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John P Gearhart - One of the best experts on this subject based on the ideXlab platform.
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prospective study on the incidence of bladder cloacal exstrophy and Epispadias in europe
Journal of Pediatric Urology, 2015Co-Authors: Raimondo M Cervellione, Paolo Caione, John P Gearhart, Alberto Mantovani, Guy Bogaert, Rita Gobet, Alan P DicksonAbstract:Summary Objective On behalf of the European Society of Paediatric Urology (ESPU), a prospective study was designed with the aim of defining the actual number of babies born with bladder exstrophy, cloacal exstrophy, and Epispadias in Europe over a 12-month period, and verifying the distribution of the exstrophy patients born during the study period among the different paediatric urology centres in Europe. Study design The study was structured with a chief investigator and one national investigator for each country enrolled in the study. The national investigators nominated one local investigator for each European centre of paediatric surgery/paediatric urology and urology where the exstrophy complex could potentially be treated. The local investigators were responsible for reporting babies treated in their institutions for bladder/cloacal exstrophy and/or Epispadias. During 2010, every 3 months, an electronic survey (Figure) was e-mailed to the local investigators asking them to report babies treated or referred for treatment during the previous 3 months. Results One-hundred and sixteen centres in 27 European counties were enrolled in the study. The overall response rate for the four online surveys was 79%. Two-hundred and thirty-eight babies were reported to be born with a condition within the bladder exstrophy Epispadias complex (BEEC): 71 primary Epispadias (66 males), 146 classic bladder exstrophy (97 males) of which two were female bladder exstrophy variant, and 21 cloacal exstrophy (17 males). Two of 67 (3%) male Epispadias, 24/146 (16.4%) bladder exstrophy, and 6/21 (28%) cloacal exstrophy were antenatally diagnosed. Associated anomalies were reported in 2/71 (2.8%) Epispadias patients, 8/146 (5.5%) bladder exstrophy patients, and 15/21 (71.4%) cloacal exstrophy patients. One-hundred and forty-seven (62%) of the 238 babies born in Europe with a condition within the exstrophy spectrum during 2010 were transferred from other institutions for treatment (36 male Epispadias, 97 bladder exstrophy, and 14 cloacal exstrophy). Only 12 centres treated six or more exstrophy and or Epispadias patients during the study period; 52 treated between one and five patients, of which 22 treated only one case in 12 months. Discussion This study provides a contemporary incidence of the BEEC in Europe. It demonstrates also that only a minority (19%) of the European centres involved in the treatment of exstrophy can be considered “high volume” exstrophy centres. Conclusion There is a case for proposing a rationalisation of the treatment of this group of conditions in a small number of exstrophy units around Europe. Download high-res image (263KB) Download full-size image Figure . Sample of the online questionnaire emailed to the local investigators.
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the tunica vaginalis flap as an adjunct to Epispadias repair a preliminary report
Urology, 2015Co-Authors: Nilay Gandhi, Ezekiel E Young, Sunil S Reddy, Annelies Carl, John P GearhartAbstract:Objective To report our preliminary institutional experience of incorporating a tunica vaginalis flap (TVF) as an adjunct into primary or secondary Epispadias repair. Patients and Methods A prospectively maintained institutionally approved database of exstrophy-Epispadias complex patients was used to identify and retrospectively review male patients who underwent Epispadias repair from September 2010 to October 2014 at the authors' institution. Patients who underwent Epispadias repair with TVF were identified and their clinical outcomes were measured. Results A total of 49 male patients were identified as meeting inclusion criteria, of which 15 (2 isolated Epispadias, 13 classic bladder exstrophy) underwent Epispadias repair incorporating a TVF. Median age at time of repair was 12 months (interquartile range [IQR] 10-15.5). A median of 4 layers (IQR 4-5) was incorporated into each repair closure, applying EVICEL Fibrin Sealant as an additional layer in all patients. All patients received preoperative testosterone injection therapy of 2 mg/kg 5 and 2 weeks before surgery for penile growth. There were no intraoperative complications. Median follow-up of 19 months (IQR 12-23) revealed 5 patients who underwent Epispadias revision, 3 (20%) of which developed a urethrocutaneous fistula. All patients had a successful repair without recurrence. Conclusion The authors have found the utilization of a TVF with Epispadias repairs to be beneficial but no better than the repair with our routine soft-tissue coverage in primary Epispadias repair. However, in patients presenting with complex anatomy and limited tissue reserves, a TVF is an important adjunct to Epispadias repair and/or revision and may lead to improved outcomes.
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sexual health outcomes in adults with complete male Epispadias
The Journal of Urology, 2015Co-Authors: Sunil S Reddy, Brian M Inouye, Uzoma A Anele, Mahmoud Abdelwahab, Brian Le, John P GearhartAbstract:Purpose: Complete male Epispadias is a rare congenital anomaly characterized by failed closure of the entire penopubic dorsal urethra. Epispadias repair is typically performed during infancy, and resultant genitourinary abnormalities can have a marked impact on adult life. We assess long-term post-reconstruction sexual health and fertility outcomes in adults with complete male Epispadias.Materials and Methods: A total of 132 patients 18 years or older with complete male Epispadias who had undergone reconstruction were identified from a prospectively maintained, institutionally approved database. Patients who could be contacted were asked to complete a telephone survey regarding sexual function. Reconstructive history and clinical details were obtained by chart/database review.Results: Of 132 patients with complete male Epispadias 74 met inclusion criteria and 15 (20%) completed the questionnaire. Seven patients (47%) reported currently being in a relationship. Although 12 patients (80%) reported overall s...
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Prospective study on the incidence of bladder/cloacal exstrophy and Epispadias in Europe
Journal of Pediatric Urology, 2015Co-Authors: Raimondo M Cervellione, Paolo Caione, John P Gearhart, Alberto Mantovani, Guy Bogaert, Rita Gobet, Alan P DicksonAbstract:Summary Objective On behalf of the European Society of Paediatric Urology (ESPU), a prospective study was designed with the aim of defining the actual number of babies born with bladder exstrophy, cloacal exstrophy, and Epispadias in Europe over a 12-month period, and verifying the distribution of the exstrophy patients born during the study period among the different paediatric urology centres in Europe. Study design The study was structured with a chief investigator and one national investigator for each country enrolled in the study. The national investigators nominated one local investigator for each European centre of paediatric surgery/paediatric urology and urology where the exstrophy complex could potentially be treated. The local investigators were responsible for reporting babies treated in their institutions for bladder/cloacal exstrophy and/or Epispadias. During 2010, every 3 months, an electronic survey (Figure) was e-mailed to the local investigators asking them to report babies treated or referred for treatment during the previous 3 months. Results One-hundred and sixteen centres in 27 European counties were enrolled in the study. The overall response rate for the four online surveys was 79%. Two-hundred and thirty-eight babies were reported to be born with a condition within the bladder exstrophy Epispadias complex (BEEC): 71 primary Epispadias (66 males), 146 classic bladder exstrophy (97 males) of which two were female bladder exstrophy variant, and 21 cloacal exstrophy (17 males). Two of 67 (3%) male Epispadias, 24/146 (16.4%) bladder exstrophy, and 6/21 (28%) cloacal exstrophy were antenatally diagnosed. Associated anomalies were reported in 2/71 (2.8%) Epispadias patients, 8/146 (5.5%) bladder exstrophy patients, and 15/21 (71.4%) cloacal exstrophy patients. One-hundred and forty-seven (62%) of the 238 babies born in Europe with a condition within the exstrophy spectrum during 2010 were transferred from other institutions for treatment (36 male Epispadias, 97 bladder exstrophy, and 14 cloacal exstrophy). Only 12 centres treated six or more exstrophy and or Epispadias patients during the study period; 52 treated between one and five patients, of which 22 treated only one case in 12 months. Discussion This study provides a contemporary incidence of the BEEC in Europe. It demonstrates also that only a minority (19%) of the European centres involved in the treatment of exstrophy can be considered “high volume” exstrophy centres. Conclusion There is a case for proposing a rationalisation of the treatment of this group of conditions in a small number of exstrophy units around Europe. Download high-res image (263KB) Download full-size image Figure . Sample of the online questionnaire emailed to the local investigators.
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modern management of the exstrophy Epispadias complex
Surgery research and practice, 2014Co-Authors: Brian M Inouye, Ezekiel E Young, Heather N Di Carlo, Ali Tourchi, John P GearhartAbstract:The exstrophy-Epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair Epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-Epispadias complex and potential surgical complications.
Robert D. Jeffs - One of the best experts on this subject based on the ideXlab platform.
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combined bladder neck reconstruction and Epispadias repair for exstrophy Epispadias complex
The Journal of Urology, 2001Co-Authors: Ilhami Surer, Robert D. Jeffs, Linda A Baker, John P GearhartAbstract:Purpose: The achievement of satisfactory continence in the management of classic bladder exstrophy remains a surgical challenge. During the last 20 years a staged approach to the management of the exstrophy-Epispadias complex has been used at many exstrophy centers to attain this goal. In select cases repairs can be combined to reduce the number of mandatory operations to achieve continence. We retrospectively review our experience with, and long-term results and complications of combined bladder neck reconstruction and Epispadias repair.Materials and Methods: A total of 19 boys with classic bladder exstrophy (17) and complete male Epispadias (2) underwent combined bladder neck reconstruction and Epispadias repair between 1982 and 1999. Primary closure was performed elsewhere in 16 cases and osteotomy was performed at primary closure in 8 (42%). All patients have undergone modified Cantwell-Ransley Epispadias repair except for 2 who underwent a Young procedure.Results: At the time of combined bladder neck...
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the modified cantwell ransley repair for exstrophy and Epispadias 10 year experience
The Journal of Urology, 2000Co-Authors: Ilhami Surer, Robert D. Jeffs, Linda A Baker, John P GearhartAbstract:Purpose: We evaluate our experience with the modified Cantwell-Ransley Epispadias repair technique to determine the complications and long-term results.Materials and Methods: The modified Cantwell-Ransley Epispadias repair was performed during the last 10 years in 93 males of whom 79 had classic bladder exstrophy and 14 had complete Epispadias. Primary repair was performed in 65 boys with classic bladder exstrophy and 12 with Epispadias, and secondary repair was done after prior failed reconstruction in 14 boys with classic exstrophy and 2 with complete Epispadias.Results: At mean followup of 68 months 87 patients had a horizontal or downward angled penis while standing. The incidence of urethrocutaneous fistulas was 23% in the immediate postoperative period and 19% at 3 months. A urethral stricture at the proximal anastomotic area developed in 7 patients and 5 (4 with exstrophy and 1 with Epispadias) had minor skin separations of the dorsal penile skin closure. Catheterization or cystoscopy in 77 cases r...
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combined bladder closure and Epispadias repair in the reconstruction of bladder exstrophy
The Journal of Urology, 1998Co-Authors: John P Gearhart, Ranjiv Mathews, Sian Taylor, Robert D. JeffsAbstract:AbstractPurpose: Children with bladder exstrophy in whom initial closure fails are less likely to achieve adequate bladder capacity for later bladder neck reconstruction and continence. Repeat bladder closure may be combined with Epispadias repair as initial management, decreasing the need for repeat anesthesia as well as providing increased outlet resistance to permit bladder growth in preparation for later bladder neck reconstruction. We compared results in boys who underwent combined bladder closure and Epispadias repair or staged reconstruction.Materials and Methods: A total of 16 boys with a mean age of 22 months with bladder exstrophy were referred for bladder closure after previous closure had failed in 15 and for delayed primary closure in 1. Osteotomies were performed in all patients, with bladder closure and Epispadias repair as initial treatment. Results were compared to those in 21 boys with a mean age of 26 months in whom exstrophy closure had failed, and who underwent standard staged reconst...
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Urolithiasis in the Exstrophy-Epispadias Complex
The Journal of Urology, 1997Co-Authors: Richard I. Silver, Robert D. Jeffs, David-alexandre C. Gros, John P GearhartAbstract:AbstractPurpose: We sought to determine the incidence of urolithiasis in patients with the exstrophy-Epispadias complex, associated risk factors and guidelines for the proper clinical management of this problem.Materials and Methods: We retrospectively reviewed the chart and x-rays of 530 patients with the exstrophy-Epispadias complex treated at our institution.Results: Stones formed in 77 of the 530 patients (15%), including 16% of those with classic exstrophy, 25% of those with cloacal exstrophy and 3% of those with Epispadias. White and male individuals were most commonly affected. Most calculi formed in the bladder, whether native or augmented by enterocystoplasty. Standard techniques were successful for stone removal. Most calculi consisted of calcium apatite, calcium oxalate monohydrate or magnesium ammonium phosphate, usually in combination with at least 1 other element. The risk of stone formation was associated with augmentation cystoplasty (p
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The Cantwell-ransley Epispadias repair in exstrophy and Epispadias: Lessons learned
Urology, 1995Co-Authors: John P Gearhart, Christopher M. Sciortino, Jacob Ben-chaim, Dennis S. Peppas, Robert D. JeffsAbstract:OBJECTIVES: We evaluated our experience with the Cantwell-Ransley Epispadias repair to determine the lessons that have been learned with the increased experience and follow-up. METHODS: A total of 75 boys (60 with bladder exstrophy and 15 with complete Epispadias) underwent a Cantwell-Ransley Epispadias repair at our institute in the last 6 years. Primary repair was performed in 58 boys (45 with exstrophy and 13 with Epispadias), and secondary repair was performed after prior failed closure in 17 boys (12 at the secondary exstrophy closure, 3 with exstrophy, and 2 with complete Epispadias). RESULTS: At a mean follow-up of 28 months, all patients had a horizontal or downward angled penis while standing. The incidence of urethrocutaneous fistulas in the immediate postoperative state was 21% and at 3 months was 15%. The incidence of urethrocutaneous fistulas was no more in those patients in whom paraexstrophy skin flaps were used at anterior closure than in those in whom the urethral plate was left intact. Two patients developed a urethral stricture at the proximal anastomotic area, and 4 patients had minor skin separation of the dorsal penile skin closure. Catheterization or cystoscopy, or both, has been performed in 60 patients and revealed an easily negotiable urethral channel in all. CONCLUSIONS: The Cantwell-Ransley Epispadias repair offers a straighter urethra, better correction of chordee and cosmesis, and a lower fistula rate in the exstrophy or Epispadias patient.
Stephen A. Kramer - One of the best experts on this subject based on the ideXlab platform.
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vesical neck reconstruction in patients with the exstrophy Epispadias complex
The Journal of Urology, 1996Co-Authors: Daniel R Mcmahon, Douglas A Husmann, Mark P Cain, Stephen A. KramerAbstract:AbstractPurpose: We evaluated the factors critical in achieving urinary continence in patients with the exstrophy-Epispadias complex.Materials and Methods: A total of 51 patients with Epispadias and 33 with classic bladder exstrophy underwent vesical neck reconstruction. Patient records were reviewed to identify factors associated with the achievement of continence, including timing of bladder closure and urethroplasty, effect of bladder capacity before and after vesical neck reconstruction, and effect of enterocystoplasty.Results: Complete urinary continence was achieved in 42 of the 51 patients with Epispadias (82 percent) and in 23 of the 33 with exstrophy (70 percent). Delayed bladder closure did not affect the ability to gain continence but increased the likelihood of subsequent enterocystoplasty (12 of 19 patients, 63 percent) compared to early bladder closure (5 of 14 patients, 36 percent).Conclusions: Preliminary urethroplasty did not enhance urinary control or reduce the need for enterocystoplast...
Sunil S Reddy - One of the best experts on this subject based on the ideXlab platform.
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the tunica vaginalis flap as an adjunct to Epispadias repair a preliminary report
Urology, 2015Co-Authors: Nilay Gandhi, Ezekiel E Young, Sunil S Reddy, Annelies Carl, John P GearhartAbstract:Objective To report our preliminary institutional experience of incorporating a tunica vaginalis flap (TVF) as an adjunct into primary or secondary Epispadias repair. Patients and Methods A prospectively maintained institutionally approved database of exstrophy-Epispadias complex patients was used to identify and retrospectively review male patients who underwent Epispadias repair from September 2010 to October 2014 at the authors' institution. Patients who underwent Epispadias repair with TVF were identified and their clinical outcomes were measured. Results A total of 49 male patients were identified as meeting inclusion criteria, of which 15 (2 isolated Epispadias, 13 classic bladder exstrophy) underwent Epispadias repair incorporating a TVF. Median age at time of repair was 12 months (interquartile range [IQR] 10-15.5). A median of 4 layers (IQR 4-5) was incorporated into each repair closure, applying EVICEL Fibrin Sealant as an additional layer in all patients. All patients received preoperative testosterone injection therapy of 2 mg/kg 5 and 2 weeks before surgery for penile growth. There were no intraoperative complications. Median follow-up of 19 months (IQR 12-23) revealed 5 patients who underwent Epispadias revision, 3 (20%) of which developed a urethrocutaneous fistula. All patients had a successful repair without recurrence. Conclusion The authors have found the utilization of a TVF with Epispadias repairs to be beneficial but no better than the repair with our routine soft-tissue coverage in primary Epispadias repair. However, in patients presenting with complex anatomy and limited tissue reserves, a TVF is an important adjunct to Epispadias repair and/or revision and may lead to improved outcomes.
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sexual health outcomes in adults with complete male Epispadias
The Journal of Urology, 2015Co-Authors: Sunil S Reddy, Brian M Inouye, Uzoma A Anele, Mahmoud Abdelwahab, Brian Le, John P GearhartAbstract:Purpose: Complete male Epispadias is a rare congenital anomaly characterized by failed closure of the entire penopubic dorsal urethra. Epispadias repair is typically performed during infancy, and resultant genitourinary abnormalities can have a marked impact on adult life. We assess long-term post-reconstruction sexual health and fertility outcomes in adults with complete male Epispadias.Materials and Methods: A total of 132 patients 18 years or older with complete male Epispadias who had undergone reconstruction were identified from a prospectively maintained, institutionally approved database. Patients who could be contacted were asked to complete a telephone survey regarding sexual function. Reconstructive history and clinical details were obtained by chart/database review.Results: Of 132 patients with complete male Epispadias 74 met inclusion criteria and 15 (20%) completed the questionnaire. Seven patients (47%) reported currently being in a relationship. Although 12 patients (80%) reported overall s...
Ezekiel E Young - One of the best experts on this subject based on the ideXlab platform.
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the tunica vaginalis flap as an adjunct to Epispadias repair a preliminary report
Urology, 2015Co-Authors: Nilay Gandhi, Ezekiel E Young, Sunil S Reddy, Annelies Carl, John P GearhartAbstract:Objective To report our preliminary institutional experience of incorporating a tunica vaginalis flap (TVF) as an adjunct into primary or secondary Epispadias repair. Patients and Methods A prospectively maintained institutionally approved database of exstrophy-Epispadias complex patients was used to identify and retrospectively review male patients who underwent Epispadias repair from September 2010 to October 2014 at the authors' institution. Patients who underwent Epispadias repair with TVF were identified and their clinical outcomes were measured. Results A total of 49 male patients were identified as meeting inclusion criteria, of which 15 (2 isolated Epispadias, 13 classic bladder exstrophy) underwent Epispadias repair incorporating a TVF. Median age at time of repair was 12 months (interquartile range [IQR] 10-15.5). A median of 4 layers (IQR 4-5) was incorporated into each repair closure, applying EVICEL Fibrin Sealant as an additional layer in all patients. All patients received preoperative testosterone injection therapy of 2 mg/kg 5 and 2 weeks before surgery for penile growth. There were no intraoperative complications. Median follow-up of 19 months (IQR 12-23) revealed 5 patients who underwent Epispadias revision, 3 (20%) of which developed a urethrocutaneous fistula. All patients had a successful repair without recurrence. Conclusion The authors have found the utilization of a TVF with Epispadias repairs to be beneficial but no better than the repair with our routine soft-tissue coverage in primary Epispadias repair. However, in patients presenting with complex anatomy and limited tissue reserves, a TVF is an important adjunct to Epispadias repair and/or revision and may lead to improved outcomes.
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modern management of the exstrophy Epispadias complex
Surgery research and practice, 2014Co-Authors: Brian M Inouye, Ezekiel E Young, Heather N Di Carlo, Ali Tourchi, John P GearhartAbstract:The exstrophy-Epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair Epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-Epispadias complex and potential surgical complications.