up period.

The modern staged repair (MSRE) of bladder exstrophy in the female: a contemporary series

The modern staged repair (MSRE) of bladder exstrophy in the female: a contemporary series

Modern staged repair of bladder exstrophy: A contemporary series

Objective Many changes have occurred in the treatment of bladder exstrophy over the last few years and many repairs are now offered. The purpose of this study was to evaluate long-term outcomes in a select group of patients in whom modern staged repair (MSRE) was undertaken. Patients and methods From an institutionally approved database were extracted 189 patients who had undergone primary closure between 1988 and 2004. The records of 131 patients (95 males) who underwent MSRE with a modified Cantwell-Ransley repair by a single surgeon in 1988–2004 were reviewed with a minimum 5-year follow up. Results Sixty-seven patients with a mean age of 2 months (range 6 h to 4 months) underwent primary closure, and 18 underwent osteotomy at the same time. Mean age at epispadias repair was 18 months (8–24). Mean age at bladder neck reconstruction (BNR) was 4.8 years (40–60 months) with a mean capacity of 98 cc (75–185). Analysis of bladder capacity prior to BNR revealed that patients with a mean capacity greater than 85 cc median had better outcomes. Seventy percent ( n = 47) are continent day and night and voiding per urethra without augmentation or intermittent catheterization. Social continence defined as dry for more than 3 h during the day was found in 10% ( n = 7). Six patients required continent diversion after failed BNR. Seven patients are completely incontinent. The mean time to daytime continence was 14 months (4–23) and the mean time to night-time continence was 23 months (11–34). No correlation was found between age at BNR and continence. Conclusions Patients with a good bladder template who develop sufficient bladder capacity after successful primary closure and epispadias repair can achieve acceptable continence without bladder augmentation and intermittent catheterization.

Can a Complete Primary Repair Approach be Applied to Cloacal Exstrophy?

Surgical reconstruction for children with cloacal exstrophy remains challenging. The operative approach to cloacal exstrophy has expanded with the addition of the complete primary exstrophy repair. We assessed the safety and efficacy of complete primary exstrophy repair for this complex condition. We performed a retrospective review of children treated from birth for cloacal exstrophy between March 1, 1994 and January 1, 2003 at our institution. We evaluated associated anomalies, method of closure, complications and urinary continence. Seven patients with cloacal exstrophy were initially treated at our institution. One mortality occurred before complete primary exstrophy repair was attempted. Six patients were converted to a classic exstrophy appearance and underwent closure using complete primary exstrophy repair principles within 7 to 182 days (mean 68, median 32) postoperatively. Postoperative development of moderate hydronephrosis was seen in 1 patient and severe hydronephrosis in 1. Three of six patients had vesicoureteral reflux. Six patients had dry intervals and spontaneous voids before toilet training. Two patients had stress urinary incontinence. Two patients have been treated with bladder neck injections. One has undergone bladder neck reconstruction and construction of a nonorthotopic channel for clean intermittent catheterization (Mitrofanoff). One patient reported complete dryness after toilet training. One child has undergone bladder augmentation. This series represents our initial efforts to use complete primary exstrophy repair for cloacal exstrophy. The application of the principles of complete primary exstrophy repair in a sequential fashion appears to be a viable and safe addition to the surgical armamentarium in this challenging patient population.

Re: Complete Primary Repair of Bladder Exstrophy in Children Presenting Late and Those With Failed Initial Closure: Single Center Experience

Re: Complete Primary Repair of Bladder Exstrophy in Children Presenting Late and Those With Failed Initial Closure: Single Center Experience

Surgery Insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy

Staged reconstruction repair of bladder exstrophy results in hydronephrosis or renal scarring in 15-25% of patients. A cosmetically acceptable and functional phallus can be achieved in 85% of patients, 20-30% of whom will require more than one operation for penile reconstruction. Episodes of penile glans loss or corporal loss are rarely reported with this technique. Widely disparate results relating to complete urinary continence and volitional voiding have been published, with urinary continence reported to occur in 7-85% of patients. The need for bladder augmentation to obtain urinary continence also varies, with reports that somewhere between 10% and 90% of patients require an augmentation procedure to gain urinary continence. Complete primary repair of bladder exstrophy using the penile disassembly technique results in hydronephrosis or renal scarring in 0-30% of patients, and hypospadias, as a consequence of this repair, will occur in 30-70% of patients. Loss of the glans and corpora appear more frequently with penile disassembly than in staged reconstruction of bladder exstrophy, however, the exact incidence of this complication is unknown. Reported complete urinary continence and volitional voiding rates are also varied following penile disassembly, ranging from 25-65%. A modified bladder-neck reconstruction to gain urinary continence is reportedly required in 15-90% of patients, with 5-10% requiring both bladder augmentation and bladder-neck reconstruction. Experience with complete primary repair of bladder exstrophy, using the penile disassembly approach, seems promising but is not a panacea. To outline the risks and benefits regarding the various surgical techniques for bladder exstrophy, we would recommend the establishment of a national registry for patients with this disorder.

Primary repair of bladder exstrophy followed by clean intermittent catheterization: Outcome of 15 years’ experience

Objectives To determine the continence and spontaneous voiding rate after neonatal reconstruction of bladder exstrophy without formal bladder neck reconstruction in patients undergoing primary reconstruction and treated with clean intermittent catheterization (CIC) after closure. Methods From 1987 to 2003, 15 consecutive patients (8 boys and 7 girls) with bladder exstrophy underwent neonatal reconstruction. Reconstruction focused on bringing the bladder neck and proximal urethra intra-abdominally and meticulously closing the pelvic floor muscles around the urethra. Three weeks postoperatively, CIC was started until toilet-training age. Bladder capacity, continence status, renal anatomy and function, and additional urologic surgical procedures during follow-up were analyzed. Results Nine patients (60%) became socially continent after primary closure without any additional bladder neck surgery. Twelve patients (80%) were continent when those who underwent endoscopic bulking injection were included. One patient became socially continent after bladder neck reconstruction, and one was dry and used CIC after bladder neck reconstruction and ileocystoplasty. One patient remained incontinent because of the parents’ refusal of surgery. Ultimately, of 15 patients, 14 were dry (93%) of whom 10 were completely continent, 3 were partially continent (dry intervals of 1 to 3 hours), and 1 was dry by catheterizable stoma. The bladder capacity was adequate for age in 80% of patients. Febrile urinary tract infection occurred in 33% of patients, and 67% needed endoscopy for urethral stenosis. Upper tract dilation and loss of renal function was not seen. Conclusions The results of our study have shown that primary repair of bladder exstrophy followed by CIC has encouraging continence and bladder capacity rates, with preservation of the upper urinary tract and limited need for additional bladder neck surgery.

Editorial comment

The authors describe their 15-year experience with primary exstrophy closure in 15 patients. As with any long-term retrospective study, the management of the presented patient population was heterogeneous and difficult to interpret. They describe a two-staged approach with initial closure before 72 hours of age and a second-stage epispadias closure between 12 and 18 months of age. Formal BNR was not performed in most patients. Three of the 12 patients without BNR underwent bladder neck injection with a bulking agent. Two patients underwent formal BNR, and the parents of 1 patient refused additional surgical treatment and the patient remained incontinent. Primary repair of bladder exstrophy followed by clean intermittent catheterization: Outcome of 15 years’ experienceUrologyVol. 67Issue 2PreviewTo determine the continence and spontaneous voiding rate after neonatal reconstruction of bladder exstrophy without formal bladder neck reconstruction in patients undergoing primary reconstruction and treated with clean intermittent catheterization (CIC) after closure. Full-Text PDF

Initial Experiences of Complete Primary Exstrophy Repair in Cloacal and Bladder Exstrophy

334 배설강 및 방광외번증은 매우 드문 선천성 기형으로 소 화기계, 신경계, 근골격계, 그리고 비뇨기계를 포함하는 다 기관 기형이다. 이는 태생기에 배설강막 이상과 중배엽조 직의 정중이동 장애로 인하여 나타난다고 알려져 있다. 하 복벽과 방광전벽의 결손으로 나타나는 방광외번증의 경우 10,000-50,000명 중 1명의 발병률을 보이고, 하복벽의 선천 성 기형 중에 가장 심하고 드문 경우인 배설강외번증의 경 우 200,000명 중 1명의 발병률을 보인다. 과거 배설강 및 방광외번증의 경우 요로전환을 시행하고 방광을 제거하는 것이 표준 치료법이었으나 수술술기의 발 달로 방광을 재건하기 시작하였고 우수한 결과들이 보고되 어 배설강 및 방광외번증의 치료에 있어 방광 재건이 표준 치료법으로 자리 잡았다. 배설강 및 방광외번증의 재건은 먼저 방광을 봉합한 뒤 요도상열을 교정하고, 그리고 항역 류 요관재이식술과 방광경부 재건의 순서로 하는 다단계 교정술이 방광 재건의 수술법으로 발전해 왔으나 근래 단 단계 교정술이 시도되고 있고 다단계 교정술과 비교하여 수술 후 합병증에 차이가 없는 것으로 보고되고 있다. 국내 에서는 배설강 및 방광외번증의 치료에 있어 단단계로 시 행된 완전일차봉합 (complete primary exstrophy repair)으로 치료하였던 보고는 없었다. 최근 본원에서 배설강 및 방광 외번증 3례를 완전일차봉합치료를 하여 완전일차봉합의 단기간 결과를 보고하고자 하였다. 증 례

Complete repair of exstrophy: further experience with neonates and children after failed initial closure.

The surgical repair of bladder exstrophy remains challenging for the urologist. Recently, complete primary repair has been used in neonates. We present our experience with this approach in neonates and children after failed initial closure. Between November 1998 and November 2000, 17 boys and 2 girls with bladder exstrophy underwent complete repair. Complete primary repair was performed in the first 72 hours of life in 4 boys. Complete repair with osteotomy was at a mean age +/- SD of 23 +/- 21 months (range 1 to 74) in 15 patients including 7 with failed initial closure. The bladder and urethra were closed in continuity with epispadias repair by total penile disassembly. All patients were kept in spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days after surgery, respectively. Ultrasound was performed before surgery and 3 months thereafter, and voiding cystourethrography was obtained 3 months postoperatively and then annually in all cases. Complete closure resulted in hypospadias in 10 boys (59%). There was no major complication. Mean followup +/- SD was 17 +/- 8 months (range 5 to 33). Temporary suprapubic urinary leakage was noted initially in 10 cases (52%) but no patient had persistent fistula. Initial postoperative renal ultrasound revealed hydronephrosis in 11 renal units (29%). However, at last followup only 1 renal unit (2%) showed pelvicaliceal dilatation. Two patients (10%) had a febrile urinary tract infection and were treated conservatively. Reflux was noted in 24 renal units (63%) but did not require surgery. The 4 boys in whom the closure was performed at birth had regular voiding with 60 to 90-minute dry intervals and mean bladder capacity +/- SD was 85 +/- 35 cc. The 15 older children had a mean bladder capacity of 74 +/- 37 cc and 5 (33%) had regular voiding with 30 to 90-minute dry intervals. Complete repair of exstrophy is feasible in neonates and older children including, those with failed initial closure with minimal morbidity. There is a short-term evidence of favorable outcome in newborns. Longitudinal followup is required to determine the future need of bladder neck reconstruction and augmentation in older patients.

EARLY OUTCOME FOLLOWING COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY IN THE NEWBORN

Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE. From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections. A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan. We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.