Bladder exstrophy with inguinal hernia: a case report

Exstrophy of urinary bladder with epispadias involves protrusion of the urinary bladder through a defect in the lower abdominal wall accompanied by separation of pubic symphysis. It is a rare but challenging condition that causes significant physical, functional, social, sexual and psychological problems later in life. Bladder exstrophy commonly involves males and most cases are sporadic.' Inguinal hernia is a complication associated with bladder exstrophy and it occurs due to lack of obliquity of the inguinal canal secondary to pubic diastasis.' Authors report here, a case of antenatally diagnosed case of classic bladder exstrophy associated with left sided inguinal hernia which was incidentally diagnosed on tenth day of life. Our neonate underwent primary bladder closure with herniotomy. Staged reconstruction of epispadias and bladder neck has been planned at a later date. Recurrence of inguinal hernia after repair is common and bilateral inguinal exploration while performing herniotomy is advised to prevent its recurrence. Prognosis of such cases depends on the degree of continence achieved. With timely reconstructive surgery, continence rates can be as high as 60-70 percent.

Analysis of the effect about tubularized urethral plate disassembly for one-stage midshaft epispadias repair in infant / 中华泌尿外科杂志

Objective To evaluate the safety and efficacy of tabularized urethral plate disassembly for epispadias repair in infant.Methods From January 2016 to September 2018,10 boys aged between 18 to 36 months old with mishaft epispadias were included.The main complaint was that the urethral opening was found on the dorsal side of the penis.Preoperative cystography revealed that 3 of them had unilateral vesicoureteral reflux (Grade Ⅰ),and white blood cells in routine urinary in all 10 patients were negative.Physical examination:the penis is short and flat,the width is 17-25 mm,average is 20.7 mm,the penis curvature is 15°-30°,average is 21.5°,and the urethral opening located on the dorsal side of the penis.The anesthesia method was selected for tracheal intubation,intravenous anesthesia combined with caudal anesthesia,and the surgical position was supine position.The surgical method:one stage of tabularized urethral plate disassembly urethroplasty:during the operation,the penile curve was corrected by free urethral plate and penile degloving.Two of them were unsatisfied with the correction of the curvature,and the ventral tunica folded was applicate.To avoid urethral plate ischemia,attention should pay to blood supply protection.The two corpus cavernosum are separated in the root,and the urethra is completely displaced to the ventral side of the cavernous body.The ventral side of the urethral under glans is shaped into a fissured that conforms to the anatomy.The prognosis and surgical choice of the midshaft epispadias were discussed combined with literature.Result The operation time was 130-200 min with an average of 157 mins.Intraoperative hemorrhage 5-30 ml,average is 16 ml,1 case of glans skin was black one day after surgery,with enhanced dressing change.After 1 month,the glans was local atrophy and scar formation.No skin incision infection case.After discharge from the hospital,the follow up through the internet and outpatients for 3-40 months,average is 21 months,2 cases with urethral fistula,more surgery to repair the fistula successfully after 6 months.The penile curvature was corrected in 8 cases,and the residual curvature of 2 cases was about 10-15°,which was temporarily observed.Patients with unilateral vesicoureteral reflux preoperatively,they still suffered from vesicoureteral reflux in the 6-12 months regular review after surgery without any intervention due to white blood cell was negative in urine routine.All cases had urinary patency and no cases with urethral stricture.Conclusion Tubularized urethral plate disassembly is a effective and simple procedure that can correct the midshaft epispadias in infant.

Analysis of the effect about tubularized urethral plate disassembly for one-stage midshaft epispadias repair in infant / 中华泌尿外科杂志

Objective@#To evaluate the safety and efficacy of tabularized urethral plate disassembly for epispadias repair in infant.@*Methods@#From January 2016 to September 2018, 10 boys aged between 18 to 36 months old with mishaft epispadias were included. The main complaint was that the urethral opening was found on the dorsal side of the penis. Preoperative cystography revealed that 3 of them had unilateral vesicoureteral reflux (Grade Ⅰ), and white blood cells in routine urinary in all 10 patients were negative. Physical examination: the penis is short and flat, the width is 17-25 mm, average is 20.7 mm, the penis curvature is 15°-30°, average is 21.5°, and the urethral opening located on the dorsal side of the penis. The anesthesia method was selected for tracheal intubation, intravenous anesthesia combined with caudal anesthesia, and the surgical position was supine position. The surgical method: one stage of tabularized urethral plate disassembly urethroplasty: during the operation, the penile curve was corrected by free urethral plate and penile degloving. Two of them were unsatisfied with the correction of the curvature, and the ventral tunica folded was applicate. To avoid urethral plate ischemia, attention should pay to blood supply protection. The two corpus cavernosum are separated in the root, and the urethra is completely displaced to the ventral side of the cavernous body. The ventral side of the urethral under glans is shaped into a fissured that conforms to the anatomy. The prognosis and surgical choice of the midshaft epispadias were discussed combined with literature.@*Result@#The operation time was 130-200 min with an average of 157 mins. Intraoperative hemorrhage 5-30 ml, average is 16 ml, 1 case of glans skin was black one day after surgery, with enhanced dressing change. After 1 month, the glans was local atrophy and scar formation. No skin incision infection case. After discharge from the hospital, the follow up through the internet and outpatients for 3-40 months, average is 21 months, 2 cases with urethral fistula, more surgery to repair the fistula successfully after 6 months. The penile curvature was corrected in 8 cases, and the residual curvature of 2 cases was about 10-15°, which was temporarily observed. Patients with unilateral vesicoureteral reflux preoperatively, they still suffered from vesicoureteral reflux in the 6-12 months regular review after surgery without any intervention due to white blood cell was negative in urine routine. All cases had urinary patency and no cases with urethral stricture.@*Conclusion@#Tubularized urethral plate disassembly is a effective and simple procedure that can correct the midshaft epispadias in infant.

Complejo extrofia vesical-epispadias. Reporte de un caso / Bladder exstrophy-epispadias complex. A case report

RESUMEN La extrofia vesical es una grave anomalía del desarrollo embriológico del seno urogenital y del sistema esquelético vecino. Se caracteriza por una falla de la pared anterior del abdomen y de la vejiga, cuya cara posterior-inferior, evertida hacia afuera, protruye en forma de hernia y exterioriza por completo el trígono, con los meatos ureterales eyaculando directamente hacia la pared abdominal; la asociación con epispadias es la regla. Se describió el caso de una paciente pediátrica con diagnóstico del complejo extrofia vesical-epispadias, entidad poco frecuente en nuestro medio, la cual evolucionó de manera favorable y se encuentra en espera de un segundo tiempo quirúrgico como parte de su tratamiento (AU).

ABSTRACT The bladder exstrophy is a serious anomaly of theurogenital sinus´ embryologic development and the close skeletal system, characterized by a failure of the abdomen anterior wall and bladder, whose posterior inferior face, turned outside out, protrudes like an hernia and completely exteriorizes the trigone, with the ureteral meatuses ejaculating right down to the abdominal wall; the association with epispadias is the rule. It was described the case of a pediatric patient with diagnosis of exstrophy- epispadias complex, infrequent entity in our country, who has evolved with great results and is waiting a second surgical time to finish her treatment (AU).

Experiencia en el abordaje de pacientes con complejo extrofia-epispadias en un centro de alto nivel de complejidad en Colombia, 10 años / 10-years experience in the management of bladder exstrophy-epispadias complex in a tertiary care health centre in Colombia

Objetivos El complejo extrofia-epispadias (CEE) se considera una de las malformaciones más severas de la línea media de compromiso multisistémico. La extrofia vesical es la presentación más frecuente en el espectro del complejo. Esta patología tiene un alto impacto en la calidad de vida. A pesar de la relación entre un cierre primario temprano y mejores resultados, en nuestro medio la remisión es tardía y la experiencia es escasa. El objetivo del siguiente estudio es mostrar la experiencia en el abordaje de CEE en los últimos 10 años en una institución de alto nivel de complejidad y remisión en Colombia. Materiales y métodos Se realiza un estudio observacional descriptivo, con una serie de casos del 2006 al 2016. Resultados En 10 años, se presentaron 5 casos de CEE en un centro de alta complejidad y remisión en Colombia. La mayoría de los pacientes han tenido múltiples intervenciones; la edad del primer procedimiento fue 829 días en promedio (27,6 meses). Se ha tenido un seguimiento postoperatorio promedio de 2,8 años. No se han presentado neoplasias en el seguimiento. Las comorbilidades más frecuentes son infección y litiasis. Ninguno de los pacientes contactados reportó inicio de vida sexual. La escala International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UI-SF) tuvo un promedio de 9 puntos. Existen factores sociales asociados en nuestro medio. Conclusión El CEE requiere un abordaje temprano y multidisciplinario en instituciones con experiencia; los resultados en continencia urinaria, función sexual, desarrollo psicosocial y calidad de vida están sujetos a tratamiento oportuno de la patología.

Objective Exstrophy-epispadias complex (ECC) is considered one of the most severe midline abnormalities. Exstrophy of the bladder is the most frequent presentation of the exstrophy-epispadias spectrum. The disease has high impact on the quality of life. In developing countries, patient referral to experienced centres is often delayed. The experience in the disease is generally poor. The aim of this study is to describe the experience in the approach to EEC in the past 10 years in an institution of high level of complexity in Colombia. Materials and methods An observational descriptive study of a case series. Results A total of 5 cases of ECC were identified in the last 10 years in an institution of high level of complexity in Colombia. All the patients had multiple interventions. The mean follow-up time was 2.8 years. No neoplasms were recognised. The most common comorbidities were lithiasis and urinary tract infections. None of the patients contacted reported starting a sex life. The International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UC-SF) gave a mean of 9 points. Conclusion The EEC requires an early and cross-disciplinary assessment in experienced centres. There are additional factors associated with the prognosis in our area.

Diagnosis and treatment of epispadias with complete prepuce / 中华泌尿外科杂志

Objective To summarize the diagnosis and treatment of epispadias with complete prepuce.Methods Between January 2007 and April 2017,clinical records were reviewed for 5 patients diagnosed as epispadias with complete prepuce.The patients were 34 to 66 months old (mean age 44.3 months),who presented with short beaked penis and nonretractile prepuce.A dorsal midline gap between corpora cavernosa could be felt on palpation.There was dorsal chordee with broad spade like glans.The preputial opening was stenosed and dorsally placed urethral opening after retraction of the prepuce.Two patients were incontinent before surgery.Epispadias were coronal type in 2 cases and penile type in 3 cases.All 5 cases were repaired by Thiersch-Duplay procedure.Results The mean follow-up time was 44.5 months (ranged 3 to 98 months).The stricture occurred in 1 patient 1 month after operation,and after dilation and indwelling catheter for a month,the problem resolved.One needed a secondary preputial plasty due to redundant ventral foreskin 8 years after operation.In 3 of 5 cases,parental satisfaction was not achieved mainly due to short length.Only 1 case was incontinent after surgery and the other four achieved urinary continence.Voiding cystourethrogram (VCUG) and ultrasonography (USG) were done in 2 patients who were incontinent before surgery.One case presented right vesicoureteral reflux of Grade Ⅱ and the other was normal.The urodynamic results of 2 patients were normal.Conclusions Epispadias with complete prepuce is extremely rare and its preoperative diagnosis remains difficult.A definitive diagnosis is based upon history,physical examination and imaging tests.Fewer complications and better continence rates were achieved in the boys with epispadias and intact prepuce.Penile length is an important factor affecting surgical outcome.

Short-term outcome of Kelly procedure for bladder exstrophy- epispadias complex / 中华泌尿外科杂志

Objective To evaluate the short-term outcome of the Kelly procedure for bladder exstrophy-epispadias complex.Methods Kelly procedure was performed in 5 cases from December 2015 in our institute. Retrospective analysis was done including the surgery and follow up.Results Two bladder exstrophy boys had histories of primary bladder closure without bladder neck reconstruction during the neonatal period. Another three epispadias boys had sever urethral defects with full incontinence. All cases undertook Kelly procedure and were followed up for 7.8 months (2-12 months). No severe complications such as glanular necrosis, urethral stricture and urethrocutaneous fistula was observed. Four cases were fully continent and one epispadias case had stress incontinence.Conclusions Kelly procedure produced satisfactory urinary continence and phallic cosmetics in bladder exstrophy-epispadias complex in children. Proper identification of the pudendal neurovascular bundle and repair of the external sphincter by levatorplasty help improve the outcome.

Our experience of penopubic epispadias repair by modified Cantwell-Ransley technique

We have operated on penopubic epispadias about 8 cases using modified Cantwell-Ransley technique. Penile reconstruction included wide mobilization of the urethral plate from the underlying corpora based on a mesentery from the ventral penoscrotal skin, corporeal lengthening by dividing the suspensory ligaments and attachments to the undersurface of the pubis, urethral and glandular tubularization, chordee correction by medial incision of the corpora with anastomosis dorsal to the urethra and penile skin coverage. Postoperatively, repairs were intubated with silicone stents for 10 days. Two urethrocutaneous fistulas developed, 1 of which closed spontaneously. One patient had a small degree of penile skin loss that did not affect the neourethra. All patients currently have a cosmetically acceptable penis. In our study the patients age ranged between 15 years minimum age to 21yrs, because our center is a tertiary care hospital so the patients presented to us at elderly age. The low complication rate of this procedure coupled with the better anatomical configuration of the neourethra makes it useful for urethral and penile reconstruction

Modified Cantwell-Ransley procedure for epispadias repair in 37 patients / 中华泌尿外科杂志

Objective To present the outcomes of modified Cantwell-Ransley procedure in 37 patients with isolated epispadias and exstrophy complex.Methods A total of 37 patients underwent modified Cantwell-Ransley epispadias repair.The median age at surgery was 6.6 years (range 1-32).Of the 37 patients,13 patients had isolated penopubic epispadias and 24 had bladder exstrophy with no previous attempts at epispadias repair.Nine patients with exstmphy complex underwent complete primary repair,while other 15 cases received urinary diversion.Results The patients were followed up for 6 months to 3 years (mean,12 months).All patients had normal conical symmetric glans/corpora and an orthotopic meatus.The complications included glans/corporal ischemia (1 case),urethral fistula (3 cases),and foreskin infection (1 case).Conclusions Modified Cantwell-Ransley procedure is safe and effective for epispadias repair.Cosmetic appearance and functional outcomes of the penis are encouraging.

Bladder exstrophy.

Bladder exstrophy, more properly, the exstrophy-epispadias complex is a rare congenital anomaly occurring once every 10,000-50,000 live births with a 2.3:1 as male & female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall. We report a case of bladder exstrophy managed by us.

Salvage reconstructive surgery in an adult patient with failed previous repair of an extrophy-epispadias complex: an operation with a functional and aesthetic purpose

Salvage surgical procedures after failed reconstruction for an extrophy-epispadias complex are extremely challenging. The goals are to restore continence and improve aesthetic appearance in order to provide quality of life and an improved body image to the patient. We describe the surgical steps in an adult patient who presented anal urinary incontinence and a poor body image due to the absence of an umbilicus and the presence of hypertrophic scars. He underwent a modified Mainz II reconstruction of the lower urinary tract at childhood for an extrophy-epispadias complex. Restoration of continence was achieved by the construction of a modified Mainz I pouch with a continent stoma in a neo-umbilicus. Body image improved dramatically by the construction of a neo-umbilicus, a surgical revision of the hypertrophic abdominal scars and an abdominoplasty. It is mandatory that such demanding surgery should only be attempted as a combined multidisciplinary effort with urologists and plastic/reconstructive surgeons.

Female Epispadias

Female epispadias is a rare congenital anomaly occuring in 1 in 484,0000 female population. Clinical course, management is discussed in the present case report to stress upon careful diagnosis and to decrease chances of mismanagement in highly morbid condition “urinary incontinence”.

A Case of Female Epispadias / 대한비뇨기과학회지

Female epispadias is an uncommon congenital anomaly in genitourinary tract. We experienced a case of female epispadias with total urinary incontinence which was improved with periurethral injection of Glutaraldehyde Cross-Linked Collagen(GAX-Collagen) into the area of the bladder neck. The procedure was simple to perform and without significant complications. Herein we report a case of female epispadias in 29-year old female.

A Case of Epispadias / 대한비뇨기과학회지

Epispadias is an extremely rare malformation that urethral opening is located on the dorsum of the penis. The severity of the epispadiac defect depends upon the degree of persistence of the cloacal membrane. Treatment goals of epispadiac patients include restoration of normal urinary control and establishment of a straight penis of adequate length that is functional for normal sexual intercourse. Recently we have experienced a case of glandular epispadias and report it with brief review of literature.

Two Cases of Epispadias / 대한비뇨기과학회지

Epispadias is an extremely rare malformation that urethral opening is located on the dorsum of the penis. Depending upon its location epispadias is classified as balanic, penile, or penopubic, the latter being by far the most common condition. Most patients of epispadias will have severe urinary incontinence and vesicoureteral reflux. The objects of management in epispadias are to achieve continence, to facilitate normal sexual activity, and to preserve renal function. Recently we have experienced two cases of epispadias without incontinence.

Penile Epispadias: A Case Report / 대한비뇨기과학회지

Penile epispadias is extremely rare congenital anomaly. Four cases of epispadias. two of which were penile. one was penopubic and one was subsymphyseal epispadias, had been reported previously in our department. We have experienced another case of penile epispadias. Herein this case of penile epispadias have been presented. A 7-year-old boy visited this clinic for short, broad based 'tent-like' penis with complete phimosis. The glans could not be inspected due to phimosis and a gutter was palpated between the two corpora. Voiding cystourethrogram revealed dorsally displaced urethra. There was no combined urological symptoms such as dysuria, frequency in urination or urinary incontinence. Urethral reconstruction by Young's method has been performed with excellent result.

A Case of Female Epispadias / 대한비뇨기과학회지

A case of female epispadias, which was observed in a 22-year old female having suffered from urinary incontinence since birth, is presented. Urethral construction resulted in complete urinary continence. The literature is reviewed briefly.