Epispadias Repair Research Articles

The Cephalotrigonal Reimplant in Bladder Neck Reconstruction for Patients with Exstrophy or Epispadias

A modified technique of ureteroneocystostomy with bladder neck plasty was used in 36 of 75 patients undergoing staged repair of bladder exstrophy or epispadias between 1986 and 1992. This procedure entails mobilizing the ureter while preserving the trigonal hiatus as with the cross-trigonal technique. The distal ureteral segments are directed superiorly toward the bladder dome rather than across the mid line. Of 75 patients 36 underwent cephalotrigonal reimplantation and 39 had a conventional cross-trigonal reimplant. Continence rate was 77% in the patients who underwent cephalotrigonal reimplantation and 72% in those who had a cross-trigonal reimplant. No patient had ureteral obstruction or vesicoureteral reflux.The ureter in exstrophy patients enters the bladder from an inferior position within the true pelvis. Directing the ureter superiorly rather than across the mid line provides a more gradual course through the hiatus and submucosal tunnel. The cranial course of the distal ureter frees more of the trigone for use in the rolled segment of the bladder neck and provides more muscle area for the tube. This is especially important in the patient in whom the distance between the mid prostate and trigone is particularly short.

Ventral preputial onlay island flap and proximal flip-flap technique for the repair of epispadias: A case report

Use of the ventral preputial island flap technique for repair of epispadias has yielded satisfying functional and cosmetic results. We report a modification of this technique in a patient with proximal penile epispadias without exstrophy. We used the Duckett modification of the onlay island flap using ventral preputial skin and coupled it with a proximal flip-flap of perimeatal skin without division of the urethral plate. This achieved an excellent result.

The Cantwell-Ransley technique for repair of epispadias : J.P. Gearhart, M.P. Leonard, J.K. Burgers et al. J Urol 148:851–854, (September), 1992

The Cantwell-Ransley technique for repair of epispadias : J.P. Gearhart, M.P. Leonard, J.K. Burgers et al. J Urol 148:851–854, (September), 1992

PAEDIATRIC UROLOGY: The Failed Exstrophy Closure: Strategy for Management

In the last 7 years, 29 boys and 11 girls have been referred with failure of their exstrophy closure; 38 patients had classic bladder exstrophy and 2 had cloacal exstrophy. Reclosure was performed for complete bladder dehiscence in 28 cases and for significant bladder prolapse in 10. Two patients underwent revision of the posterior urethra after primary closure due to a severe urethral stricture secondary to the use of para-exstrophy skin flaps. Six patients underwent posterior iliac osteotomy at the time of initial bladder closure and in 34 no osteotomy was performed. Thirty-seven patients underwent either posterior iliac osteotomy (15) or anterior innominate osteotomy (22). Epispadias repair together with reclosure of bladder exstrophy was done in 20 boys. The upper tract has remained normal in all patients. Fourteen have undergone subsequent bladder neck reconstruction. Seven patients have undergone simultaneous bladder neck reconstruction and augmentation cystoplasty and 1 has undergone augmentation cystoplasty and Mitrofanoff procedure; all are dry on intermittent self-catheterisation. The failed exstrophy reconstruction represents a formidable dilemma. However, a well planned reconstruction including osteotomy (even if previously performed), reclosure with or without epispadias repair or revision of the urethra can be performed with an excellent chance of proceeding with staged reconstruction.

Techniques to Create Urinary Continence in the Cloacal Exstrophy Patient

Of 15 patients with cloacal exstrophy currently under management 11 have undergone procedures for the establishment of urinary continence. There were 8 genetic female subjects, 6 genetic male subjects raised as girls and 1 genetic male subject raised as a boy. The genetic female subjects had all undergone urethral reconstruction. In 4 patients urethral reconstruction was done with local tissues and they have subsequently undergone bladder neck reconstruction: 1 is continent and voids through the urethra, 2 are dry on intermittent catheterization and 1 is dry on intermittent catheterization after augmentation with a hindgut patch. One girl underwent a Kropp procedure along with bladder augmentation, and she is dry on intermittent catheterization, in 1 urethral reconstruction was done with an ileal nipple through which she performs intermittent catheterization and 2 await a continence procedure.The urethral reconstructions in the genetic male subjects were more difficult. Of the 6 genetic male subjects raised as girls 5 have undergone continence procedures and 1 awaits establishment of urinary continence. In only 1 patient was urethral reconstruction possible from local tissues to allow intermittent catheterization. In the other 4 the perineal urethra was closed in favor of an abdominal stoma for intermittent catheterization. Ileal plication with nipple formation of the stoma was done in 3 patients and a Benchekroun stoma was used in 1. The genetic male subject raised as a boy underwent exstrophy reclosure plus epispadias repair and subsequent bladder neck reconstruction and augmentation. Augmentation was performed in 5 patients with hindgut segments in 3 and ileum in 2. All 5 patients are currently dry on intermittent catheterization, although 1 required revision of the ileal nipple to a Benchekroun stoma.Staged reconstruction can produce acceptable urinary continence in this complex anomaly. An innovative approach is required to find the most suitable solution for each patient anatomy, bladder size and function, and mental, neurological and orthopedic status.

Management of the Failed Exstrophy Closure

In the last 5 years we have seen 15 boys and 9 girls with failure of exstrophy closure, including 20 who had classical bladder exstrophy and 4 who had cloacal exstrophy. At the time of presentation 18 patients had undergone 1 closure while 6 patients had undergone 2 prior exstrophy closures. Closure was performed for complete bladder dehiscence in 16 cases and for significant bladder prolapse in 8. None of the patients underwent any form of osteotomy at the time of initial closure and in 19 closure was done within 72 hours of birth. Two patients underwent posterior iliac osteotomy elsewhere at the time of secondary closure. All patients referred to our institution underwent either posterior iliac osteotomy (8 patients) or anterior innominate osteotomy (16 patients). Epispadias repair along with reclosure of bladder exstrophy was done in 13 boys. The upper tract remained normal in all patients. There were no instances of failure in this group of reclosures. Eight patients have undergone subsequent bladder neck reconstruction of whom 7 are dry for 4 hours and 1 remains totally incontinent. Two patients have undergone simultaneous bladder neck reconstruction and augmentation cystoplasty, and they are dry on intermittent selfcatheterization. The failed exstrophy closure presents a formidable dilemma. However, a secure reclosure can be achieved with careful surgical technique, a well performed osteotomy (even if previously performed) and concomitant epispadias repair for male subjects.

Bladder Exstrophy: Increase in Capacity Following Epispadias Repair

The achievement of satisfactory continence in the management of classical bladder exstrophy remains a surgical challenge. This may be particularly difficult in the patient with a small bladder capacity after initial closure. The 12-year experience at our institution with bladder exstrophy is reviewed and attention is focused on the approach to those patients with a small bladder capacity. A total of 155 children with bladder exstrophy were treated. Of these 155 patients 28 boys were believed to have a bladder capacity inadequate for satisfactory bladder neck reconstruction (less than 60cc). All patients have undergone staged reconstruction with a urethroplasty for epispadias and they are available for evaluation. After a median interval of 22 months bladder capacity increased by a median 54.5cc. No child had hydronephrosis after the epispadias repair or bladder neck reconstruction. Of the 28 patients 25 have undergone bladder neck reconstruction with a median followup of 4.5 years and 88 per cent (22 of 25) demonstrate continence with a dry interval greater than 3 hours. Another 2 patients have been rendered continent following bladder augmentation. These results demonstrate that satisfactory continence is an achievable goal in the exstrophy patient with a small bladder capacity when bladder neck reconstruction is preceded by epispadias repair.

Augmentation Cystoplasty in the Failed Exstrophy Reconstruction

Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done.Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures.

Functional repair of bladder extrophy and total epispadias: F.X. Zhang, et al. Chin J Pediatr Surg 7:29–30, (February), 1986

Functional repair of bladder extrophy and total epispadias: F.X. Zhang, et al. Chin J Pediatr Surg 7:29–30, (February), 1986

Experience with Free Grafts in Urethral Reconstruction

A total of 33 patients underwent hypospadias or epispadias repair by patch or tubular grafts of skin or bladder epithelium from July 1980 to January 1985. Indications included previous circumcision, proximal primary hypospadias, severe chordee alone, inadequate local skin during extended urethroplasty at 2-stage hypospadias repair, multiple previous reconstructions and failed epispadias repair. Genital skin grafts were used in 25 patients, extragenital skin grafts in 4 and bladder epithelial grafts in 4.Complications occurred in 13 patients (39.4 per cent): 8 required reoperation for a fistula, stricture or diverticulum, and 5 underwent internal urethrotomy or urethral dilation. Of the 8 patients 3 needed more than 1 revision. These revisions were short and generally uncomplicated. Final results uniformly were good functionally and cosmetically.Based on our experience and that of others we believe that despite a high incidence of minor complications a free graft serves as an excellent substitute urethra for treatment of a variety of urethral problems in children.

Pediatric Urethral Reconstruction Without Proximal Diversion

A new method of transurethral drainage was used in 36 consecutive patients who underwent urethral reconstruction for repair of hypospadias and epispadias. The surgical procedures included 16 island flap urethroplasties, 15 distal urethral repairs with Mathieu’s technique, 3 epispadias repairs and 2 urethroplasties with a modified Thiersch-Duplay technique. To accomplish urinary drainage in all cases a 7 or 10F silicone tube with multiple perforations was inserted proximal to the bladder neck and through the repair, and cut 2mm. distal to the urethral meatus. The tube was secured to the meatus with loosely tied 4-zero polypropylene sutures and allowed to drain into a diaper.Satisfactory results were obtained in all patients. Complications included a small urethrocutaneous fistula in 6 children, a distal urethral stricture in 1 and severe bladder spasms in 2. The incidence of fistula formation did not appear to increase with this method of urinary drainage. Advantages of the method included a decreased hospital stay and minimal discomfort.

The Anatomical Course of the Neurovascular Bundles in Epispadias

References on the anatomical course of the neurovascular bundles of the penis in epispadias are rare. We studied the anatomy of the neurovascular bundles in 5 patients undergoing primary epispadias repair and 13 adolescents undergoing correction of erectile deformities. In all primary cases the neurovascular bundles were truly lateral as they ran along the distal and middle portions of the corporeal bodies, and became anterolateral only proximally. The bundles were identified in only 5 of 13 secondary cases and were in the same position as in the primary cases. Knowledge of this anatomy is important to protect these structures from surgical injury in cases of epispadias and exstrophy.

A supero-medial thigh flap for urethral reconstruction in epispadias and hypospadias

A reliable axial flap from the adductor region has been used for many years to repair a series of urethral defects formerly regarded as difficult problems. In particular the flap has proved invaluable in the treatment of epispadias “cripples”. Suggestions are also made for correcting the reverse chordee deformity due to the shortage of skin on the dorsum which, if untreated, spoils the functional result. The use of a supero-medial thigh flap was reported by Hirshowitz et al. (1980, 1982) for repair of the scrotum and vulva. The same flap had been discovered independently in 1978 in this department and used for the repair of epispadias and hypospadias “cripples”. Its reliability was soon recognised and the presence of axial vessels from the medial femoral circumflex artery and vein were demonstrated by transillumination at the time of surgery and by study in the post-mortem room. Some patients with epispadias are referred at puberty with an unrepaired scarred penis and a reverse chordee deformity. The defect cannot be repaired with local tissue alone and the same difficulty is encountered with some hypospadias “cripples”. Occasionally untreated cases of perineal hypospadias are seen with a marked shortage of local tissue. In our experience scrotal flaps may fail to heal and orthodox groin flaps may have difficulty in reaching the target. Although the superomedial thigh flap will require at least two stages it is a reliable technique and it provides a generous amount of tissue with which to solve what may at first sight seem to be an insuperable problem. It is easy to underestimate the total tissue defect in these cases before correcting the chordee or reverse chordee deformity: for instance the repair of epispadias and ectopia vesicae in an adult may need flaps up to 20 cm in length (Fig. 1).

The use of the pedicled transverse island flap of the inner prepuce in hypospadias, epispadias and urethral stricture repair

The use of the pedicled transverse island flap in reconstructive repairs is described. Experience with 35 hypospadias repairs shows that excellent cosmetic and functional results may be obtained, and 5 epispadias cases were corrected in this way with good results. The use of this technique is advocated in cases of severe urethral stricture and is demonstrated on 3 patients. The different techniques are described with emphasis on the critical surgical points and on the importance of the wound dressing.

The diagnosis of unilateral testicular obstruction in subfertile males.

Thirty-two subfertile males with sperms in the ejaculate and unilateral testicular obstruction are reported: the diagnosis was established by exploration of scrotum in 26, clinically in 2, 3 had had previous partially successful epididymovasostomies, and 1 had had an epispadias repair. The past medical history gave relevant information in 27 (84%), and useful findings were made on clinical examination in a further 3 cases. Fifteen patients had sperm counts over 20 million per ml, and 15 were less than 10 million per ml. Twenty-six (81%) had serum antisperm antibodies detected by tray agglutination test (TAT), 21 (81%) of whom had evidence of head-to-head (HH) agglutinins in pure or mixed form. Comparison with 162 vasectomised males and 160 naturally infertile males with antisperm antibodies showed that 55% of the former and 24% of the latter had HH agglutinins on TAT, differences that were highly significant on statistical analysis. Evidence of obstruction was found in 14 (37%) of 38 naturally infertile males with antisperm antibodies and HH or mixed agglutination, but only in 12 (10%) of 122 with TT agglutinins: this difference was also highly significant. Clinical history, physical examination and serum antisperm antibodies, especially if HH agglutinins are present, can suggest the possibility of unilateral testicular obstruction, but confirmation of the diagnosis requires exploration of scrotum.

Penile lengthening after previous repair of epispadias

Penile lengthening after previous repair of epispadias

Penile lengthening after previous repair of epispadias: W. H. Hendren. J Urol 121:527–534, (April), 1979

Penile lengthening after previous repair of epispadias: W. H. Hendren. J Urol 121:527–534, (April), 1979

Stone Formation Following Repair of Epispadias with Subcutaneous Pedical Flap From Scrotum: A Case Report

SummaryA case of Epispadias type III was operated in July, 1963 when chordee was released and the urethral mucosal defect produced in the post-urethra was covered by a 1.3×1.3 cm subcutaneous scrotal flap brought dorsally through a gap created between the crurae. Urethral reconstruction was done from the local tissues. After eight years patient reported with a giant stone in the urethra at the site of the scrotal flap.

Male epispadias: surgical repairs of urethropenile deformity.

A surgical technique for the repair of complete epispadias, with emphasis on correction of the urethropenile deformity and attempt to restore normal anatomic relations has been presented. The epispadiac orifice is dissected and followed proximally to beyond the bladder neck, then brought out perineally between the bifurcated crura. The penis is denuded from its skin, then embedded in the scrotum, except for the glans penis which protrudes from the midscrotum. Penile skin is folded into a tube to be used later for urethral reconstruction. Excellent cosmetic and functional results are consistently obtained by this multi-stage technique.

Urologic Surgery in Infancy and Childhood.

Children are not simply little adults, as the author points out in his introduction. Certain complex urosurgical procedures are mainly applicable to and suitable for the young child because of physiologic and morphologic differences and potential for progressive maturation. An atlas entirely directed to genitourinary surgery of children has long been needed. Like many monographs, the current text approaches this need, but falls short of the goal. The author obviously has enjoyed a rich experience in pediatric urology; the strengths of this work include clear, systematically described procedures, and excellent art work. Fine points of technique, and potential pitfalls, are discussed with adequate explanation to add justification and understanding. Some subjects are not covered; other subjects might have been covered more extensively. The section of hypospadias omits a number of techniques popular in the United States. The section on epispadias repair makes no reference to any of several methods of