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PubMed1987

Computed tomography of Kock and modified Kock continent ileal reservoir.

Kock or modified (Javadpour) ureteroiliostomy was performed in 5 patients following cystectomy for bladder carcinoma or exstrophy. Computed tomography was utilized to demonstrate alterations in pelvic anatomy after construction of the ileal reservoirs. Detailed evaluation of the inlet and outlet antireflux arms of the continent reservoir was possible. Appreciation of the expected alterations in pelvic anatomy accompanying the Kock or modified Kock procedure will be necessary to detect postoperative complications and to achieve early recognition of recurrent carcinoma. Potential advantages of the modified Kock ileal reservoir, as developed by Javadpour, are presented.

Aged↗
PubMed1995

Prenatal detection of anterior abdominal wall defects with US.

The size and position of an anterior abdominal wall defect, its contents, and its association with other anomalies are features that can be diagnosed in utero with ultrasound and that allow a differential diagnosis to be made. The correct prenatal diagnosis is extremely important for patient management. The key feature for sonographically distinguishing these conditions is the position of the defect in relation to the umbilical cord insertion. Omphaloceles and pentalogy of Cantrell usually involve a midline defect at the umbilical cord insertion. Gastroschisis most frequently consists of a small, right-sided paraumbilical defect. Eccentric, large lateral defects are typically present in limb-body wall complex or amniotic band syndrome. Bladder and cloacal exstrophy involve the infraumbilical region. In addition, the size of the defect, the organs eviscerated, the presence of membranes or bands, and any associated abnormalities help determine the correct diagnosis. Increased knowledge of these uncommon fetal conditions should result in better detection, more accurate diagnosis, and improved management of anterior abdominal wall defects.

Abdominal Muscles↗
PubMed1995

Ileocystoplasty in children: assessing safety and success.

From 1987 to 1992, bladder augmentation using clam ileocystoplasty was performed in 39 children 1.5-17.5 years old (mean age 9.7 years). Indications for augmentation included neuropathic bladder, epispadias-exstrophy complex and posterior urethral valves. A satisfactory bladder capacity at safe storage pressures of < 20 cm saline was achieved in all patients. Hydroureteronephrosis resolved or improved in 33 of the 36 moieties (91.7%). No patient showed biochemical or radiological deterioration of the upper tracts. The incidence of symptomatic urinary tract infection fell significantly postoperatively. Seven patients (17.9%) developed a total of 10 complications, with rupture of the augmented bladder occurring in 4 patients (10.3%).

Adolescent↗
PubMed2005

The use of refluxing ureter in the creation of a Mitrofanoff channel in children undergoing bladder augmentation: is a formal reimplantation necessary?

To study the fate of a refluxing ureteral stump used as a Mitrofanoff channel (MC) for clean intermittent catheterization (CIC), without reimplantation in augmented bladders. Ten boys and seven girls, 1-14 years old (mean 6.5 years) seen in period 1998-2005, underwent creation of MC using refluxing or potentially refluxing ureters. Concomitant bladder augmentation (BA) was performed for various abnormalities of lower urinary tract, including exstrophy epispadias complex, neurogenic bladder cloaca and posterior urethral valve. The uretero-vesical junction was maintained intact during creation of the MC. Follow-up period ranged from 2-72 months (mean 32.5 months). Satisfactory CIC was possible in 14 children while in two children the stoma became obstructed due to non-usage. Urinary leak via the ureteric MC was seen in only one patient who partially responded to anticholinergic therapy and a shortened CIC interval but this needed to be formally closed subsequently. A refluxing ureter can be used in the creation of a MC without formal reimplantation in the majority of children undergoing BA.

Adolescent↗
PubMed1998

Myofascial wrap to treat intractable urinary incontinence in children.

OBJECTIVES: The management of intractable urinary incontinence in the patient with cloacal or bladder exstrophy/epispadias, failed bladder neck plasty, or failed augmentation cystoplasty remains a surgical challenge. The myofascial wrap, a modification of the rectus fascial wrap, was developed to treat intractable urinary incontinence due to sphincteric incompetence in these problematic cases. A full-thickness, vascularized pedicle of anterior rectus sheath, rectus abdominis muscle, and posterior rector sheath is incorporated into a bladder neck wrap to provide support, mucosal coaptation, and active muscular tone. METHODS: Eight patients (5 females and 3 males) with total urinary incontinence due to sphincteric incompetence underwent the myofascial wrap. Urinary tract pathology included cloacal exstrophy (2), female epispadias (2), classic bladder exstrophy (1), male epispadias (1), myelomeningocele (1), and a pelvic tumor (1). The procedure is performed by harvesting a full-thickness strip of pedicled rectus muscle along with the anterior and posterior fascial sheaths. The strip is passed underneath and then over the bladder neck in a near 360 degrees wrap. The free end of the wrap is anchored into the pubic bone in an ipsilateral subperiosteal pouch. RESULTS: Six of the 8 patients are completely continent, and 2 patients void spontaneously without the need for catheterization. CONCLUSIONS: The myofascial wrap provides support, mucosal coaptation, and muscular tone to an incompetent sphincter and bladder neck. Favorable results in a very difficult population of pediatric patients warrant its continued use.

Adolescent↗
PubMed1985

Cloacal exstrophy: a 22-year experience.

Our 22-year experience in the management of 12 patients with cloacal exstrophy is discussed. All patients underwent functional bladder closure. Of 7 evaluable patients 3 (43 per cent) have continent intervals of 3 to 4 hours and constitute the first report of satisfactory urinary continence following functional bladder closure in patients with cloacal exstrophy. Of 8 patients with an XY karyotype 6 were raised as girls with satisfactory genitoplasty, while 2 raised as boys had functionally inadequate external genitalia. The management of the rudimentary hindgut varied and the alternative approaches are discussed. It appears that this patient population can be rehabilitated to lead productive lives.

Abnormalities, Multiple↗
PubMed2005

Bladder neck fistula after the complete primary repair of exstrophy: a multi-institutional experience.

PURPOSE: The major goals of complete primary repair of exstrophy (CPRE) are the re-creation of normal anatomy which allows bladder cycling and to reduce the number of future procedures necessary to achieve continence. It is unclear whether CPRE is associated with a higher bladder neck fistula rate than the traditional staged repair. We review a multi-institutional experience with the CPRE technique to evaluate the rate of bladder neck fistula. MATERIALS AND METHODS: A retrospective review of 18 boys and 4 girls who underwent CPRE at 4 tertiary pediatric urology referral centers during the last 6 years was performed. All pertinent technical aspects were reviewed, including timing of procedure, whether osteotomies were performed, and number of layers used to reconstruct the bladder neck and urethra. Complications were noted, especially that of bladder neck fistula. RESULTS: Mean followup was 22.6 months. Of the patients 14 (64%) underwent primary closure within the first 48 hours of life and only 1 required osteotomies. The remaining 8 patients underwent closure between 5 days and 3 months of age (mean 24.6 days) and all required osteotomies. Bladder neck fistula occurred postoperatively at the pubic junction in 9 males (41%). Four cases had a 2 layer closure that was covered with a single layer small intestinal submucosa onlay (Surgisis) and no patient had a fistula. Fistulas developed in 62.5% of patients with delayed closure vs 29% of those with immediate closure (p = 0.135). Two fistulas closed spontaneously and 7 required surgical closure at a mean of 7.5 months after the fistula occurred. CONCLUSIONS: This multi-institutional study demonstrates that bladder neck fistulas occur in almost half of patients following CPRE by experienced pediatric urologists. While spontaneous closure is possible, most will eventually require repair. The long-term implications of this finding with regard to continence and the need for additional bladder neck procedures remain to be seen. We are encouraged by the preliminary results of small intestinal submucosa coverage and will continue to evaluate its use at the time of primary exstrophy closure.

Bladder Exstrophy↗
PubMed1998

Covered exstrophy: a rare variant.

A case of covered exstrophy without sequestration of a bowel segment is reported. A 4-year-old female presented with dribbling of urine. Treatment to date has been simple excision of the covered membrane with functional closure of the bladder and bilateral posterior iliac osteotomies, with reconstruction of the bladder neck and genitalia to be performed at a later date. The embryogenesis of this rare variant, a review of the reported cases, and management options are discussed. Keyword Covered exstrophy. Exstrophy. Bladder variants

Bladder Exstrophy↗
PubMed2002

Surgical reconstruction of exstrophy-epispadias complex: analysis of 13 patients.

BACKGROUND: Because exstrophy-epispadias complex is uncommon and satisfactory surgical reconstruction outcomes are difficult to achieve, the surgical repairs by one surgeon (PS) were analysed over a 14-year period. METHODS: Retrospective analysis was performed on 13 patients with the complex who underwent surgery between January 1986 to August 2000. Cosmesis and continence were evaluated. Complications including wound dehiscence and urethrocutaneous fistula were reported. RESULTS: Of six patients with classical exstrophy who underwent functional bladder closure, all had good cosmesis except one who had partial dehiscence. Four patients with isolated epispadias had satisfactory cosmesis. Urethrocutaneous fistula was found in one boy in the classical exstrophy group. Continence was achieved in three out of four patients who had bladder neck reconstruction. One girl whose bladder neck was severely obstructed after functional bladder closure, had continent catheterizable stoma. CONCLUSION: Functional bladder closure yielded satisfactory cosmetic outcome. Bladder neck reconstruction made the patient dry in 75% of cases.

Adolescent↗
PubMed2001

Umbilical reconstruction in patients with exstrophy: the kangaroo pouch technique.

PURPOSE: Before the current attitude of umbilical preservation and transposition at bladder closure in patients with exstrophy the navel was systematically removed. Many patients without an umbilicus complain about this deformity. We report a simple technique of umbilical reconstruction using a rectangular skin flap fashioned as a kangaroo pouch. MATERIALS AND METHODS: Four patients 10 to 20 years old with exstrophy underwent this procedure. A small cutaneous pouch was fashioned by folding a vertical rectangular skin flap and the pouch was anchored deeply to the rectus fascia. A compressive dressing was packed into the new umbilicus and left in place for several days. RESULTS: The 4 patients have an excellent cosmetic result with adequate location, good morphology and sufficient depth at a mean followup of 8 months (range 6 to 11). CONCLUSIONS: Our technique of umbilical reconstruction is easy to perform and provides good mid-term cosmetic results. This technique is particularly adapted for patients with exstrophy who often require external genital reconstruction at the same time.

Adolescent↗
PubMed1990

Cloacal exstrophy: individualized management through a staged surgical approach.

Cloacal exstrophy, centered on the maldevelopment of the primitive streak mesoderm and cloacal membrane, results in bladder and intestinal exstrophy, omphalocele, gender confusion, and hindgut deformity. The surgical management and outcome of 10 of 14 survivors (1965 to 1988) are described. Genotypic males (6) were assigned male (2) or female (4) phenotype. Genotypic females (4) were unchanged. All had omphalocele closure in the newborn period. Two had loop stomas. Eight had end stomas (ileostomy [6], ileocolostomy [2]). Toddler and adolescent reconstruction differed in each. Early in the study, abdominoperineal pull-through failed in four patients, necessitating permanent stoma. Four patients had a stoma from the outset. Augmentation using colon remnant improved water loss and nutrition in two infants. Exstrophy turn-in for urinary reservoir was considered in all, but was impossible in three who required urinary diversion. Six patients had exstrophy turn-in and now void by clean intermittent catheterization (4), continent vesicostomy (1), and incontinent (1). Hindgut augmentation improved urinary capacity in two. Two genotypic-phenotypic males had penile lengthening. Four genotypic male-phenotypic females had early orchiectomy with subsequent clitoroplasty or vaginoplasty. Four genotypic-phenotypic females had clitoroplasty or vaginoplasty. Cloacal exstrophy is compatible with a useful life and sound psychologic development, but requires staged reconstruction with long-term support and follow-up.

Abnormalities, Multiple↗
PubMed1977

Embryologic considerations of diphallus and associated anomalies.

Two patients, 1 adult and 1 infant, with complete duplication of the penis are described. The adult had a single bladder, a bifid scrotum, a low abdominal wall hernia, separation of the symphysis pubis, bilateral inguinal hernias, rectal prolapse, bilateral vesicoureteral reflux and bilateral staghorn calculi. The infant had duplication of the bladder, hypoplasia of the left kidney, lumbosacral anomalies, a hypoplastic left lower extremity, an imperforate anus, a infraumbilical wall hernia and separation of the symphysis pubis. A survey of the anomalies accompanying the 2 main types of diphallus (shaft and glans, and glans alone)showed that there was a preponderance of anomalies of posterior structures such as colon, bladder and spine in the shaft and glans diphallus, and a higher incidence of isolated exstrophy of the bladder in diphallus of the glans alone. The embryogenic implications of these findings are discussed.

Abnormalities, Multiple↗
PubMed1999

[Bladder augmentation in reconstruction of the urinary tract (1985-1997)].

Bladder augmentation with intestinal or urinary segments has virtually replaced other treatments in the management of both neuropathic and no neuropathic bladder dysfunction that has not responded to pharmacotherapy nor other intervention. We present herein our experience in 55 patients who underwent augmentation cystoplasty. Their mean age were 12.2 years (range 2.5-22.8) and the mean follow-up time was 4 years (1-13.1). They were divided in three groups according to the diagnosis: vesical or cloacal exstrophy (14 patients), neuropathic bladder (36) and posterior urethral valves (5 patients). Indications were: 1) to get a low pressure, high volume reservoir and avoid upper urinary tract damage in low-compliance bladders (41 patients); 2) as an undiversion (8 patients), and 3) prior to renal transplantation. Cystoplasty was performed with bowel segments in 47 cases and ureter in 8, adding some other urological procedures in 22 patients. Mean bladder capacity after 1 year was 400 ml versus 112 as previous value. 52 out of the 55 patients are continent after augmentation. There was no impairment of the renal function in the 5 patients with prior renal failure who underwent cystoplasty. Vesicoureteral reflux disappeared in 78.6% of the patients after cystoplasty. The complications were urinary stones in 5 cases, upper urinary tract infections in 3, and spontaneous bladder perforation in 1 patient. Augmentation cystoplasty is the best choice to achieve a low pressure reservoir, to assure contingency and to avoid progressive damage of the upper urinary tract in neuropathic or no neuropathic pediatric bladder dysfunctions.

Adolescent↗
PubMed1995

Endoscopic treatment of vesico-ureteric reflux and urinary incontinence: technical problems in the paediatric patient.

OBJECTIVE: Bovine collagen has been successfully used for the endoscopic treatment of urinary incontinence (UI) and vesico-ureteric reflux (VUR) in children for more than 8 years, although its long-term efficacy has sometimes been questioned. Its failure is generally ascribed either to technical difficulties or to instability of collagen at the site of the implant. To assess the efficacy of the procedure we evaluated the children treated at this hospital between 1990 and 1993. PATIENTS AND METHODS: A total of 156 children, aged 5 months to 13 years, were treated for VUR. Over the same period, 25 patients aged between 2 and 14 years (nine with neuropathic bladder and 16 with exstrophy-epispadias complex who were still incontinent after bladder neck reconstruction) had periurethral or pericervical glutaraldehyde cross-linked bovine collagen injection for UI. Five more children with exstrophy-epispadias complex (aged 1-3 years) underwent periurethral collagen injection to stimulate bladder enhancement and allow subsequent bladder neck reconstruction. RESULTS: In children treated for VUR, a single injection proved successful in 72.2% of cases (127 ureters); a second collagen injection raised the success rate to 81%. Continence improved in all nine neuropathic bladders and in 10 of 16 children with exstrophy-epispadias complex treated for UI after bladder neck reconstruction. In four of the five exstrophy-epispadias complex patients who were treated to stimulate bladder enhancement, bladder capacity increased by 25%. CONCLUSIONS: Endoscopic treatment of VUR seems to be a valid alternative to open surgery, even though concerns remain about the long-term efficacy of collagen implantation. An important distinction should be made between early and late failure of the procedure. Early failure, which we define as persistence of reflux, is usually due to incorrect technique or technical difficulties. Late failure, or recurrence of reflux, which has previously been attributed to the biodegradability of collagen, seems to be due to the displacement of the injected collagen. Micturition itself or high bladder pressure (such as detrusor instability) could be responsible for the displacement of the injected collagen medially and distally, where it can no longer support the submucosal ureteric tunnel. In the treatment of urinary incontinence, both the implant technique and the choice of the site of injection seem to have a considerable effect on the results. In our experience, endoscopic collagen injection is effective in the treatment of both urinary incontinence and VUR in paediatric patients. Accurate selection of patients and technical adjustments and refinements are essential to obtain the best results.

Adolescent↗
PubMed1978

Urolithiasis in adolescents.

The characteristics of 31 adolescent patients aged 11 to 20 years with urolithiasis were examined by means of a ten-year retrospective chart review. The majority of stones were found to be secondary to preexisting conditions, with the most common being bladder dysfunction (neurogenic or exstrophy). Exogenous corticosteroid therapy appeared to play a role in lithogenesis in five patients and was the only apparent contributing factor in an 11 1/2-year-old girl. Childhood inborn errors of metabolism accounted for the stones in four of the teenagers. An additional four adolescents did not have any definable biochemical, genetic, or anatomic abnormalities and, therefore, no obvious etiology.

Adolescent↗
PubMed1991

Modern treatment of cloacal exstrophy.

We have treated 12 newborns with cloacal exstrophy (10 classical and 2 variants) since 1980. Eleven infants underwent repair and they are all surviving; the one nonoperated infant died of pulmonary hypoplasia. There are six genetic females and six genetic males, five of whom are being raised as females. The initial operation consisted of separating the bowel from the bladder to create an intestinal stoma; closing the omphalocele; and reapproximating (5), closing (4), or leaving the exstrophied bladder undisturbed (2). The importance of creating a "tailgutostomy" instead of an ileostomy to prevent problems with diarrhea, dehydration, and acidosis is emphasized. There have been 71 subsequent operations in these 11 patients (28 general surgical, 25 urological, 9 neurosurgical, and 9 orthopedic). Quality of life, rather than survival, is now the major issue facing patients with cloacal exstrophy. All of these patients except one are neurologically normal. The defect is closed in all patients. Three patients wear no appliances, 7 wear one (gastrointestinal [GI]), and one wears two (GI and genitourinary [GU]). We developed a scoring system to analyze bowel and bladder continence: voluntary control = 3; control with an enema program or intermittent catheterization = 2; incontinence with a well-functioning stoma = 1; and incontinence without a stoma = 0. The best continence score is 6 (GU + GI). We currently have 7 patients with a continence score of 1 (colostomy + incontinent bladder); 1 with a score of 2 (ileostomy + incontinent ureteroenterostomy); 2 with a score of 4 (enema program + continent urinary diversion); and 1 with a score of 5 (enema program + continent bladder).(ABSTRACT TRUNCATED AT 250 WORDS)

Bladder Exstrophy↗
PubMed2003

Continent urinary diversion and the exstrophy-epispadias complex.

PURPOSE: The use of continent urinary reservoirs has gained wide acceptance, particularly in urinary reconstruction in children with a small capacity or neuropathic bladder. When augmentation cystoplasty is combined with clean intermittent catheterization, patients are often able to achieve continence with low intravesical filling pressures and renal preservation. Often this approach requires fashioning a continent cutaneous stoma, which remains the most challenging aspect of continent urinary reservoirs. We analyzed our experience with continent diversion in patients with exstrophy-epispadias to determine complications and long-term results. MATERIALS AND METHODS: We performed a retrospective database review of 704 cases of exstrophy-epispadias. Medical records were then used to identify those patients who had undergone creation of a continent urinary reservoir. Charts were reviewed to determine initial diagnosis, augmentation technique, continence mechanism, age, preoperative and postoperative bladder capacity, continence status and complications. RESULTS: Of the 91 patients identified (68 male, 23 female) who had undergone continent urinary diversion classic bladder exstrophy was present in 80, cloacal exstrophy in 8, complete male epispadias in 2 and female epispadias in 1. The most common techniques for augmentation and continent diversion were ileocystoplasty (41 patients [45%]) and sigmoid cystoplasty (30 [33%]), respectively. Appendix was used in 67 patients (74%) and variants of the Mitrofanoff procedure using segments of tapered ileum or ureter were used to create a continent stoma in 10 (11%). Bladder neck transection was performed in 59 patients (65%). Mean age at augmentation and continent diversion was 8 years (range 2 to 25), with a mean preoperative bladder capacity of 77 cc (15 to 220). Mean followup was 6 years (range 6 months to 12 years). Of the 91 patients 85 (93%) were continent with clean intermittent catheterization per stoma. Of these 85 patients 13 required anticholinergics and alpha-agonists to achieve continence. Six patients (7%) were incontinent after the procedure. Analysis of bladder capacity measurements after augmentation and continent diversion revealed that mean postoperative volume and mean volume increase were 404 cc (range 250 to 640) and 524%, respectively. The most common complications were bladder stone formation (24 patients [26%]) and stomal stenosis (21 [23%]). Bladder stones recurred in 9 patients and stomal stenosis in 3. Other less common complications were vesicourethral fistula (3 patients) and a small bladder perforation (2). CONCLUSION: Augmentation and continent diversion procedures can increase the functional capacity of the small contracted noncompliant exstrophic bladder, and allow the vast majority of patients to achieve continence and preserve renal function. Bladder calculi and stomal stenosis pose the most significant long-term complications in these patients.

Adolescent↗