Advances in pediatric urology which have affected the management of bladder exstrophy.
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Bladder Exstrophy↗
Biomedical subjects
R D Jeffs
Publications and source records attributed to R D Jeffs.
Prevalence and repair of inguinal hernias in children with bladder exstrophy.
PURPOSE: We delineated the prevalence, recurrence rates and optimal treatment of inguinal hernia in the exstrophy population. MATERIALS AND METHODS: Of 181 children with exstrophy followed at our hospital inguinal hernias developed in 121 (66.8%). RESULTS: In a 12-year period inguinal hernias developed in 81.8% of the boys and 10.5% of the girls. In 18.2% of the cases the hernia was repaired via a preperitoneal approach at the same time as exstrophy closure. The remaining patients underwent an inguinal operation. Most patients had a wide defect at the internal ring in addition to a patent processus vaginalis. The overall recurrence rate was 8.3%. The incidence of synchronous or asynchronous bilaterality was 81.8%. CONCLUSIONS: Children with bladder exstrophy should be carefully examined for inguinal hernias before bladder closure. If a unilateral hernia is present, the contralateral side should be explored. Careful preperitoneal repair should emphasize repair of the internal ring.
Adolescent↗
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.
Bladder Exstrophy↗
Techniques to create continence in the failed bladder exstrophy closure patient.
We reviewed retrospectively 315 patients with bladder exstrophy treated at our hospital between July 1976 and April 1992 to assess the outcome of those who failed primary closure of the bladder. Of the patients 47 required reclosure of the bladder, including 28 who have undergone a procedure to restore urinary continence. Methods used to achieve dryness included bladder neck reconstruction in 18 patients, bladder neck reconstruction along with augmentation in 4, augmentation alone in 4, repeat bladder neck reconstruction in 1, and reclosure with creation of a continent stoma and augmentation in 1. Nine of 18 patients who underwent primary bladder neck reconstruction are dry on intermittent catheterization, while 8 of the remaining 9 are dry and voiding without catheterization. Four patients who underwent primary bladder neck reconstruction and augmentation, and 4 who underwent augmentation after bladder neck reconstruction are dry on intermittent catheterization. The patient who underwent reclosure, bladder augmentation and creation of a continent abdominal stoma is dry on intermittent catheterization. Virtually all patients who failed the initial closure and later bladder neck reconstruction for continence require augmentation and intermittent catheterization to remain dry. Of 28 patients who underwent salvage procedures only 1 had upper tract changes. With attention to detail and the use of a variety of reconstructive techniques children who have failed exstrophy closure can achieve continence and have stable renal function.
Bladder Exstrophy↗
Dermal grafts for correction of severe chordee associated with hypospadias.
We report on 24 patients with hypospadias and severe chordee that could not be straightened with conventional techniques. We used small dermal grafts to augment the ventral tunica albuginea, which resulted in a completely straight, normal appearing erection in all patients. Subsequent urethroplasty was not complicated by the presence of the dermal graft. This procedure is technically straightforward and consistently results in a completely straight penis. Although required only rarely in hypospadias surgery, we recommend the dermal graft technique of tunica albuginea augmentation for cases of refractory chordee.
Adolescent↗
Complications of paraexstrophy skin flaps in the reconstruction of classical bladder exstrophy.
We reviewed the cases of the exstrophy/epispadias complex treated at our institution between July 1976 and April 1992. A total of 78 patients was identified who had paraexstrophy skin flaps used in the bladder closure, of whom 31 (40%) had a complication as a result of the flaps. The main complication encountered was a urethral stricture where the paraexstrophy skin flaps joined the urethral plate area. Multiple maneuvers were undertaken to correct these problems, including direct vision internal urethrotomy (12 cases), multiple urethral dilations (4), open revision (3) and full thickness skin grafts (5). Seven patients had such a complex stricture situation that they required either continent urinary diversion (5), colon conduit diversion (1) or cutaneous ureterostomy (1), the latter 2 patients having undergone vesicostomy elsewhere before referral. Of the remaining 24 patients who did not undergo a diversionary procedure 12 have undergone an epispadias repair and bladder neck reconstruction, 7 underwent an epispadias repair and 5 await further treatment. Freedom from complications in the initial closure of exstrophy significantly improves the chances of successful reconstruction. The avoidance of problems leading to obstruction, infection, hydronephrosis and reflux nephropathy will provide better kidneys regardless of bladder suitability for function or augmentation. Our use of paraexstrophy flaps has decreased but when they are required, special care in design, placement and followup is advised to avoid complicating strictures and their sequelae.
Bladder Exstrophy↗
Prostate size and configuration in adults with bladder exstrophy.
A total of 13 men born with classical bladder exstrophy underwent magnetic resonance imaging examination of the pelvis to evaluate the size and configuration of the prostate and pelvic organs. Mean patient age was 25.2 years (range 19 to 38). Of the patients 4 are voiding per urethram and 9 have undergone urinary division. Measurements included prostate volume, weight and maximum axial cross sectional area; pubic diastasis, and seminal vesicle size. The mean prostatic cross sectional area was 10.1 +/- 3.4 cm.2. The mean estimated prostatic volume and weight were 20.7 +/- 8.2 cc and 21.7 +/- 8.6 gm., respectively. The mean seminal vesicle length and width were 2.1 +/- 0.99 and 1.1 +/- 0.38 cm., respectively. The volume, weight and maximum cross sectional area of the prostate appear normal compared to published norms. In none of the patients did the prostate extend circumferentially around the urethra and the urethra was anterior to the prostate in all patients. Also, the puborectalis muscle group was widely separated and only provided lateral support of the prostate in patients who were continent and who had undergone prior posterior iliac osteotomy. Thus, the attainment of continence in this complex group of patients is multifactorial and prostate growth as evaluated by magnetic resonance imaging may not influence continence in these patients.
Adult↗
Complete genitourinary reconstruction in female epispadias.
While female epispadias is a rare congenital anomaly, the treatment of complete epispadias in the female patient does not significantly differ from that of their male counterparts, although the female defect can be overlooked as a cause of incontinence. Attention must be given to the creation of an adequate urethral channel so that an adequate bladder capacity can be achieved and eventual bladder neck plasty can be performed. Finally, attention must be given to the reconstruction of the external genital defect. During the last 7 years 11 female patients with complete epispadias were treated. Of these patients 4 were referred from elsewhere and 3 had failed a previous procedure (2 had recently undergone external genital and urethral reconstruction, 1 underwent urinary diversion after multiple failed bladder neck procedures). Nine patients underwent bladder neck plasty: 5 are completely continent day and night, 3 are dry for greater than 3 hours during the day, and 1 is dry for only 1 to 3 hours during the day and wet at night, for an overall continence rate of 87.5%. Our experience with these patients has taught us that the bladder in this condition is much like that found in complete male epispadias. Therefore, creating a urethral outlet with sufficient length and resistance along with simultaneous reconstruction of the external genitalia allows for bladder regrowth, thus, facilitating achievement of greater volumes and bladder neck reconstruction with an excellent chance of success.
Adolescent↗
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.
Bladder Exstrophy↗
Recombinant human tumor necrosis factor enhances radiosensitivity and improves animal survival in murine neuroblastoma.
An analysis of the potential of recombinant human tumor necrosis factor to enhance the radiosensitivity of C1300 murine neuroblastoma was undertaken. Female A/J mice bearing C1300 murine neuroblastoma (right hindlimb) underwent the following treatments: group 1-0.25 cc normal saline intraperitoneally times 2, group 2-0.5 mcg./gm. recombinant human tumor necrosis factor intraperitoneally times 2, group 3-400 cGy. right hindlimb, group 4-800 cGy. right hindlimb, group 5-400 cGy. right hindlimb plus 0.5 mcg./gm. recombinant human tumor necrosis factor intraperitoneally times 2 and group 6-800 cGy. right hindlimb plus 0.5 mcg./gm. recombinant human tumor necrosis factor intraperitoneally times 2. All animals were followed for 21 days after treatment initiation with interval measurements of tumor volume. All single modality treatments were more effective than normal saline in reducing average tumor volumes during the study period (800 cGy. equals recombinant human tumor necrosis factor greater than 400 cGy. greater than normal saline). The addition of recombinant human tumor necrosis factor to radiotherapy moderately augmented antitumor response at radiation doses of 400 cGy. but marked enhancement was attained at radiation doses of 800 cGy. (p less than 0.02). This enhancement was achieved without increased animal morbidity or mortality. Animals receiving recombinant human tumor necrosis factor in addition to 800 cGy. demonstrated increased survival when compared with animals receiving 800 cGy. alone (p less than 0.02). Although statistical synergy was not proved, recombinant human tumor necrosis factor appears to augment significantly radiation-induced tumor regression at no toxic cost to the animal.
Animals↗
The Cantwell-Ransley technique for repair of epispadias.
A total of 26 boys with bladder exstrophy (20) and epispadias (6) underwent initial urethral reconstruction between 1988 and 1991 using the 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. All exstrophy patients had adequate phallic length, with 13 having an intact urethral plate and 13 having had prior paraexstrophy skin flap interposition. 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 and all but 1 (previously diverted) are voiding per urethram. The neourethra in such patients allows for easier access for endoscopy and the ventral position aids in maintaining correction of the dorsal chordee. The low complication rate of this procedure coupled with the better anatomical configuration of the neourethra makes it useful for urethral and penile reconstruction in the exstrophy and epispadias patient.
Adolescent↗
Late massive hematuria as a complication of conservative management of blunt renal trauma in children.
The conservative management of blunt renal trauma in children is well accepted and well documented in the medical literature. The majority of children who sustain blunt renal trauma do well with such an approach. However, the complications of conservative management are also well documented. We present 2 patients with blunt renal trauma who were treated conservatively and suffered massive life threatening hemorrhage several weeks after the initial injury. Angiography was not performed until late and in both cases it identified the cause of bleeding. Percutaneous transcatheter embolization provided immediate definitive treatment in both patients.
Adolescent↗
Bladder exstrophy-epispadias complex: prostatic evaluation by transrectal ultrasonography.
Seven patients with bladder-exstrophy-epispadias complex underwent high resolution prostatic ultrasonography in order to establish the presence and appearance of their prostate gland and seminal vesicles. Six patients had been born with classic bladder exstrophy, and one patient with complete epispadias. The size of the prostate gland was in the normal range in three patients. Three patients had small glands, and in one patient no prostatic tissue could be identified. Two patients showed an unusual position or echoappearance of their glands. The seminal vesicles in five patients were relatively large, contained multiple cystic spaces, and/or extended posteriorly and inferiorly to the prostate gland proper. Our study demonstrates that the prostate gland and seminal vesicles are present in patients with bladder exstrophy. The unusual appearance, position, and size in most patients, however, suggests faulty embryologic development or changes secondary to surgery reflecting the complex nature of the condition. Likewise, the enlarged seminal vesicles may indicate impaired drainage of these structures secondary to the initial bladder closure or subsequent bladder neck reconstruction.
Adolescent↗
Chemoimmunotherapy in conjunction with surgery: strategies for management of murine neuroblastoma.
The combination of biological response modifiers with cytotoxic drugs has proven to be synergistic in several tumor systems. Recombinant human tumor necrosis factor (rhTNF) has been shown to enhance the antitumor efficacy of etoposide (VP-16) in the treatment of C1300 murine neuroblastoma. However, after completion of therapy, tumor growth resumes and results in subsequent death. In an effort to assess the impact of combining surgery with rhTNF/VP-16 therapy, A/J mice bearing the C1300 murine neuroblastoma were treated within adjuvant or neoadjuvant protocols. Adjuvant-treated animals had a longer interval to disease recurrence (P = .01) and smaller average recurrent tumor volumes postexcision (P less than .05) compared with surgical controls. Histological evidence of tumor recurrence and liver metastases was seen in both adjuvant-treated and surgical control animals. Neoadjuvant-treated animals had a longer interval to disease recurrence (P = .03) and smaller average recurrent tumor volumes up to 14 days postexcision (P less than .02) compared with surgical controls. In addition, 30% of the neoadjuvant-treated animals had no microscopic evidence of disease recurrence, and only 14% had histological evidence of liver metastases. The surgical controls in the neoadjuvant experiment all had histological evidence of disease recurrence and liver metastases. Thus, the combination of surgery and rhTNF/VP-16 in the adjuvant or neoadjuvant setting appears to significantly delay the progression of C1300 murine neuroblastoma. Furthermore, administering chemoimmunotherapy prior to surgical excision in a neoadjuvant manner appears to be most beneficial as regards prevention of local disease recurrence and distant metastases.
Animals↗
Pediatric testicular tumors: the Johns Hopkins experience.
Testicular neoplasms constitute 1 percent of all childhood malignancies and rank eighth in childhood cancer mortality. From 1970 to 1988, 25 testicular tumors in children eighteen years and under were seen. The majority of the patients were white (88%). Pathologic analysis of the tumors revealed that 68 percent were germinal and 32 percent were nongerminal. Staging was undertaken in all patients with serum markers, chest x-ray film, and computerized tomography scans or lymphangiography. All patients underwent radical orchiectomy, and further therapy was given dependent on tumor type and stage. The survival among this cohort was excellent, with only 3 patients succumbing to their disease. Detailed results of treatment, and approaches to avoid excess treatment morbidity are reviewed.
Adolescent↗
Anterior innominate osteotomies for failure or late closure of bladder exstrophy.
The bony pelvis was analyzed in 12 patients undergoing a further operation after initial bladder closure. Of the patients 5 had undergone a prior posterior osteotomy. All patients had wide diastasis of the pubis (average 5.5 cm.). In 9 of these patients late closure or reclosure of a failed initial bladder repair was done and 3 underwent a repeat bladder neck reconstruction. In all patients a new procedure, anterior iliac osteotomy with internal or external fixation, was performed. This procedure provides increased mobility of the pubis and increased correction. It avoids turning of the patient while under anesthesia for repeat preparation and in most cases postoperative traction is not needed. There were no instances of dehiscence, nonunion or infection. Three cases of transient femoral palsy were noted. All patients had a normal gait 4 months postoperatively. The mobility obtained after anterior osteotomy allows for excellent approximation. In view of evidence that approximation of the pubis improves closure and eventual continence results, we believe that osteotomies, even when repeated, are useful in revision surgery if there is bony diastasis.
Bladder Exstrophy↗
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 self-catheterization. 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↗
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.
Bladder Exstrophy↗