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Reconstruction of the umbilicus has cosmetic and functional value in the surgical treatment of the child with bladder exstrophy. We report on 12 patients who underwent creation of a neoumbilicus as part of genitourinary reconstruction.
OBJECTIVE: To determine the factors that control quality of life as perceived by adolescent patients with bladder exstrophy, and to compare their views using standard instruments. PATIENTS AND METHODS: Sixteen patients (mean age 19 years, range 16-21, 11 male and five female) were recruited from the departmental database; they represented 46% of those available for the study. After giving informed consent, each had a semi-structured interview, augmented by completing a self-reported scale, with the principal investigator. They then completed the Culture-Free Self-Esteem Inventory-2 (CFSEI-2) and the Brief Symptom Inventory (BSI). The interviews were recorded on audiocassette, transcribed verbatim and evaluated using interpretative phenomenological analysis. RESULTS: In the interviews there was a remarkable consistency in the domains identified as important to the patients. There was a wish to be normal and to be treated as such. This was defined (amongst other items) as being able to void with an appropriate noise, being treated as peers at school and at home, and having an umbilicus. All patients reported some bullying (all but one in the past), but only severely in three. Concerns about self-image centred on scars and genital appearance. Very similar and effective coping strategies had been created, including practical (e.g. suitable clothes) and emotional (e.g. joking, control of revealed information) aspects. Special arrangements made to help (care by a special assistant or use of a disabled lavatory at school) served only to emphasize their abnormality and were resented. No overt psychiatric or psychological morbidity was detected. There was no difference in scores with the CFSEI-2 or BSI from established age-related norms. CONCLUSION: This study confirms the anecdotally reported strong resilience and personality of adolescents with exstrophy. The domains that patients considered important were not those that their carers might have expected or that are used in standard quality-of-life instruments. No morbidity was identified by the two instruments used. In exstrophy, and perhaps in other uncommon conditions, the patients' views of relevant domains should be considered in assessing quality of life.
Surgical anastomosis of the ureter in the sigmoid could lead to osteomalacia. The pathogenesis of this disease is rather complicated and involves several factors, the most important of which is hyerchloremic acidosis associated with modifications of transport across the intestinal lining, and in particular, the elimination of bicarbonates and the reabsorption of H+ ions. This systemic acidosis makes the calcium balance negative by mobilizing calcium bicarbonate from bone and possibly by inhibiting alkaline phosphatase activity. The role of PTH and vitamin D has yet to be satisfactorily explained. The authors present cases of osteomalacia following two uretero-sigmoidostomies, one for bladder exstrophy and the other after bladder removel for tumour. The physiopathological mechanisms of osteomalacia are briefly recalled together with indications of treatment.
PURPOSE: The efficacy of the artificial urinary sphincter to treat sphincteric incontinence in pediatric patients with spina bifida has been clearly reported. The possibility of maintaining spontaneous voiding has usually been the main reason for prosthetic device surgery. We reviewed our experience with the artificial urinary sphincter in patients without spina bifida who had had previous surgery of the bladder neck or proximal urethra. MATERIALS AND METHODS: From 1990 to 2004, 112 children and adolescents underwent implantation of an AMS 800 artificial urinary sphincter. Of the patients 19 males and 4 females (20.5%) between ages 4 and 17 years (mean 8.1) had no spina bifida. Instead there were bladder exstrophy in 12 patients, anorectal malformation with a rectourethral or vesical fistula in 7 and epispadias in 4. A bladder neck cuff between 5.5 and 7.5 cm, and a 61-70 balloon were used in all patients. RESULTS: Only 1 patient was lost to followup. In 22 patients (95.6%) mean followup was 80 months (range 4 to 155). Three sphincters in patients with exstrophy were removed because of erosion and/or infection 5, 49 and 60 months after initial surgery, respectively. A total of 19 sphincters remained in place (86.3% survival rate) with 5 revisions (26.3%) because of the pump (2), the cuff (2) or balloon fluid leakage. In this group 13 patients (68.4%) voided spontaneously and 6 (31.6%) performed clean intermittent catheterization, although 3 also voided spontaneously. Overall continence was good in 87% of patients because 2 were still incontinent at night. CONCLUSIONS: The artificial urinary sphincter is a good long-term solution to urinary incontinence secondary to sphincter incompetence despite multiple previous surgeries of the bladder neck or proximal urethra. Patients with bladder exstrophy and many previous bladder procedures are more exposed to complications such as erosion compared with patients with epispadias or anorectal malformation. The high percent of patients maintaining spontaneous voiding and the good rate of continence are the most important benefits of this type of surgical option for sphincter incompetence.
The outcome of 15 children and young adults who underwent reconstruction of continent urinary reservoirs (CUR) from January 1987 to 1990 is presented. Ten patients were male and 5 female with an age range of 3 to 20 years. There were 13 patients with bladder exstrophy and 2 with incontinent epispadias. In 8 cases the urinary diversion was performed for an inadequate bladder capacity following successful closure (3), failed attempted closure (3), female epispadias (1) and following successful urethroplasty in 1 case of male epispadias. A tiny fibrotic bladder plate unsuitable for attempted closure was the indication for diversion in 3 patients. Faecal and urinary incontinence following ureterosigmoidostomy (2 patients) and trigonosigmoidostomy (2) was the reason to consider re-diversion in 4 previously diverted patients. In 10 patients an Indiana pouch was performed. The Mitrofanoff procedure was used in 5 cases with caecum (2), sigmoid colon (2) or transverse colon (1) as the urinary reservoir. All patients are continent on clean intermittent catheterisation with stable renal function. Three patients developed large stones within the reservoir and needed open cystolithotomy. This series supports the efficacy of CUR as an alternative procedure to traditional forms of urinary diversion in the management of selected patients with exstrophy/epispadias complex.
Long-term results of genital function and cosmetic appearance were analyzed in 53 patients with bladder exstrophy. Satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 55 per cent of the patients. Normal erectile function was preserved in all patients and 61 per cent of the postpubertal patients had experienced satisfactory intercourse. The recent use of formal penile elongation by partial mobilization of the crura of the corpora cavernosa from the pubic rami combined with single-stage or multistage urethroplasty has produced a significant improvement in results. Of the patients 12 are married and 5 have fathered children.
The author reports on 15 patients with bladder-sigma anastomosis. The postoperative complications and late results up to a period of 10 years are discussed. Irreparable urethral strictures and bladder exstrophies are indications for this operation. It is only with reserve than it can be recommended for contracted bladder. According to the authors' experience and information by others, pyelonephritis and electrolyte disturbances seem to be rare complications. Because of the small number of cases a definite judgement of the method and its long-term consequences for the urinary system is not possible yet.
We present our experience in seven patients (three boys and four girls) with bladder augmentation. Two patients had a neurogenic bladder, two a "nonneurogenic" neurogenic bladder and three a bladder exstrophy. Between the exstrophy patients, two had a rectocistoplasty and one a colon conduit diversion. The rest of patients (two neurogenic bladders and two "nonneurogenic" neurogenic bladder) had a colon conduit diversion. The intestinal segment used for bladder augmentation were ileocecal sement (two patients), ileon (one patient) and colon sigmoid (four patients). Bladder augmentation were tubularized in four patients and detubularized in three. In the follow-up, five patients have a normal renal function (one transplanted), one patient his function is unchanged and other is waiting for the transplant. Of the seven patients, six are fully continente day and night (two with CI and one with anticholinergic drugs) and one has a diurnal continence no more than two hours.
A method for creation of an artificial bladder from the rectum, which was used in 22 children with exstrophy of the bladder with good anatomic and functional results is suggested. In clinical and histologic studies, the inferiority of the exstrophied bladder tissues and unexpediency of their use for plasty have been proved. A long-term result of treatment in 31 of the 35 patients operated on with the use of different methods was studied by the data of complex evaluation of the functions of an artificial bladder, upper urinary tracts and rectal sphincter apparatus.
PURPOSE: Exstrophy variants are very rare and have a better prognosis than classical exstrophy. The authors came across a case of superior vesical fissure (SVF) together with esophageal atresia and tracheoesophageal fistula (EATEF) and a case of SVF with gross limb anomalies. These associated malformations have not been reported so far in the literature and hence we reviewed all the cases of exstrophy variants presented to us with particular emphasis on the associated malformations. METHODS: Records (n=9) of patients who were diagnosed as exstrophy variants at our institution between 1989 and 2000 were evaluated retrospectively. RESULTS: Out of 9 cases, 7 cases had associated malformations: EATEF, urethral atresia, absent radius, large umbilical hernia, low anorectal malformation, true diphallus with bifid scrotum, or high anorectal malformation. CONCLUSION: The high incidence of associated congenital malformations, noted in our exstrophy variant series, raises doubts about the clubbing together of the exstrophy variants with classical exstrophy. Further investigation of such cases may elucidate shared or unique causes of the dysembryogenic mechanisms in the etiologies of variants of bladder exstrophy.
BACKGROUND: The term exstrophy-epispadias complex (EEC) has been coined for a group of congenital malformations that includes epispadias, bladder exstrophy and cloacal exstrophy. It is usually thought that these malformations develop against a similar embryological background. This background, however, is still obscure. This is mainly due to the lack of availability of abnormal human or non-human embryos showing the crucial developmental steps in the morphogenesis of EEC malformations. In this paper, we present chick embryos that show cloacal exstrophy at early developmental stages. To the best of our knowledge, this is the first documentation of this rare malformation in young embryos. MATERIALS AND METHODS: Embryos with cloacal exstrophy (n=4) were found among embryos from two experimental series (n=50) that were primarily performed to document the early morphogenesis of facial and cardiovascular malformations. The malformations were induced by the administration of suramin according to established protocols. Suramin can induce a spectrum of malformations including facial clefts, heart defects, and cloacal exstrophy. RESULTS AND CONCLUSIONS: Besides the presence of an abnormal opening into the cloaca, all embryos were characterised by an abnormal broadening of the caudal trunk at the level of the leg buds, which, in the youngest embryos, was associated with the abnormal presence of large aneurysmatic swellings of the dorsal aortae at this side. We postulate that these aneurysmatic swellings might be the primary defects leading to the development of EEC malformations. These space-occupying anomalies seem to cause abnormal distensions of the developing pelvis and of the infra-umbilical portion of the developing body wall. In consequence, the mid-portion of the developing ventral body wall between the origin of the umbilical cord and the cloacal plate becomes stretched and thinned out. Tension and thinning of the ventral body wall might ultimately lead to its rupture with exposure of the lumen of the embryonic cloaca and allantois. This new concept on the morphogenesis of the EEC is the first not to be inferred from the conditions seen in fetal or postnatal human cases but is based entirely on data from malformed embryos.
During the last 10 years 17 patients have been seen at this institution for persistent urinary incontinence after Young-Dees-Leadbetter bladder neck reconstruction. Of these patients 16 were born with classical bladder exstrophy and 1 with complete epispadias. Six patients underwent 1, 10 underwent 2 and 1 underwent 3 prior bladder neck procedures. As salvage procedures 8 patients underwent another Young-Dees-Leadbetter procedure, 1 repeat bladder neck reconstruction and augmentation cystoplasty, 3 augmentation alone, 4 bladder augmentation with creation of a continent abdominal stoma and 1 augmentation with implantation of an artificial urinary sphincter. Of the 8 patients who underwent a repeat Young-Dees-Leadbetter procedure 7 are dry for 3 hours or more and 1 is dry for greater than 3 hours on intermittent self-catheterization. All of those who are dry for greater than 3 hours are dry at night and 1 wears pads when engaging in strenuous physical activity. Of the 9 patients who underwent augmentation cystoplasty along with other adjunctive procedures 8 are continent for greater than 3 hours on intermittent catheterization, 6 are dry at night if they perform catheterization at bedtime and 1 remains totally incontinent after removal of the artificial urinary sphincter. Thus, with persistence and creativity a child with a previously failed bladder neck reconstruction or even multiple failed repairs can be made socially continent, providing a satisfactory alternative without resorting to urinary diversion.
By using a three-dimensional computed tomography (CT) scanner, we compared the anatomic features of the pelvis of three fetuses of same gestational age, one with a normal pelvis representing the reference model, one with classic bladder exstrophy, and one with cloacal exstrophy. The tomography slices were selected at the same levels for each case. Three angles expressing external opening of the pelvis were defined. Comparing normal and abnormal pelvises allowed definition of three criteria for the correction of the malformation: (a) the sum of the differential angles gives the amplitude of the correction needed; (b) a supraacetabular osteotomy appears to allow best closure of the pelvic ring; (c) only three slices of a CT scan are needed, which cannot be harmful, especially for neonates. Therefore, we believe that a CT scan of the pelvis should be performed whenever an osteotomy is planned in the surgical reconstruction of bladder and cloacal exstrophy.
Young-Dees-Leadbetter bladder neck reconstruction was performed in five females and two males with bladder exstrophy, two females and one male with epispadias, and two females with congenital short urethra. Eight patients had simultaneous augmentation colocystoplasty (7 exstrophy, 1 female epispadias), seven of whom are continent for three hours or more. Two patients void spontaneously and five are managed by clean intermittent catheterization (CIC) with which there were considerable problems in the beginning. Three of the four patients in whom simultaneous bladder augmentation was not undertaken remained incontinent because of small hypocompliant bladders. One has had a secondary augmentation cystoplasty and two may need it. Young-Dees-Leadbetter bladder neck reconstruction is a satisfactory operation for sphincteric urinary incontinence despite problems with CIC. However, bladder capacity may be considerably reduced after reconstruction. Augmentation cystoplasty should be considered not only in exstrophy patients undergoing bladder neck reconstruction but also in patients with other abnormalities.
Exstrophy of the bladder (EB) and exstrophy of the cloaca (EC) are generally recognizable as distinct clinical entities. In patients with EB, the posterior bladder wall is exposed through a midline defect of the abdomen. The umbilicus is inferiorly displaced and located close to the superior margin of the exstrophic bladder. Genital abnormalities are common in boys and girls who may present epispadias and a small, split phallus or a split clitoris, a bifid uterus, and a duplicate or exstrophic vagina. In contrast to classic EB, EC is commonly associated with omphalocele, spinal defects, and incompletely formed external genitalia and is always associated with imperforate anus. Some authors state that EC and EB constitute two distinct disorders, but others consider them part of a "continuum," representing different levels of severity within the same spectrum. The use of the acronym OEIS to refer to the combination of omphalocele, exstrophy, imperforate anus, and spinal defects, in our opinion, has not helped to clarify the clinical definition, pathogenesis, or cause of this multiple congenital anomaly (MCA) pattern, mostly because the term makes no distinction between EC or EB. Here we present the epidemiological analysis of a group of characteristics in infants with EC and infants with EB to determine if they constitute two different entities. We also analyze if the different combinations of omphalocele, imperforate anus, and spinal defects are more frequent in infants with EC than in infants with MCA patterns other than EC and EB. The prevalence in our data for EC was 1:200,233 live births and 1:35,597 for EB. The clinical analysis indicated that the study defects (omphalocele, spine defects, spina bifida, and imperforate anus) tend to occur together in the same child with a higher frequency if the child has the EC defect than in infants with MCA patterns that did not include EC or EB. Our findings of low birth weight, twinning, single umbilical artery, and preferentially associated malformations suggest that EC is the result of damage occurring very early in development and that EC and EB are two different expressions of a primary polytopic developmental field defect.
OBJECTIVE: To report our experience of the use of desmopressin to improve nocturnal dryness in patients who have undergone a staged reconstruction of the exstrophy-epispadias complex (EEC), who although continent by day, have nocturnal incontinence because their nocturnal urinary output exceeds their bladder capacity. PATIENTS AND METHODS: Seven children (aged 8-12 years) who had undergone a staged reconstruction for EEC (six with classical bladder exstrophy, one with incontinent epispadias) were treated with intranasal desmopressin for persistent nocturnal incontinence despite daytime dryness. Previous additional procedures for continence had been self-augmentation in one and periurethral collagen injection in three others. The criteria for inclusion in the study were: normal renal function, no upper tract deterioration, no urinary tract infections, spontaneous voiding during the day with dry intervals between micturitions, a postvoid residual volume of <10% of bladder capacity and night-time incontinence for 7 nights/week. Desmopressin was administered at bedtime at increasing dosages from 10 to 30 microg until effective. Body weight, arterial blood pressure, and serum electrolytes were measured, and all patients assessed using renal ultrasonography, a voiding diary and a nocturnal pad-test. RESULTS: Desmopressin at doses of 10-30 microg was successful in keeping all the patients dry. The nocturnal urinary output was decreased so that it did not exceed bladder capacity. There was only one minor side-effect (nose bleeding). CONCLUSIONS: In selected patients with EEC, desmopressin is effective in improving nocturnal dryness, with no significant side-effects.
PURPOSE: The management and reconstruction of the abnormal bladder have made great strides in the last 20 years. Long-term experience comprises patients born more than 20 years ago whose treatment may have been less satisfactory than that available today. The effects of puberty and adolescence on the abnormal bladder and its management in childhood are reviewed. MATERIALS AND METHODS: The current literature on long-term followup is reviewed, and adolescent urology clinic database from 1981 to 1999 is analyzed. RESULTS: In patients with bladder symptoms but no demonstrable abnormality, such as monosymptomatic enuresis, there is usually improvement with time. In the neuropathic bladder function and opportunities for surgery deteriorate especially after puberty. It is essential that proper management be established in childhood. The bladder of a boy with posterior urethral valves also deteriorates with time but in an unpredictable manner (60% develop high pressure and 40% become hypotonic). Constant followup must be done even in patients who are symptomless. The long-term fate of the exstrophy bladder is particularly hard to determine because techniques of reconstruction have changed so dramatically in the last 20 years. In the short term it would seem that urodynamic abnormalities are seen in about 25% of patients, even in those who are continent. Those who have a working bladder at age 10 years have only a 23% chance of having the same by age 20 years. In patients with renal failure due to lower tract dysfunction the bladder must have low pressure and completely empty (with clean intermittent catheterization if necessary) before a transplant can be considered. CONCLUSIONS: The bladder that is abnormal in childhood undergoes changes at puberty that alter storage and voiding function. When the original abnormality is minor, the change may lead to functional improvement. In the severe anomalies changes are almost invariably for the worse and may precipitate renal failure. Children with significant bladder dysfunction will seldom grow out of it.
Genital anomalies associated with exstrophic deformities are mainly caused by the wedge effect of the cloacal membrane. The more primitive the exstrophy the more severe are the genital defects. With exstrophy of the cloaca genital tract invariable. The main practical problem relates to the male subject with epispadias alone or in association with bladder exstrophy. Correction of the penile deformity requires release of the chordee by freeing the short urethra from the corpora cavernosa and lengthening the organ by partial separation of the crura from the bony rami. The possession of an adequate phallus is generally the prime consideration of the patient and his parents, and surgical reconstruction should aim to provide maximal correction of the anomalies at an early age.