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PubMed1997

[Continent urostomy: sigmoid reservoir and sigmoid hydraulic valve].

We report our experience of continent sigmoidostomy. The technique consisted in urinary diversion with sigmoid pouch and hydraulic valve. Eleven patients underwent this procedure (10 men and 1 women, mean age 48 years, range 20 to 77 years). Indications were bladder tumor in 7 cases, bladder exstrophy in 2 patients, neurogenic bladder in 1 case and 1 bladder with a small capacity secondary to a stricture of traumatic urethra. The pouch was made according to the detubularized model. The sigmoid was opened on its antimesenteric edge, leaving the distal portion of the sigmoid intended to do the sigmoid valve. The posterior edges of the colonic segment opened were alined then secured by a Dexon 3/0 whipping then the anterior adges were secured, as the former after reimplantation of the ureters according to Camey Leduc or Politano Leadbetter's procedure. The post operative follow-up was marked by a fistula of the pouch in one case treated by securing it. All the patients were continent day and night. The purpose of this study was the description of the technique and the results of the continent sigmoïdostomy.

Adult↗
PubMed1986

Bladder duplication with one exstrophy and one cloaca.

Bladder duplication is a rare anomaly. Less than 100 cases of all types have been described in the literature. When duplication of the bladder occurs, it has previously been described as a mirror image or as a septate bladder. A newborn female presented with what appeared to be a complete bladder exstrophy, a large ruptured omphalocele with perforated large bowel, an imperforate anus, and a bifid clitoris. Urine was seen emanating from various sites: the bladder mucosa, the mucous fistula constructed at the time of the colostomy, and the left hemiclitoris. The anatomic puzzle was resolved when multiple studies disclosed the problem to be a bladder duplication. The bladder on the anterior abdominal wall was exstrophied, the intraabdominal bladder was a cloaca, and the left hemiclitoris contained a phallic urethra, which drained the intraabdominal bladder. Total correction with normal function was obtained. This patient demonstrates that bladder duplication may present in various ways.

Abnormalities, Multiple↗
PubMed2000

OEIS complex--a population study.

Using a novel method for the analysis of infants with multiple malformations, we investigated the cluster of associated malformations called the OEIS (omphalocele, bladder exstrophy, imperforate anus, spine defect) complex among 5,260 infants with multiple malformations identified in four large registers of congenital malformations, corresponding to 5.84 million births. The existence of the OEIS complex was clearly demonstrated and malformations entering it could be defined. Other than the four classical malformations, omphalocele, bladder exstrophy, imperforate anus, and spine malformation, a strong association with spina bifida and intersex was stressed. Spine malformations occurred not only in the lumbosacral level but also more cranially, and an association also with upper spina bifida could be demonstrated. No specific association with any other malformation, including cardiac defects, was apparent. The OEIS complex is an unusually clearly defined entity among the various nonrandom associations which have been described.

Abnormalities, Multiple↗
PubMed1990

Urethral duplication with single bladder and multiple genitourinary abnormalities: an example of continent urinary diversion.

A 13-year-old girl with multiple genitourinary malformations, incomplete bladder exstrophy, urethral duplication with single bladder, septate vagina, and total urinary incontinence is presented. Prior to admission she had undergone surgery for teratoma and calculi of the bladder and partial fecal incontinence. A continent urinary diversion was done by bladder augmentation using a cecal-colonic segment and by surgical closure of the bladder neck. The distal end of the appendix was brought to the skin as an inconspicuous, easily catheterizable, watertight stoma, our modification of the Mitrofanoff procedure. Excision of the vaginal septum, creation of an introitus, and unification of the split clitoris improved the cosmetic appearance of the external genitalia and improved the outlook for a normal sexual life and pregnancy.

Abnormalities, Multiple↗
PubMed2001

Fraser syndrome with bladder pseudoexstrophy.

We report a one year old girl with Fraser Syndrome (the association of craniofacial abnormalities, syndactaly and cryptophlamos) and multiple urogenital abnormalities including clitorimegaly, left renal agenesis and a unique urinary bladder exstrophy variant (psuedoexstrophy) with intact bladder which herniates through the lower abdominal wall defect.

Abnormalities, Multiple↗
PubMed2006

Rectosigmoid pouch (Mainz Pouch II) in children.

PURPOSE: Continent anal urinary diversion is a therapeutic option in bladder exstrophy. We report our long-term results with the rectosigmoid pouch (Mainz pouch II), a modification of the classic ureterosigmoidostomy. MATERIALS AND METHODS: A total of 38 children with a mean age of 5 years (range 0.5 to 17) underwent a Mainz pouch II procedure between 1991 and 2004. Most patients (33) had bladder exstrophy or incontinent epispadias. In 14 children (37%) urinary diversion was performed after failed primary reconstruction. In 6 children conversion was performed from an incontinent type of urinary diversion. Renal function, continence and metabolic changes were analyzed. A total of 35 children were followed for a mean of 112 months (range 5 to 147). RESULTS: All children were continent during the daytime but 3 (8.6%) suffered from nighttime incontinence requiring pads. With respect to the upper urinary tract, 6 children (15.8%) had development of pyelonephritis, mostly with stenosis of the ureterointestinal anastomosis. Reimplantation of the ureter was required in 10 of 69 RU (14.5%), of which 7 (10.1%) were due to ureterointestinal stenosis and 3 (4.3%) were due to reflux. Serum creatinine was within normal limits in all children. During followup acid-base balance was monitored, and early alkali supplementation was initiated in 24 of 35 children (69%) when the base excess was less than -2.5 mmol/l. One child had development of clinical acidosis requiring hospitalization. After followup of more than 10 years annual rectosigmoidoscopy was performed in 16 children/young adults without pathological findings. CONCLUSIONS: The Mainz pouch II procedure for children with genitourinary anomalies promises excellent continence rates. However, periodic followup studies are important to check the upper urinary tract and prevent metabolic acidosis. Due to the risk of malignancy at the ureterointestinal anastomosis, endoscopy should be performed annually beginning at postoperative year 10. The Mainz pouch II procedure is safe in the long term. Without stoma, appliance or catheterization this type of continent urinary diversion is specifically suitable for children.

Adolescent↗
PubMed2003

20-year experience with iatrogenic penile injury.

PURPOSE: We review our experience with the management of iatrogenic penile injuries. Apart from circumcision, serious damage to the penis can occur following hypospadias repair, surgery for priapism or total loss of the penis following surgical repair of bladder exstrophy. MATERIALS AND METHODS: A retrospective analysis of patients with iatrogenic penile amputation referred to us between 1980 and 2000 was undertaken. Causes of injury and choice of management were reviewed. RESULTS: Of the 13 cases treated during the 20-year period mechanism of primary injury was circumcision in 4, hypospadias repair in 6, priapism in 1, bladder exstrophy repair in 1 and penile carcinoma in 1. A variety of techniques were used for phallic reconstruction. Penile degloving, division of suspensory ligament and rotational skin flaps achieved penile augmentation and enhancement. Reasonable cosmesis and penile length were achieved in all cases. In indicated cases microsurgical phalloplasty was technically feasible. However long-term followup showed various complications including erosions from the use of a penile stiffener. CONCLUSIONS: The ultimate goal of reconstructive surgery is to have a penis with normal function and appearance. The management of penile injury requires a wide variety of surgical techniques that are tailored to the individual patient. Expedient penile reconstruction is successful and therapeutic delay is associated with complications.

Adolescent↗
PubMed1978

Vesicointestinal fissure revisited.

This complicated anomaly of bladder exstrophy, intestinal fistula, and various degrees of colonic atresia with imperforate anus is readily recognizable. The long-term result of conventional management has been disappointing. Fecal and urinary incontinence have been unavoidable and a source of disappointment to patients and parents. An alternative method of total management is detailed. This variation of management may result in earlier and more complete social acceptability of the child so afflicted. At as young an age as possible, the vesicointestinal connection is detached and the bladder defect as well as the intestinal opening closed. The short colon is freed and transected at its midpoint. The right half of the colon is brought out through a separate wound as a colostomy. The distal half of the divided colon is brought out as mucous fistula on the left. This distal colonic segment is the proposed future colonic loop for urinary diversion to be fashioned in the first year of life. The exstrophied bladder is removed at a later date. Epispadias in the male may be repaired later. A Pediatric Ostomy Club has been organized to include a pediatric stomal therapist and involved nurses and physicians. The group gives the parents advice and moral support. We believe that this approach will permit the child to go to school and carry on relatively normal activity and to be socially acceptable. An overview of our total experience in the management of 25 patients with vesicointestinal fistula details the result in 9 surviving patients.

Anus, Imperforate↗
PubMed2006

Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy-epispadias complex: long-term results.

OBJECTIVE: To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive. PATIENTS AND METHODS: We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.6-16.7) with EEC who had injections with Macroplastique between January 1991 and February 2004; 34 had bladder exstrophy and 18 primary epispadias. For this study we defined success as complete dryness with no use of pads or nappies. Improvement was defined as being occasionally wet but with dry intervals lasting >or= 4 h. RESULTS: The mean (range) follow-up was 4.6 (0.5-9) years. Twenty patients had one injection, 10 had two, 13 had three, six had four, two had six and one had seven injections. In most patients a maximum of three injections predicted the outcome. The injection of Macroplastique was successful in nine patients (17%; with an annual follow-up, two at 1-2 years, three at 2-5 years and four at >5 years), whilst 17 (33%) improved significantly (one at <1 year, two at 1-2 years, eight at 2-5 years and six at >5 years). Those patients comprised five of 18 (27%) with epispadias and four of 34 (12%) with exstrophy. A history of previous surgery and gender had no significant effect on the outcome. Overall half the patients benefited from the procedure. CONCLUSIONS: This series confirms that injection with Macroplastique is minimally invasive, durable in significantly many patients and has a reasonable success rate. A history of previous surgery and gender had no significant effect on the outcome. Patients with epispadias are more likely to benefit from an injection with Macroplastique than those with bladder exstrophy. A maximum of three injections is predictive with reasonable certainty of any benefit from the procedure.

Adolescent↗
PubMed2007

OEIS complex: prenatal ultrasound and autopsy findings.

OBJECTIVE: To describe prenatal ultrasound and autopsy findings in fetuses with OEIS (omphalocele, bladder exstrophy, imperforate anus, spina bifida) complex. METHODS: This was a retrospective study of the nine cases with OEIS complex diagnosed at our center using detailed fetal ultrasound during the last 10 years. We summarized the fetal ultrasound findings that led to the diagnosis and compared them with the autopsy results. RESULTS: All affected fetuses were diagnosed using detailed fetal ultrasound after 16 weeks' gestation. The main prenatal findings were omphalocele, skin-covered lumbosacral neural tube defect, non-visualized bladder and limb defects. Prenatal sonography failed to detect the abnormal genitalia, bladder exstrophy and anal atresia. All cases had abnormalities in a 'diaper distribution', which helped in making the prenatal diagnosis. Eight of the nine couples chose to terminate the pregnancies following multidisciplinary counseling. The pregnancy that was continued was a case with dizygotic twins discordant for OEIS, and the affected fetus died in utero. CONCLUSIONS: The combination of the following ultrasound findings: ventral wall defect, spinal defect and a non-visualized bladder with or without limb defects, are characteristic of OEIS complex. Diagnosis can be made with confidence as early as 16 weeks' gestation, although earlier diagnosis may be possible.

Abnormalities, Multiple↗
PubMed2006

Oblique pelvic osteotomy in the exstrophy/epispadias complex.

We reviewed retrospectively 45 patients (46 procedures) with bladder exstrophy treated by bilateral oblique pelvic osteotomy in conjunction with genitourinary repair. The operative technique and post-operative management with or without external fixation are described. A total of 21 patients attended a special follow-up clinic and 24 were interviewed by telephone. The mean follow-up time was 57 months (24 to 108). Of the 45 patients, 42 reported no pain or functional disability, although six had a waddling gait and two had marked external rotation of the hip. Complications included three cases of infection and loosening of the external fixator requiring early removal with no deleterious effect. Mid-line closure failed in one neonate managed in plaster. This patient underwent a successful revision procedure several months later using repeat osteotomies and external fixation. The percentage pubic approximation was measured on anteroposterior radiographs pre-operatively, post-operatively and at final follow-up. The mean approximation was 37% (12% to 76%). It varied markedly with age and was better when external fixation was used. The wide range reflects the inability of the anterior segment to develop naturally in spite of close approximation at operation. We conclude that bilateral oblique pelvic osteotomy with or without external fixation is useful in the management of difficult primary closure in bladder exstrophy, failed primary closure and secondary reconstruction.

Age Factors↗
PubMed1984

The genetics of urinary tract malformations.

In this discussion I have excluded consideration of the genetics of purely renal malformations, such as polycystic kidneys, and of functional disorders of the kidney. Systematic family studies are available for renal agenesis, duplication of the ureters, vesico-ureteric reflux (each probably due to maldevelopment of the ureteric bud), bladder exstrophy and hypospadias as isolated malformations. Renal agenesis has a birth frequency of about 1.2 in 10 000 and the proportion affected of sibs is about 3 per cent. Duplication of the ureter has a birth frequency of about 1 per cent and the proportion affected of sibs and parents of probands is about 12 per cent. Vesico-ureteral reflux also has a prevalence in early childhood of about 1 per cent and the proportion of sibs affected is about 10 per cent. Bladder exstrophy has a birth frequency of about 1 in 20 000 and perhaps about 1 per cent of sibs are affected. Hypospadias has a birth frequency in boys of about 1 in 300 and the proportion affected of brothers is about 10 per cent. Further family studies are needed of these malformations when they occur in isolation. Either the multifactorial threshold model or dominant inheritance with reduced penetrance and varied expressivity would fit the data available.

Bladder Exstrophy↗
PubMed2004

Periurethral constrictor in pediatric urology: long-term followup.

PURPOSE: We report long-term followup of a new device for the treatment of urinary incontinence in children. MATERIALS AND METHODS: A periurethral constrictor was implanted in 29 boys and 13 girls 3 to 17 years old (mean age 10.2, median age 10) during the last 9 years. Of the patients 29 had neurogenic bladder, 12 had bladder exstrophy and 1 had megalourethra. Bladder augmentation was done simultaneously in 34 patients. The device was implanted around the bladder neck in 41 cases and at the bulbous urethra in 1. Continence was achieved in all cases when the device remained in situ. RESULTS: Followup ranged from 4 to 104 months (mean 63, median 75). In 23 patients (82.1%) in the neurogenic group and the patient with megalourethra the device remains in situ and continence is preserved. Clean intermittent catheterization is performed without difficulty. In 4 patients the device was extracted due to erosion or infection. The device was extracted due to erosion and urine extravasation in 10 exstrophy group patients. Two patients have the device and perform post-void catheterization. CONCLUSIONS: Based on the data from this long-term study we conclude that the periurethral constrictor is a safe alternative for the treatment of urinary incontinence in children. Like other devices it must be used with caution in cases of bladder exstrophy.

Adolescent↗
PubMed2003

Long-term evaluation of metabolic profile and bone mineral density after ileocystoplasty in children.

PURPOSE: We evaluated the long-term effects of ileocystoplasty on linear growth, serum electrolytes, acid-base profile and bone mineral density (BMD) in a group of neurologically intact children with a mean followup of 8.9 years. MATERIALS AND METHODS: Between 1988 and 1997, 9 girls and 16 boys with a mean age of 6 years (range 1 month to 14 years) underwent ileocystoplasty for etiologies other than myelomeningocele and neuropathic bladder. Indications for ileocystoplasty were small noncompliant bladder secondary to bladder exstrophy in 12 cases, bladder outlet obstruction in 10 and post-partial cystectomy for rhabdomyosarcoma in 3. All patients underwent clinical evaluation, supine height measurement, serum electrolytes, arterial blood gases and BMD measurement using a fan beam dual energy absorpitometry scan. BMD was measured at L1-L4 and corrected for age and sex. RESULTS: Followup ranged from 4 to 13 years (mean 8.9). Serum creatinine was normal in 20 of the 25 patients. All patients had normal supine height measurement with a mean of 45th (+/- 9) centile on growth charts. Serum electrolytes, calcium, phosphorus and arterial blood gases were normal in all patients. Mean BMD corrected for age and sex was 89 (+/- 10)%. BMD was normal in 17 of 25 (68%) patients. Mild reduction in bone density between 1 and 2 standard deviations below the age/sex mean was documented in 3 (12%) patients and the remaining 5 (20%) showed marked osteopenia of 2 or more standard deviations. Of the latter 5 patients 2 had increased serum creatinine, 1 had a history of radiotherapy for pelvic rhabdomyosarcoma and 2 had cloacal exstrophy and short bowel, all of which might have contributed to the osteopenia. CONCLUSIONS: Ileocystoplasty for children with normal kidney function is not associated with alterations in serum electrolytes or arterial blood gases in the long term. However, 32% of patients showed variable degrees of reduction in BMD. Although marked reduction in BMD was associated with cofactors, 12% of patients had evidence of mild osteopenia in absence of those cofactors. We recommend routine preoperative and longitudinal followup BMD measurements for children undergoing intestinal bladder augmentation.

Adolescent↗
PubMed1992

Exstrophy of the bladder: primary closure after iliac osteotomies without external or internal fixation.

A total of 6 consecutive infants with exstrophy of the bladder underwent iliac osteotomy in association with primary closure of the bladder without internal or external fixation of the bony pelvis. Hospitalization time was short and there were no resulting problems with healing, bony deformities or subsequent ambulation. Fixation of the bony pelvis after iliac osteotomy does not appear to be necessary in nonambulatory patients undergoing primary closure for bladder exstrophy.

Bladder Exstrophy↗
PubMed1980

Bladder reconstruction in exstrophy.

Between 1968 and 1978, 35 patients with complete exstrophy of the bladder have been treated. In 31 cases reconstruction was attempted using a 3-step procedure. First, the bladder was closed with a posterior iliac osteotomy and in the male patient the penis was lengthened. Next, an antireflux procedure and bladder neck reconstruction were done. The modification of the Young-Dees procedure was used, which allowed reconstruction of the vesicourethral angle as well as creation of an anterior muscular loop. Finally, the anterior epispadias was repaired. Of 16 patients who completed surgical treatment more than 1 year ago 6 female and 5 male subjects are cured, that is continent with a normal excretory urogram and no reflex. One male patient is improving and 4 boys are failures. The operative technique, postoperative training, results and selection of patients for functional closure are discussed.

Bladder Exstrophy↗
PubMed1995

Augmentation cystoplasty in boys with posterior urethral valves.

The role of augmentation cystoplasty in the neuropathic and exstrophy bladder has been well documented. However, its place and the timing of such surgery in the "valve bladder" are not well established. We report our experience with augmentation cystoplasty in 20 boys with previously treated posterior urethral valves. Urodynamic studies confirmed poorly compliant, unstable bladders with low functional capacities, which had failed to respond to anticholinergic treatment in all patients. The bladder was augmented with ileum in 9 cases, stomach in 7, colon in 2 and ureter in 2. A Mitrofanoff channel was fashioned in 6 cases. Upper tract dilatation improved in 17 patients and remained stable in 3. Of the patients 17 are dry day and night. Eleven patients void spontaneously without significant residual urine, 7 are on clean intermittent catheterization for residual urine of greater than 50 ml. and 2 are completely dependent on catheterization. Augmentation cystoplasty is a safe and effective method to achieve continence with a low capacity, poorly compliant bladder in children with posterior urethral valves who do not respond to medical management. In contrast to the neuropathic and exstrophy bladder, the augmented valve bladder allows spontaneous voiding without significant residual urine in the majority of cases. Early intervention in these patients may prevent deterioration in renal function.

Adolescent↗