Vesical Exstrophy Research Articles

Vesical Extrophy Diagnosed Late: About a Case

Vesical exstrophy is a malformation that mainly affects the urinary, genital and musculoskeletal system, whose prevalence is estimated at 2.07/100,000 births. It is usually managed in the first hours of life, but there are still patients who have never consulted for this malformation. We report a case of bladder exstrophy diagnosed late in a 15-year-old patient never treated. Our attitude was the description of the malformation at the anatomical, genital, histological, functional and therapeutic point of view. The patient to benefit from a continental enlargement ileocystoplasty with ostomy.

Bladder exstrophy: An atypical journey of a rare case of an adult

Bladder exstrophy is a rare and complex malformation. Often associated with diverse deformations, an accurate diagnosis is a must for adequate management. In the African setting, especially in a remote area, delivery rarely occurs within a healthcare structure, thus reducing the chance of an early diagnosis. Due to a low density of specialists, people in rural areas refer to traditional healers for healthcare problems, the thing that delays the time to diagnosis. We report, according to the CARE guidelines (https://www.equator‐network.org/reporting‐guidelines/care/), a case of an 18‐year‐old male patient who was transferred to us for better management of a reddish hypogastric mass leaking urine. Initially considered as a congenital wound, his parents applied traditional products to heal it. Following the poor outcomes, the parents will consult several general physicians; unfortunately, no one gave a clear diagnosis. The clinical examination on admission revealed a good general condition and noted the presence of a reddish, ovoid structure of about 9 centimeters of the minor axis and 11 centimeters of the major axis in the hypogastric region allowing urine to flow in its upper part. The external genitalia examination revealed a retracted and short penis with urine exit at its base through the reddish structure. The patient was transferred outside the country for better management due to a lack of dedicated equipment. Although vesical exstrophy is a rare disease, an early diagnosis allows adequate management and good outcome. Thus, a well‐performed neonatal examination is required to avoid later diagnosis and complications.

Ectopic scrotum with undescended testis

Ectopic scrotum is defined as abnormal position rather than normal position of the scrotum. Congenital scrotal anomalies are penoscrotal transposition, bifid scrotum, accessory scrotum, and ectopic scrotum. Associated anomalies with ectopic scrotum are inguinal hernia, ectopic ureter, vesical exstrophy, renal dysplasia, and agenesis. Ectopic scrotum is a rare congenital anomaly. We report a case of ectopic scrotum with undescended testis.

Diagnostic anténatal des anomalies de la paroi abdominale antérieure

Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects. These 22 AAWDs consisted in 13 cases of omphalocele (including four cases of Beckwith-Wiedemann syndrome), four of gastroschisis, one of pentalogy of Cantrell, three of vesical exstrophy and one of cloacal exstrophy. Prenatal ultrasonography provided the diagnosis of 14 of these defects with a changing sensitivity with the gestational age varying from 17% in the first trimester to 71.4% and 77.8% in the second and third trimesters, respectively. The relevance of this examination was improved when performed by an imaging specialist. The prenatal diagnosis of these defects indicated an amniocentesis in eight cases, allowing the diagnosis of two cases of trisomy 18. It also motivated a therapeutic termination of the pregnancy (TTP) in ten cases. Prenatal ultrasonography allowed better prenatal follow-up and planning of the delivery of the continued pregnancies. It indicated an emergency C-section in only one case by showing intestinal complications of gastroschisis. Four NNs died (two cases of omphalocele and two of gastroschisis), three of which postoperatively and the prenatal diagnosis did not improve survival. Prenatal ultrasonographic diagnosis provided a precise morphological study determining the type of the AAWD, a complete malformation assessment, and the prognosis factors. This resulted in adequate multidisciplinary pre and postnatal care, including a rigorous ultrasound follow-up, a TTP in case of associated defects, and emergency delivery once the complications of poor diagnosis are detected.

Multiple failed closure of bladder in children with vesical exstrophy: Safety and efficacy of temporary ileal patch augmentation in assisting bladder closure.

Objective:The surgical approach to small bladder template in exstrophy bladder is difficult. Previously, many of these children underwent ureterosigmoidostomy and in recent times, the trend is to do a delayed primary closure. We have used ileal patch as a temporary cover for these small bladders with a view to encourage bladder growth and early results are encouraging.Materials and Methods:In five of the 45 children with bladder exstrophy managed by radical soft-tissue mobilization over 10 years, primary bladder closure was not possible due to repeated failed closures. A detubularized ileum was used to patch the bladder initially and after 4 months the patch was excised and bladder closure with sphincter repair was done in second stage.Results:In five children (three girls and two boys) the mean age at initial bladder closure was 14 months and mean age at ileal patch was 22 months. In four patients, the bladder grew facilitating closure and in one patient it failed. There were no complications with the use of gut in patch.Conclusion:A temporary ileal patch seems promising in managing failed bladder closure in exstrophy patients. Long-term studies are needed before such a technique can be used in all patients with failed primary bladder closures.

Gollop-Wolfgang complex and cloacal exstrophy, a strange association

Cloacal exstrophy and Gollop-Wolfgang complex are very rare pathologies and their association has been reported in only one patient. We present a case of a newborn of indeterminate sex with anomalies of the lower limbs, and an anterior abdominal wall defect. External genitalia were not observed, ectrodactyly of lower limbs, omphalocele, lipomeningocele and imperforate anus were detected. During the diagnostic and therapeutic surgery other anomalies were found, such as vesical exstrophy, cecal fistula, uterine duplication, vaginal agenesis, urethral agenesis, ectopic ureters, stenosis of the left ureter, biphid clitoris and patent urachus. The abdominal ecography showed ectopic right lower quadrant localization of right kidney. Radiographic images of lower limbs showed bifurcation of left femur and absent tibia in both limbs. Due to the findings a diagnosis of cloacal exstrophy and Gollop- Wolfgang complex was made. The patient developed sepsis, liver failure, metabolic acidosis and hyponatremia, she died at seven weeks of age.

Reply

There is no doubt that ejaculation after bladder exstrophy-epispadias complex (EEC) reconstruction cannot be normal. Even after functional bladder neck reconstruction, the prostate is not surrounding the urethra circumferentially, 1 Gearhart J.P. Yang A. Leonard M.P. et al. Prostate size and configuration in adults with bladder exstrophy. J Urol. 1993; 149: 308-310 PubMed Google Scholar and the lack of a normal functioning bulbospongiosus muscle prohibits forceful ejaculation. 2 Woodhouse C.R.J. Prospects for fertility in patients born with genitourinary anomalies. J Urol. 2001; 165: 2354-2360 Abstract Full Text Full Text PDF PubMed Google Scholar Additionally, surgical injury and malposition of the verumontanum could contribute greatly to ejaculatory disorders in patients with EEC. 3 Hanna M.K. Williams D.I. Genital function in males with vesical exstrophy and epispadias. Br J Urol. 1972; 44: 169-174 Crossref PubMed Scopus (31) Google Scholar However, the effect and the role of the pelvic floor reconstruction on ejaculation in EEC is still unknown. To a certain extent, the reappearance of the pubic gap is constant, regardless of the pelvic ring reconstruction method used, in particular, after all variations of osteotomy 4 Satsuma S. Kobayashi D. Yoshiya S. et al. Comparison of posterior and anterior pelvic osteotomy for bladder exstrophy complex. J Pediatr Orthop B. 2006; 15: 141-146 Crossref PubMed Scopus (19) Google Scholar With regard to this insight, we are convinced that in most cases of classic bladder exstrophy, osteotomy is not necessary to achieve better results whether in antegrade ejaculation or continence.

Massive hematuria due to portal hypertension in a child with bladder augmentation for vesical exstrophy

We report a case of a 17-year-old patient with severe hematuria from an ileocystoplasty-augmented bladder for vesical exstrophy, with associated cirrhosis secondary to autoimmune hepatitis, diagnosed years later. Varix development and further bleeding from ectopic varices, related to cirrhosis and portal hypertension, are a rare cause of hematuria in patients with surgically augmented bladder volume. This child was successfully treated with a transjugular intrahepatic portosystemic shunt procedure as a bridge to hepatic transplantation.

COMPLETE PHALLOPLASTY USING THE FREE RADIAL FOREARM FLAP FOR CORRECTING MICROPENIS ASSOCIATED WITH VESICAL EXSTROPHY

Purpose: We present a new surgical technique for reconstructing the penis in a man with micropenis associated with vesical exstrophy.Materials and Methods: A free radial forearm flap was used to create a penis of normal length and diameter. The flap was wrapped around the native micropenis. A penile prosthesis was then inserted in the flap to provide erection.Results: The flap was well vascularized and no skin damage was observed 6 years after reconstruction. The patient achieved sexual intercourse on a regular basis. He is satisfied with the result.Conclusions: Free transfer of the radial forearm flap may be done in select men with micropenis associated with vesical exstrophy for penile reconstruction. An inflatable prosthesis may be inserted in the flap to provide erection. The results of this technique have remained stable in the long term. This method provides a new tool for phalloplasty in these difficult cases.

BLADDER TRANSPLANTATION IN RATS USING FK-506

We created viable bladder tissue by transplantation with immunosuppression. For bladder transplantation the bladder of newborn Brown-Norway rats was excised and each was transplanted into a pouch created in the distal omentum of a 5-week-old Lewis rat. In 15 group 1 rats no immunosuppressive agent was used. In 20 group 2 rats 0.6 mg./kg. FK-506 daily were given intramuscularly until a predetermined day of harvest. Recipient rats were sacrificed on day 3, 5, 7 or 14 after bladder transplantation, and the bladder grafts were harvested and formalin fixed. Hematoxylin and eosin staining was done to examine bladder graft survival and the degree of rejection, and immunohistochemical testing was performed for assessing the vesical nervous system. In 5 rats in the control group bladder augmentation was performed by anastomosing the bladder graft to the native bladder. Each augmented bladder was harvested 21 days later for histopathological assessment. Overall bladder graft survival was 96.4%. Each successfully transplanted bladder graft appeared macroscopically as a thin walled cyst. In group 1 all bladder grafts showed rejection with cellular infiltration. In group 2 there was mild rejection in 5 rats and no evidence of rejection in the remaining 15. All group 2 bladder grafts had intact nerve distribution. Bladder augmentation was successful in all 5 cases and the mucosa was normal throughout each augmented bladder. Because FK-506 successfully prevents rejection, our technique would appear to have the potential for creating viable bladder tissue that may be used for bladder augmentation in cases of vesical exstrophy or neurogenic bladder.

Vesical exstrophy: Repair using radical mobilisation of soft tissues: J.H. Kelly. Pediatr Surg Int 10:298–304, (July), 1995

Vesical exstrophy: Repair using radical mobilisation of soft tissues: J.H. Kelly. Pediatr Surg Int 10:298–304, (July), 1995

Duplication of appendix with segmental dilatation of the colon and vesical exstrophy

Duplication of appendix with segmental dilatation of the colon and vesical exstrophy

Hypertension following primary bladder closure for vesical exstrophy

Since the initiation of staged reconstruction for bladder exstrophy, hypertension has been a known complication of the procedure. The etiology of the elevation in blood pressure was presumably secondary to ureteral dysfunction caused by edema at the ureterovesical junction and secondary renal obstruction. This report details the clinical course of three patients who developed significant hypertension following primary bladder closure with posterior iliac osteotomies and bony immobilization by skin traction. None of the patients had urinary obstruction and all had their hypertension alleviated by removal of the traction. The presumed etiology of the elevated blood pressure within these patients is similar to that experimentally produce by inappropriately applied traction resulting in tension on the sciatic nerve and reflex induced vasoconstriction. The need to maintain appropriately applied traction and the necessity of monitoring the postoperative blood pressure is stressed.

Continent Urostomy with Hydraulic Ileal Valve in 136 Patients: 13 Years of Experience

The hydraulic ileal valve, which we developed in 1975, ensures continence by adapting to 5 different urinary reservoirs. The valve is made by isolating a 14cm. long intestinal loop with the mesentery. The isolated ileal segment then is folded inward on itself throughout its entire length. We performed 136 continent urostomies with this hydraulic valve. An ileocecal reservoir was used in 122 patients, ileum in 8, sigmoid in 1, rectum in 1 and bladder (continent cystostomy) in 4. Indications for continent urostomy were bladder tumor in 55 patients, complex vesicovaginal fistulas in 5, neurogenic bladder in 13, vesical exstrophy in 12 and miscellaneous reasons in 5.Of the patients 103 (75 per cent) were continent immediately. Continence was obtained after repair of the valve in 24 patients (17.6 per cent). Therefore, 127 patients over-all were continent. Mean followup of our patients was 38 months (range 3 to 154 months). Continence remained excellent with self-catheterization performed easily in 88.3 per cent of the patients. Over-all, all of our continent urostomies were well tolerated biologically and radiologically.

Tolerance to Bilateral Subcutaneous Access Nephrostomy in Dogs

Subcutaneous nephrostomy is a technique which allows access to the renal pelvis for physiological study in the experimental animal over a prolonged period of time without the complications attendant upon an externally draining appliance. One end of an 8 Fr. polyvinyl tube was placed in the renal pelvis at open surgery and the other end was capped and buried in the subcutaneous space for subsequent access by percutaneous needle puncture. This procedure is preferable to surgically created vesical exstrophy, exteriorization of the renal pelvis or conventional nephrostomy, techniques which have been proposed for such purposes. Tolerance to subcutaneous access nephrostomy was investigated in a group of 10 dogs followed for periods ranging from six months to two years. It was found that bilateral subcutaneous access nephrostomy did not alter kidney function but was associated with altered peristalsis. It was also associated frequently with bacteriuria, but not pyelonephritis. Neither calcific deposits nor calculi were seen as a consequence of the tube, and the procedure was well tolerated by the animal although, in some, progressive weight loss was observed. In general, this technique proved to be an excellent procedure for prolonged physiological studies of the urinary tract in dogs, and might similarly find some clinical application in humans.

Patterns of Renal Damage in the Management of Vesical Exstrophy

We reviewed the patterns of pyelonephritic damage that occurred during surgical management of bladder exstrophy. Renal injury patterns indicate that papillary configuration and obstruction coupled with urinary infection were the primary factors in producing damage. Diversion by the ileal conduit resulted in frequent injury, whereas the colon conduit was associated rarely with renal damage. The incidence of renal injury from bladder closure and continence attempts suggested closure as a reasonable course, especially in view of present continence results.

Vesical exstrophy with epispadias twenty-year follo-up

A case we believe to be the longest with successful anatomic closure of exstrophy and epispadias without reflux or incontinence is reported. The persistence of the squamous metaplasia in the bladder despite twenty years of “turn-in” is noteworthy. The original method of closure reveals no “secret” to account for the success. The method of repair of traumatic rupture is presented.

Genital function in males with vesical exstrophy and epispadias : M. K. Hanna, and D. Innes Williams. Brit. J. Urol. 44: 169–174 (April), 1972

Genital function in males with vesical exstrophy and epispadias : M. K. Hanna, and D. Innes Williams. Brit. J. Urol. 44: 169–174 (April), 1972

Genital function in males with vesical exstrophy and epispadias.

British Journal of UrologyVolume 44, Issue 2 p. 169-174 GENITAL FUNCTION IN MALES WITH VESICAL EXSTROPHY AND EPISPADIAS M. K. Hanna M.B., B.Ch.(Cairo), F.R.C.S., F.R.C.S.(Ed.), M. K. Hanna M.B., B.Ch.(Cairo), F.R.C.S., F.R.C.S.(Ed.) The Institute of Urology, London, W.C.2 Surgical Registrar, The Hammersmith Hospital, Du Cane Road, London, W.12.Search for more papers by this authorD. Innes Williams M.D., M.Ch., F.R.C.S., D. Innes Williams M.D., M.Ch., F.R.C.S. The Institute of Urology, London, W.C.2Search for more papers by this author M. K. Hanna M.B., B.Ch.(Cairo), F.R.C.S., F.R.C.S.(Ed.), M. K. Hanna M.B., B.Ch.(Cairo), F.R.C.S., F.R.C.S.(Ed.) The Institute of Urology, London, W.C.2 Surgical Registrar, The Hammersmith Hospital, Du Cane Road, London, W.12.Search for more papers by this authorD. Innes Williams M.D., M.Ch., F.R.C.S., D. Innes Williams M.D., M.Ch., F.R.C.S. The Institute of Urology, London, W.C.2Search for more papers by this author First published: April 1972 https://doi.org/10.1111/j.1464-410X.1972.tb10062.xCitations: 39 AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume44, Issue2April 1972Pages 169-174 RelatedInformation

Epispadias.

Diastasis of the symphysis pubis has been commonly associated with exstrophy of the bladder but can occur without this anomaly. The most common anomaly, excluding bladder exstrophy, is epispadias. This defect of the urethra is the subject of this report, and the material consists of a review of the cases of epispadias seen at the Columbia-Presbyterian Medical Center in the past fifteen years. The investigation was undertaken primarily to seek a correlation between the radiographic and clinical findings. Epispadias occurs once in 30,000 births and afflicts males three times more frequently than females (1). This condition may be considered a precursor to vesical exstrophy. There are varying degrees of absence of the anterior portion of the urethra, the severity of which determines the clinical findings and symptoms. In females clitoric, subsymphyseal, and complete epispadias are described. However, since sphincteric involvement is present in 90 per cent of the females afflicted, incontinence is prevalent. Wide separation of the labia, bifid clitoris, flattened mons pubis, and diastasis of the pubic bones vary in this maldevelopment of the urethra. In males glandular, penile, and complete forms exist. The most common type is glandular with the urethra opening on the dorsum of the phallus behind the glans. In the penile form the meatus may be seen at any point along the shaft; most frequently it occurs at the base of the penis. A dorsal sulcus lined by urethral mucosa is present, associated with a spade-like glans which is uncovered (1, 3–6). In the complete form, incontinence is the rule, owing to the inadequate development of the sphincteric mechanism. The external sphincteric structures form an open arc whose ends are attached to the separated pubic bones. The rami are connected by an interpubic band anterior to the urethra. As in the female, the urethra is open to the bladder neck and the bladder itself is thin-walled with small capacity. Due to the incontinence there is a small bladder capacity since the bladder has never had to stretch to accommodate a large volume of urine. Material Fourteen cases of epispadias were reviewed, consisting of 3 females and 11 males. The females had complete epispadias with incontinence, and after their operative procedure, 2 girls showed vesicoureteral reflux, one with incontinence. Five of the 11 males had complete epispadias, with preoperative incontinence in 4 cases. In this group surgery was followed by only slight improvement to a state of stress incontinence in one who was previously totally incontinent, total incontinence in another who had been incontinent prior to surgery, and continence in 3, one of whom now showed vesicoureteral reflux. During a two-year follow-up, no evidence of clinical pyelonephritis was found in the patient who showed vesicoureteral reflux. Long-term follow-up results are not available.