The urinary diversion in children with bladder exstrophy and epispadias: alternative to primary bladder closure (original) (raw)
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Journal of case report and scientific images , 2022
Epispadias is a congenital anomaly in which the urethral meatus opens dorsally on top of the penis, medial to the glands, or with absent or bifid clitoris in females. It often occurs as part of a complex anomaly called the bladder extrophy epispadias complex (BEEC) along with exposed bladder and bifid pelvic brim. The diagnosis of bladder extrophy epispadias complex is often clinical with identifications of the various component of the anomalies. Although this condition is rare it present with wide spectrum of deformities which ranged from mild to more complex anomalies, its management especially the complex variety has been a major challenge to pediatric surgeons worldwide. With a male to female ratio of 2-3:1, the occurrence of isolated epispadias in female patient is even rarer. Our patient is a 4-year old girl who presented with urinary incontinence since birth. She has never achieved urinary continence up to the age of 4-years, there is no history of any prior urethral procedures, no history of any trauma to the perineum, no passage of stone or blood in the urine. Child is the second of her mother's three children and her younger ones has achieved urinary continence. Pregnancy, delivery and puerperal history were essentially uneventful. On examination, we found a young girl, calm, but apprehensive otherwise stable, abdomen was full, moves with respiration, no area of tenderness, no palpable organomegaly, no intra-abdominal masses felt, there was distorted female genitalia with bifid clitoris and patulous urethral meatus. There was a normal vaginal orifice with intact hymen with poorly developed mons pubis and labia. A clinical diagnosis of isolated epispadias with urinary incontinence to rule out ectopic ureter in a 4-year old girl was made. She was worked up and prepared for surgical repair, and had a single stage procedure done. Post-operative cosmetic appearance of the genitalia was acceptable. Urethral catheter was removed on the postoperative day 10 and patient had achieved some continent of urine. She generally did well and has been on outpatient follow up visits at the pediatrics surgical outpatient clinic.
Care of child having bladder exstrophy and epispadias
International Journal of Applied Research, 2021
The bladder exstrophy-epispadias-cloacal exstrophy complex is caused by a developmental abnormality that occurs 4-5 weeks after conception in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles. The treatment includes Bladder closure and abdominal wall repair operation, Kelly procedure, Bladder Augmentation, and Epispadias repair.
Combined bladder exstrophy and epispadias repair
BMJ Case …, 2011
A 2-year-old male child of exstrophy-epispadias complex presented to us. He had not received any treatment at the time of presentation. It was decided to perform a combined bladder exstrophy and epispadias repair under general anaesthesia. There was a wide diastasis of symphysis pubis, it was decided to perform a bilateral anterior innominate and vertical iliac osteotomy. The bladder was closed in two layers. The urethroplasty and penile reconstruction was done by modifi ed Cantwell-Ransley repair. At the completion of procedure to prevent distraction of pubis, the baby was strapped using elastoplast bandage. The child had a very good cosmetic outcome, good pubic and rectus muscle approximation. On clamping the suprapubic catheter, the patient started passing urine per urethrally and there was a small penopubic fi stula. Thereafter the suprapubic catheter was removed. The urine culture was sterile and the patient was discharged on prophylactic antibiotics. The patient is due for follow-up.
World journal of …, 2010
Purpose Presentation of bladder exstrophy epispadias complex (BEEC) during adulthood is rare. A major surgical challenge encountered in adults with BEEC is the closure of the anterior abdominal wall defect. In this case series we report our experience with the abdominal closure without osteotomy. Materials and methods Five adult male patients with BEEC were managed at our center. None of the patients had any prior attempts of surgical correction. A right renal tumor was incidentally discovered in one patient and a right partial nephrectomy was performed. All patients underwent urinary diversion and abdominal wall closure at the same operative setting except one. Cystectomy was performed in two patients while the vesical plate muscular coat was utilized for providing support to the anterior abdominal wall in the other three patients. One patient underwent an ileal conduit, while the other patients underwent continent cutaneous diversion. None of our patients underwent osteotomy. Results Follow-up ranged from 6 to 52 months (mean = 31). Patients who underwent continent cutaneous diversion were continent by day and night. One patient had wound infection and partial disruption and secondary sutures were performed. One patient developed stomal stenosis and was managed by stomal dilatation. All patients showed marked satisfaction with their body image following surgery. Conclusion Management of BEEC in adults is challenging. Utilization of the vesical plate muscular coat for supporting the anterior abdominal wall is a reliable method for abdominal reconstruction. Urinary diversion and abdominal wall closure in one surgical procedure without osteotomy is feasible.
The Journal of Urology, 2001
Purpose: The surgical repair of bladder exstrophy remains challenging for the pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial closure. Materials and Methods: Between November 1998 and April 1999, 9 boys and 2 girls with bladder exstrophy underwent complete repair. This procedure was performed in the first 72 hours of life in 4 boys and at age 3 months in 1 girl. Complete repair with osteotomy was performed after failed initial closure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The bladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respectively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery.
Complete Repair of Bladder Exstrophy in Boys: Can Hypospadias Be Avoided?
European Urology, 2005
Introduction: Complete repair of classic bladder exstrophy in male newborns has been successful with minimal morbidity. However, the technique may create hypospadias in some cases. We have recently adopted a modification to obtain an orthotopic meatus in bladder exstrophy boys. Materials and methods: Between November 1998 and December 2002 complete repair of classic bladder exstrophy was carried out in 27 boys. Complete penile disassembly was performed in 22 boys including 4 newborns and 18 older children; mean age 3 AE 2 years old. Modified disassembly was used in the last 5 boys including 4 newborns and a 9-month-old boy. During repair of epispadias, the dissection starts on the ventral aspect of the penis as usual. The urethral plate is separated from both corpora cavernosa to allow ventral transposition of the plate. In the complete disassembly technique, the urethral plate is completely separated from both hemiglans. In this modification, while the urethral plate is completely separated from both corpora cavernosa, the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans. Thus, when the plate is tubularized with fine interrupted sutures the meatus ends up at the tip of the glans penis. The symphysis pubis is re-approximated as usual and corpora cavernosa are approximated dorsally in the midline. Results: There was no major complication. Mean follow-up was 43 AE 7 and 7 AE 4 months for complete and modified disassembly groups respectively. Of the 22 boys, who underwent complete disassembly, 15 (68%) ended up with hypospadias and 7 (32%) had an orthotopic meatus. Modified disassembly has resulted in an excellent cosmetic appearance and orthotopic meatus in the 5 boys. Penile length was not shorter than boys who underwent complete disassembly. Parents of the 5 boys noticed normal straight morning erection. Conclusion: Although complete penile disassembly allows ventral placement of the urethra, hypospadias is created in approximately two thirds of the cases. When the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans (modified disassembly), orthotopic meatus can be obtained in all boys. With modified disassembly, posterior mobilization of the bladder and urethra does not result in corporal angulations or shortening because of the proportionate inward movement of the corpora that accompanies symphyseal approximation. The modification is feasible in newborns and infants. These short-term results may obviate the need for later penile reconstructive procedure.
2011
After primary surgery seven of the 10 girls were continent and all could void normally. • Of the three failed cases (including two that underwent BNP using absorbable suture), two became dry after injection of bulking agent into the BN and the remaining girl underwent BN reconstruction; she was socially continent at the final follow-up. • Ultimately, all 10 girls were socially continent, including six who were completely dry. • Urodynamic studies showed an increase in mean bladder capacity (P < 0.001) and a significant time-dependent improvement of the leak-point pressure and maximum urinary flow rate. CONCLUSIONS • The present series suggests that singlestage subsymphyseal urethrogenitoplasty together with cystoscopic-guided BNP for management of female epispadias is reliable and effective with definite advantages. • The high success rate and safety of the procedure are important factors for the introduction of this method as a valid option for treatment of this rare congenital anomaly.
CHAPTER 92 Bladder Exstrophy and Epispadias
2011
Introduction Exstrophy and epispadias are part of a spectrum of anomalies characterised by exposure of part or all of the mucosa of the lower urinary tract (bladder and urethra) to the external environment through a defect in the anterior abdominal wall. At one end of this spectrum is cloacal exstrophy, which is the most complex of these anomalies. It is characterised by exposure of the bladder mucosa together with the mucosa of the hind gut to the external environment through an anterior abdominal wall defect. At the other end of the spectrum is epispadias, which is characterised by a normal abdominal wall and bladder with a dorsal external urethral meatus, which may be anywhere from the base of the penis to the glans and may or may not be associated with an incompetent bladder neck and sphincter mechanism. Between these two extremes exist variations in pathology, with the most prevalent being classic bladder exstrophy. In most of the anomalies in this spectrum, there is concomitan...