Paraurethral gland carcinoma in a man with bladder exstrophy diagnosed 41 years after bladder plate resection.
Explore the source record for details and available documents.
Adult↗
SEARCH · PubMed Health
Results for “Bladder Exstrophy”
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Complete repair of bladder exstrophy: management of resultant hypospadias.
PURPOSE: In our hands complete primary repair (CPR) of bladder exstrophy results in hypospadias in two-thirds of boys. To our knowledge hypospadias repair following CPR of bladder exstrophy has not been reported previously. We report our experience with hypospadias repair following CPR of bladder exstrophy. MATERIALS AND METHODS: A total of 22 boys with bladder exstrophy underwent CPR using Mitchell's technique between November 1998 and January 2002. The procedure resulted in hypospadias in 15 boys (68%). The site of the meatus was distal penile in 5 patients, mid penile in 3 and proximal penile in 7. Hypospadias repair was performed in all 15 boys. The 5 patients with distal penile hypospadias underwent repair consisting of Thiersch-Duplay urethroplasty with dorsal incision in 3 and tubularized vertical island flap with glanular tunnel in 2. All patients with mid (3) or proximal (7) penile hypospadias underwent Mustarde repair with glanular tunnel. RESULTS: Followup ranged from 6 to 18 months (median 13). The Thiersch-Duplay procedure with dorsal incision resulted in fistula/stenosis in 2 of 3 boys. None of the remaining 12 boys with tubularized penile flap urethroplasty (tubularized vertical island flap or Mustarde) with glanular tunneling had development of fistula or stenosis. Complete degloving of the penis and penile skin redistribution were not necessary with the Mustarde technique. CONCLUSIONS: Thiersch-Duplay urethroplasty with dorsal incision does not seem to be a good option for hypospadias repair following CPR of bladder exstrophy. The use of penile skin as a tubularized flap with glanular tunneling seems to yield excellent functional/cosmetic results.
Bladder Exstrophy↗
[Urinary incontinence in epispadias and bladder exstrophy with special reference to free autologous muscle transplantation].
This is a report on 16 patients with epispadias of grades II to IV and on 35 patients with exstrophy of the bladder, grades III and IV. The following surgical treatment was carried out: in children with epispadias, simple closure of the urethra in one case, neck of the bladder plasty according to Young-Dees in two cases, the method after Thiersch-Duplay in three cases, whereas four patients were treated after Williams, five after Johnston and one patient after Ombrédanne in reverse. Seven additional operations were done mainly for prepuce correction and closure of fistula after urethroplasty. Three of the 16 patients were continent already before the operation. 4 children were partially continent. Two of these showed improved continence, thus achieving prolonged periods of dryness. 9 children remained incontinent. Hence, free muscle transplantation to the bladder was performed in four patients. A total of 45 operations were performed on children with exstrophy of the bladder. Of these, 12 concerned primary closure of bladder; in 13 cases, an ileum bladder was constructed, whereas cystosigmoideostomy was done in 5 times, uretersigmoideostomy 5 times, cutaneous ureterostomy once, rectum bladder once, and a colon conduit once. The postoperative continence performance of the 12 patients with primary bladder closure was unsatisfactory. Three children only attained periods of dryness up to two hours; 5 remained completely incontinent, whereas in 4 it was subsequently necessary to construct a urinary passage into the intestine by means of an ileum bladder in 2 cases, a colon conduit in one case, and ureterosigmoideostomy in one case. Free muscle transplantation to the bladder was performed in two patients. Free muscle transplants were also performed on 2 children, in one case after traumatic tearoff of the urethra and in one case after iatrogenic lesion of the musculus sphincter vesicae externus in adrenogenital syndrome (AGS) according to Prader III and IV. This means that a total of eight free muscle transplants were performed. All children were primarily incontinent. Postoperatively, complete continence could be achieved in four cases, once an improvement with dry periods up to 2 hours. The two patients with bladder exstrophy remain incontinent, in one patient with epispadias the postoperative interval has been too short so far to arrive at a final judgement. It follows from the examinations that free muscle transplantation is a good method for treating urinary incontinence, the suspension effect playing an important part. However, in patients with bladder exstrophy the indication should be considered with caution.
Adolescent↗
Complete repair of bladder exstrophy in the newborn: complications and management.
PURPOSE: Complete repair of bladder exstrophy in the newborn includes bladder, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While reports have shown the need for early reimplantation of the ureters in 50% of patients and repair of hypospadias in those in whom the urethral plate does not reach the tip of the glans, to my knowledge none has described immediate postoperative complications in these patients. MATERIALS AND METHODS: Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehiscence with loss of the proximal urethra and a significant amount of penile skin occurred in 2 male patients. Bladder prolapse with loss of the urethra well onto the penis occurred in 2 newborns, of whom 1 had undergone 2 closures before referral and 1 had partial loss of the glans on 1 side. One patient had only minor bladder prolapse but lost most of the urethra and penile skin. The final male patient lost most of the urethra and had separation of the pubis but the bladder maintained a subcutaneous position and did not prolapse. The female patient had major prolapse of the bladder and loss of the urethrovaginal septum. RESULTS: The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. The patient with major prolapse and 2 prior closures was treated with reclosure, bladder augmentation and continent stoma reconstruction, and awaits epispadias repair. In the male patient with marked prolapse and partial loss of the glans has undergone reclosure with bilateral innominate and vertical iliac osteotomy was done and he awaits epispadias repair. The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. The female patient was treated with reclosure with combined bilateral osteotomy and mobilization of an anterior bladder tube to use as a urethra with no attempt to establish continence. CONCLUSIONS: Complete repair of bladder exstrophy in the newborn period requires experience with the exstrophy condition, appropriate pelvic and patient immobilization, and excellent postoperative care. Complications with this repair are similar to those with modern versions of the staged repair, and its failures are similar. However, penile skin loss with dehiscence or prolapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimulation are valuable adjuncts. Other than the patient who underwent augmentation and continent stoma construction, none has undergone any type of continence procedure, all are awaiting an increase in bladder capacity and followup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy surgeon. While earlier reports of this procedure seem encouraging, complications are real and difficult to manage, even in experienced hands.
Bladder Exstrophy↗
Reconstruction of the external genitalia in females with bladder exstrophy.
OBJECTIVES: A very characteristic genital anomaly accompanies bladder exstrophy in females. This includes anterior displacement and narrowing of the vagina and separation of the clitoris into two distinct bodies. The authors have had a relatively large experience with correction of this rare genital anomaly. From this experience, it began to appear that certain procedural details were associated with improved outcomes. The objective of this article was to review this experience and detail the reconstruction of the external genitalia in these patients. METHODS: All cases of bladder exstrophy in females presenting from January 1, 1970 to December 31, 1992 were reviewed. A total of eight patients who underwent surgical correction of the external genitalia were included. Data was obtained regarding urologic treatment, associated anomalies, corrective gynecologic procedures, complications and subsequent course. RESULTS: Corrective gynecologic surgery included enlargement of the vaginal orifice in all eight patients. Five patients had reapproximation of the bifid clitoris. Three patients performed postoperative dilatation therapy. Postoperatively, one patient experienced dyspareunia requiring a repeat procedure to enlarge the vaginal orifice. No patient had complications related to pelvic organ prolapse. CONCLUSIONS: Care should be taken not to extend the perineal incision too far posteriorly in reconstruction of the external genitalia in females with bladder exstrophy. Failure to do so may further predispose the patient to uterine prolapse. Post-operative dilator therapy may be an important adjunctive technique.
Adolescent↗
Covered exstrophy: a rare variant of classical bladder exstrophy.
Covered exstrophy is an extremely rare variant of exstrophy-epispadias complex. It is less distressing and easier to manage than classical exstrophy of the bladder. We report three cases of this entity, two associated with anorectal malformation and one with unilateral renal agenesis, along with a review of the literature.
Abnormalities, Multiple↗
Bladder exstrophy associated with complete urethral duplication: a rare malformation with excellent prognosis.
PURPOSE: We report the association of complete urethral duplication and bladder exstrophy in 5 males, and describe the main characteristics to achieve diagnosis. MATERIALS AND METHODS: From 1983 to 2000, 5 males with single bladder exstrophy and complete urethral duplication were seen at 3 institutions. Although bladder exstrophy was obvious at birth, only 1 patient had the preoperative diagnosis of associated urethral duplication. All patients were evaluated with abdominal ultrasound, voiding cystourethrogram after bladder closure and cystoscopy. RESULTS: All patients were totally incontinent, including 4 after primary bladder exstrophy closure. Although it was initially unnoticed by the urologist, most patients had leakage from the tip of the penis during the Valsalva maneuver. In all cases bladder exstrophy was single and deeply situated in the pelvis with excellent elasticity. All patients had a normal sized penis with less severe dorsal chordee and conical shaped glans. The duplicate epispadiac urethra did not have a verumontanum. Only 1 of the 5 patients had the correct diagnosis made before primary closure of bladder exstrophy, while in the other 4 diagnosis of a duplicate ventral urethra was made at the time of epispadias or fistula repair. There were no other associated malformations. In all cases the dorsal epispadiac urethral plate was excised and bladder emptying occurred through the ventral urethra, which at cystoscopy had a normal verumontanum. Postoperatively, all patients became continent and voided with good flow through the ventral urethra and, as opposed to many cases of exstrophy and epispadias, had normal size and excellent cosmesis of the penis. CONCLUSIONS: Complete urethral duplication is a rare variant of the exstrophy-epispadias complex. Patients with this anomaly present with a larger and more deeply situated bladder plate than classical exstrophy and a larger penis. A high index of suspicion is necessary to make a preoperative diagnosis. Excision of the dorsal urethral plate and maintenance of the ventral urethra are the treatment of choice, resulting in a continence and normal voiding.
Adult↗
Long-term results of ureterosigmoidostomy in children with bladder exstrophy.
The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution of the question of disposal of the diverted urine.
Adolescent↗
Primary repair of bladder exstrophy followed by clean intermittent catheterization: outcome of 15 years' experience.
OBJECTIVES: To determine the continence and spontaneous voiding rate after neonatal reconstruction of bladder exstrophy without formal bladder neck reconstruction in patients undergoing primary reconstruction and treated with clean intermittent catheterization (CIC) after closure. METHODS: From 1987 to 2003, 15 consecutive patients (8 boys and 7 girls) with bladder exstrophy underwent neonatal reconstruction. Reconstruction focused on bringing the bladder neck and proximal urethra intra-abdominally and meticulously closing the pelvic floor muscles around the urethra. Three weeks postoperatively, CIC was started until toilet-training age. Bladder capacity, continence status, renal anatomy and function, and additional urologic surgical procedures during follow-up were analyzed. RESULTS: Nine patients (60%) became socially continent after primary closure without any additional bladder neck surgery. Twelve patients (80%) were continent when those who underwent endoscopic bulking injection were included. One patient became socially continent after bladder neck reconstruction, and one was dry and used CIC after bladder neck reconstruction and ileocystoplasty. One patient remained incontinent because of the parents' refusal of surgery. Ultimately, of 15 patients, 14 were dry (93%) of whom 10 were completely continent, 3 were partially continent (dry intervals of 1 to 3 hours), and 1 was dry by catheterizable stoma. The bladder capacity was adequate for age in 80% of patients. Febrile urinary tract infection occurred in 33% of patients, and 67% needed endoscopy for urethral stenosis. Upper tract dilation and loss of renal function was not seen. CONCLUSIONS: The results of our study have shown that primary repair of bladder exstrophy followed by CIC has encouraging continence and bladder capacity rates, with preservation of the upper urinary tract and limited need for additional bladder neck surgery.
Bladder Exstrophy↗
Bladder exstrophy: penile lengthening procedure.
A review of bladder exstrophy experience at our institution was undertaken with reference to penile adequacy and intersymphyseal distance. Drift of the symphysis apart even with iliac osteotomy was noted to occur within two years of initial closure but remained stable after three years. This drift can be prevented if the sacral tuberous and sacral spinous ligaments are sectioned. Further, the adequacy of the phallus seems to depend directly on the intersymphyseal distance. Thus, we suggest a variation in the staged approach to bladder exstrophy repair with these facts in mind.
Age Factors↗
Pregnancy in women with bladder exstrophy.
Little is known about pregnancies in women with bladder exstrophy. Of 28 women with bladder exstrophy who were evaluated 7 became pregnant and 5 delivered successfully. Problems seen during pregnancy, the long-term effects and the need for early, informed counseling by the urologist are discussed.
Adolescent↗
Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy.
The surgical results of 28 consecutive initial bladder closures and 25 consecutive initial bladder neck reconstructions performed for classical bladder exstrophy at our hospital between 1975 and 1982 are presented. Partial bladder prolapse occurred in 2 cases and complete wound dehiscence never occurred following the initial primary bladder closure. Urinary continence following bladder neck reconstruction was assessed from parental interviews. An excellent surgical result was defined either as achievement of a daytime dry interval for more than 3 hours or less than 1 incontinent episode per day. According to these parameters, an excellent surgical result was achieved in 86 and 80 per cent of children, respectively. In 21 children evaluated with excretory urograms between 1/2 and 6 years after bladder neck reconstruction 10 per cent of the renal units showed significant hydronephrosis and deterioration of function. The 2 patients who had upper tract deterioration were not followed postoperatively at our institution and the diagnosis of bladder outlet obstruction was delayed when excretory urograms were not obtained during the first postoperative year. This review of the surgical results following primary bladder closure and bladder neck reconstruction for classical bladder exstrophy demonstrates that secure abdominal wall closure and urinary continence can be achieved with minimal morbidity and with infrequent deterioration of renal function following staged functional bladder closure.
Bladder Exstrophy↗
Evaluation of smooth muscle and collagen subtypes in normal newborns and those with bladder exstrophy.
PURPOSE: Many patients who undergo bladder exstrophy closure as newborns, subsequent epispadias repair and later bladder neck reconstruction become completely continent yet complications can occur. After successful initial exstrophy closure and later epispadias repair some patients may fail to gain sufficient capacity for bladder neck reconstruction or satisfactory capacity and continence after bladder neck reconstruction. In an attempt to understand the pathogenesis of these failures we compared bladder biopsies from normal neonates and those with exstrophy. MATERIALS AND METHODS: Bladder biopsies obtained from the midline of the bladder wall just above the base of the trigone from 12 newborns with exstrophy were compared to bladder sections from 9 neonatal cadavers. All bladder specimens were stained with monoclonal antibodies against type I, III or IV collagen and a subset was further stained with Masson's trichrome to define the extracellular matrix. All specimens were then analyzed using a color digital image analysis system. RESULTS: At initial examination of the extracellular matrix there was an increase in the collagen-to-smooth muscle ratio from 0.38 in controls to 1.2 in newborns with exstrophy, comprising an increase in collagen and decrease in smooth muscle. The collagen component of the extracellular matrix was then further defined to quantitate the amount of each collagen type (I, III and IV) deposited. We then evaluated the ratio of collagen type-to-total collagen sampled. Compared to control bladders there was no statistical difference in the amount of type I or IV in the bladders of newborns with exstrophy at initial closure. However, there was a 3-fold increase in type III collagen (0.14 +/- 0.05 to 0.46 +/- 0.2%, p < 0.001) in the bladders of neonatal controls versus newborns with exstrophy. CONCLUSIONS: This alteration in collagen makeup may represent an earlier developmental stage of the exstrophy bladder at birth, which then remodels and changes after successful initial closure. Further studies are underway to examine the collagen composition of bladders at bladder neck reconstruction, failed closures and augmentation.
Biopsy↗
[An unusual form of bladder exstrophy].
A rare case of bladder exstrophy is presented. It is described as being similar to a superior vesical fissure, but it differs from it by the abnormal course of the urethra which is separated from the bladder and runs along the clitoris.
Bladder Exstrophy↗
Functional outcome and specific complications of gastrocystoplasty for failed bladder exstrophy closure.
PURPOSE: The encouraging initial results of gastrocystoplasty led us to perform it for failed bladder exstrophy closure. We assess the functional outcome of the augmented bladder and evaluate complications related directly to use of the stomach in this specific group of children. MATERIALS AND METHODS: We performed gastrocystoplasty in 22 children an average of 9.5 years old with a small, poorly compliant bladder after staged reconstruction of bladder exstrophy failed. Followup ranged from 6 months to 6 years (mean 3 years). RESULTS: Complete urinary continence was achieved in 14 children (64%). Voiding via the urethra was possible in 13 patients (60%) but post-voiding residual urine was significant in 12. Bladder capacity increased from a mean of 77 to 270 ml. Bladder capacity decreased during followup in 3 children, requiring repeat augmentation. Six children had isolated dysuria and 2 had dysuria with hematuria. Perforation of the gastric patch and a bleeding gastric ulcer occurred in 1 patient each. CONCLUSIONS: The disadvantages of gastrocystoplasty outnumber its advantages after failed bladder exstrophy closure. Urethral sensation makes dysuria a major discomfort. Safety is not optimal, since perforation may occur. Voiding is not efficient because gastrocystoplasty provides continence only when it is associated with intermittent catheterization. Bladder capacity is insufficiently augmented and inconsistent during followup. We believe that the use of gastrocystoplasty in cases of failed bladder exstrophy closure should be reconsidered.
Adolescent↗
Total duplicate exstrophic bladder and urethra: variation of incomplete bladder exstrophy.
A rare variant of incomplete bladder exstrophy with an intact internal bladder and urethra and a duplicate exstrophic bladder and urethra is reported.
Bladder Exstrophy↗
Use of tissue expanders in final reconstruction of infrapubic midline scar, mons pubis, and vulva after bladder exstrophy repair.
Two patients following bladder exstrophy repair presented for final cosmetic reconstruction with the characteristic lower abdominal midline scar, bisected mons pubis, and laterally displaced labia majora. Tissue expanders were used to obtain additional skin and subcutaneous tissue. After adequate serial expansion, the expanders were removed, scar tissue excised, and primary approximation of healthy tissues performed. A tension-free closure and esthetically pleasing midline incision, mons pubis, and vulva were obtained.
Adolescent↗
Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights.
PURPOSE: We present the pelvic floor anatomy of the major pelvic floor musculature in classic bladder exstrophy, including the levator ani, obturator internus and obturator externus. By improving our knowledge of pelvic floor anatomy we hope to understand better the relationship of the pelvic floor to the bony anatomy as well as the role of osteotomy in changing pelvic floor anatomy to enhance urinary control after surgery. MATERIALS AND METHODS: 3-Dimensional computerized tomography was done in 6 boys and 1 girl, including 5 patients 2 days to 5 months old (mean age 7 months) undergoing primary closure and 2 who were 4 and 8 years old undergoing repeat closure. The pelvic floor musculature, including the levator ani, obturator internus and obturator externus, in these cases was compared to that in 26 age and sex matched controls. RESULTS: The levator ani musculature encompasses a significantly wider area of 9.5 cm.2 in patients with classic bladder exstrophy than in controls. The anterior segment of the levator ani was shorter (1.2 cm.) and the posterior segment of the levator ani was longer (2.5 cm.) than in controls. The degree of divergence of the levator ani in classic exstrophy was significantly more outwardly rotated (38.8 degrees) than controls. In addition, the transverse diameter of the levator hiatus was 2-fold that in our control group and in that of published controls, while the length of the hiatus was 1.3-fold that in normal controls. There was also significant flattening, involving a 31.7 degree decrease in steepness between the right and left halves of the levator ani, of the puborectal sling in classic bladder exstrophy versus controls. Because of these findings, there is more anterior superior rotation in the pelvic floor in exstrophy cases. The obturator internus was more outwardly rotated (15.1 degrees) in exstrophy and the obturator externus also showed more outward rotation (16.9 degrees) than in controls. CONCLUSIONS: This study provides better understanding of the pelvic floor anatomy in classic bladder exstrophy. Significant differences have been documented in the pelvic floor in classic bladder exstrophy cases and controls. Hopefully these differences may have a pivotal role in providing new insight into long-term issues, such as urinary and fecal incontinence, and pelvic organ prolapse, in classic bladder exstrophy.
Bladder Exstrophy↗