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PubMedDate not supplied

Rotational profile of lower extremities in bladder exstrophy patients with unapproximated pelvis: a clinical and radiologic study in children older than 7 years.

Fourteen patients (nine boys, five girls) with bladder exstrophy were analyzed radiologically and clinically. All were older than 7 years and had a pubic diastasis >2 cm. Anteroposterior and lateral center-edge angles were measured by direct radiography. Acetabular version, femoral anteversion, tibial torsion angles, and patellofemoral congruency angle were measured by computed tomography imaging. All were active with regard to their daily life and sports activities. The average foot-progression angle was +8 degrees . Spherical congruency was present in all hips, and none showed dysplasia. The average angle of acetabular version was apparently less than normal, but femoral anteversion angles were found to be increased. Increased external tibial torsion was observed in all patients. Twelve (71%) of 14 patients had positive congruence angles, the average being +6.1 degrees . Two patients had subjective complaints of patellofemoral instability. Increased femoral anteversion and external tibial torsion may lead to patellofemoral instability, and the bladder exstrophy patients should be followed up regarding this problem as well.

Adolescent↗

PubMed1993

Techniques to create continence in the failed bladder exstrophy closure patient.

We reviewed retrospectively 315 patients with bladder exstrophy treated at our hospital between July 1976 and April 1992 to assess the outcome of those who failed primary closure of the bladder. Of the patients 47 required reclosure of the bladder, including 28 who have undergone a procedure to restore urinary continence. Methods used to achieve dryness included bladder neck reconstruction in 18 patients, bladder neck reconstruction along with augmentation in 4, augmentation alone in 4, repeat bladder neck reconstruction in 1, and reclosure with creation of a continent stoma and augmentation in 1. Nine of 18 patients who underwent primary bladder neck reconstruction are dry on intermittent catheterization, while 8 of the remaining 9 are dry and voiding without catheterization. Four patients who underwent primary bladder neck reconstruction and augmentation, and 4 who underwent augmentation after bladder neck reconstruction are dry on intermittent catheterization. The patient who underwent reclosure, bladder augmentation and creation of a continent abdominal stoma is dry on intermittent catheterization. Virtually all patients who failed the initial closure and later bladder neck reconstruction for continence require augmentation and intermittent catheterization to remain dry. Of 28 patients who underwent salvage procedures only 1 had upper tract changes. With attention to detail and the use of a variety of reconstructive techniques children who have failed exstrophy closure can achieve continence and have stable renal function.

Bladder Exstrophy↗

PubMed1986

Ureterosigmoidostomy and bladder exstrophy: a long-term followup.

Of 158 patients who underwent ureterosigmoidostomy for exstrophy of the bladder from 1925 to 1970, 52 were available for followup by telephone interview. Of these patients 34 still had a functioning ureterosigmoidostomy (30 had complete continence day and night) but 18 required diversion by other methods. All of the patients were believed to be socially well adjusted and leading productive lives. Of the patients surviving 15 or more years after ureterosigmoidostomy 11 per cent had colon cancer. Most of the remaining patients were unaware of the risk of adenocarcinoma engendered by the ureterosigmoidostomy. Patients undergoing ureterosigmoidostomy must be fully informed of all health risks and adequate surveillance programs should be arranged. In selected patients ureterosigmoidostomy remains a useful form of urinary diversion, with excellent continence and good social adaptation.

Adolescent↗

PubMed1991

[Genital prognosis of girls with bladder exstrophy or epispadias].

The authors report ten cases of women with previous exstrophy of the bladder or epispadias with incontinence followed up to child-bearing age. Six of the ten females had urinary diversion and four retained their bladder and were continent. Three key aspects are considered: physical appearance of the external genitalia which was satisfactory in all cases; preservation of sexual function, known in only four of the ten cases and which was reported by these patients to be satisfactory, and finally, the ability to bear children. Three patients became pregnant resulting in four normal births (one patient had twins). Cesarean sections were recommended in patients with pregnancies at term. Cesarean should be performed systematically in patients with bladder reconstruction.

Adolescent↗

PubMedDate not supplied

Medicinal leeches in the postoperative care of bladder exstrophy.

This article reports the use of medicinal leech therapy for the relief of severe postoperative vascular congestion of the penis in a male infant with exstrophy of the bladder. When more conventional methods of decongestion were unsuccessful, medicinal leech therapy markedly improved the infant's chance of remaining both phenotypically and functionally male. The uneventful and successful use of medicinal leeches in this patient demonstrates that medicinal leeches may be safely used in the newborn period, if appropriate precautions are taken to prevent both infection and anemia.

Animals↗

PubMedDate not supplied

[A new method of ureter-sigmoid anastomosis in bladder exstrophy].

A new ureterosigmoidostomy (USS) procedure in exstrophy of the bladder in children comprises establishing a long submucous channel (SC) in the upper or middle third of the sigmoid. This is done by tenial, atraumatic for the mucosa, cut of the sigmoid wall followed by mucosal dissection along the length 2-3 mm larger than the diameter of previously immobilized ureter. After that a special instrument creates upwards an oblique SC, the ureter is placed into the SC opening made in the wall musculoserous layer. Finally, the shunt is established. The same USS procedure was conducted on the other ureter 3-4 cm above the previous shunt. The openings are intubated. Follow-up conducted for 6 years at best showed a complete urine retention existence in 7, a partial one in 1 cases. Radiologically, renal function improved in all the patients. Neither enteropelvic reflex, nor urolithiasis were observed.

Adolescent↗

PubMedDate not supplied

[Plastic repair of the anterior abdominal wall in bladder exstrophy].

In 1987-1993 surgical treatment for exstrophy of the bladder was performed in 35 children at the age 8 months--3 years. All the patients underwent one-stage transplantation of the ureters into the semi-isolated segment of the sigmoid colon with antireflux protection of the ostia, bladder extirpation and repair of the anterior abdominal wall. The most difficult stage of the intervention is repair of the abdominal wall defect after removal of the large bladder. Related complications arising in 16% of the surgical patients impose the problem of the technique of safe filling of the abdominal wall defect. In 16 patients with large bladder its demucosation was followed by strengthening of the detrusor muscles with the help of interrupted sutures with fixation to underdeveloped oblique abdominal muscles. The skin defects was filled by raised movable skin flaps on both sides of the divided pubis. The above technique reduced the number of postoperative complications three times. Intestinal eventration was not observed. The wound suppuration and partial defect of the sutures occurred in 1 case. The rest 15 children exhibited healing with minimal scarring and good cosmetic effect.

Abdominal Muscles↗

PubMed1992

Bladder exstrophy and anterior pelvic osteotomy.

Between August 1988 and December 1991, 36 children with bladder exstrophy underwent surgery for primary bladder reconstruction. Each child was either untreated or had already been treated unsuccessfully. The operative technique involved bilateral osteotomy of the superior ramus of the pubic bone. In infants the cartilaginous ischiopubic junction, acting as an articulation, allowed symphyseal approximation, while in older children this was achieved by fracture of the inferior ramus of the pubic bone. The bladder was either closed or, in most cases, the exstrophic bladder plate was inserted deep into the pelvis, allowing subsequent epithelialisation of the bladder and further formation and growth. Follow-up up for 3.5 years showed bladder capacities of 40 to 150 ml. Some patients underwent an additional augmentation enterocystoplasty. Primary bladder reconstruction remained uncompromised in 7 patients who developed moderate (and 1 complete) rediastasis of the pubic bones. All exstrophic bladders are reconstructible, particularly in older children.

Bladder Exstrophy↗

PubMed1996

The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder?

OBJECTIVES: To define the possible cause of failure and the eventual potential of the bladder in 23 exstrophy patients, who underwent more than two failed prior attempts at closure. METHODS: Twenty-three patients were selected from the exstrophy data base who had two or more prior closures. Eighteen patients had undergone 2 previous closures and 5 patients 3 previous closures for either complete dehiscence or significant prolapse. At the time of initial closure, 19 patients did not have an osteotomy. At secondary closure, 10 underwent osteotomy while at third closure 5 had an osteotomy. At the time of reclosure at our institution all underwent an osteotomy. RESULTS: Reoperative repair at our institution was successful in all patients. Six patients achieved a bladder size suitable for bladder neck reconstruction and of them 3 are dry. The bladder size was inadequate in 9 patients and 8 are being monitored for possible bladder growth. CONCLUSIONS: Tension-free closure with osteotomy and immobilization are important factors both in an initial or any subsequent closure. The chance of obtaining an adequate bladder capacity for bladder neck plasty and eventual continence, following multiple reclosures, is markedly diminished.

Bladder Exstrophy↗

PubMedDate not supplied

Three-dimensional anatomy of the pelvis in bladder exstrophy: description of bone pathology by using three-dimensional computed tomography and its clinical relevance.

The alterations in pelvic structure that occur in bladder exstrophy were evaluated in six children (one girl and five boys) by using three-dimensional computed tomography (3D-CT). The length and angle of anterior and posterior segments and the slanting angle of iliac wings were measured. 3D-CT scans of the pelvis of four patients with a normal pelvis were obtained to serve as controls. Both anterior and posterior segments were abnormally rotated externally in patients with bladder exstrophy. Whereas the length of the posterior segment is normal, the length of the anterior segment is significantly shorter, and the iliac wings projected more inward than those in the age-matched control subjects.

Adolescent↗

PubMed1988

Functional reconstruction of exstrophied bladder. Timing and technique. Follow-up of 39 cases.

Thirty-nine cases of functional reconstruction of exstrophied bladder are reviewed. There were 20 females and 19 males. In a first group of 31 staging was planned and carried out in 26 while 3 are awaiting a second stage. In a second group of 8 a single operation was performed. Out of the 31 early cases there were 2 deaths and 10 failures, 16 show good or very good functional result. All 16 show moderate renal scarring from reflux pyelonephritis which occurred between I and II stage. Single operation consisted of innominate osteotomy, bladder and bladder neck and urethral reconstruction and anti-reflux procedure (osteotomy was omitted in a 3 days old baby). Three, operated upon when aged 8 mths., 1 year and 4 years, were breakdowns of previous closure. They are incontinent and will need further surgery at the bladder outlet. Four are dry in the morning and suffer from occasional stress incontinence and enuresis. One, now aged 4 years, still wears pads. These last 5 are awaiting final assessment of and eventually further surgery to improve continence. In all 8 cases pyelo-calyceal cavities are normal except for a moderate right dilatation in one because of kinking of the reimplanted ureter. Delay in bladder closure (mean age 5 mths.) and severe changes to the bladder wall and possibly staging seems to be responsible for most of the failures and for renal damage occurring, after closure, in bladders showing moderate compliance. Single operation allows full protection of upper tract and kidney and should be preferred in patients aged over 2 months. Better functional results may be obtained if operation is performed soon after birth.

Adolescent↗

PubMed1999

[Long-term follow-up of a bladder exstrophy case after successful repair].

We present a female case of bladder exstrophy where the patient was followed up for 15 years. In 1982 primary closure of the bladder and urethra with bilateral iliotomy by the posterior approach was performed at the age of 4 months. However, realignment was necessary, since the wound split open postoperatively. We used a corset specially prepared for this patient to prevent wound dehiscence during the subsequent postoperative course. Four years later, VUR was surgically eradicated. At the age of 9 years old, her bladder function was satisfactory, demonstrating a normal CM pattern with synergia and Pmax 128 cm H2O on UPP. She had no urge or stress incontinence. The most recent IVP and DMSA renal scan revealed almost normal findings. VCUG showed no VUR and renal function tests (PSP, Ccr 24) also confirmed no interval deterioration. We believe that the approximation of the intersymphyseal band at the time of bladder neck closure is the single most important factor for ensuring urinary continence.

Bladder Exstrophy↗

PubMed1985

Urethral duplication and complete bladder exstrophy.

Urethral duplication is a rare anomaly that has been classified into epispadiac, hypospadiac, spindle, Y-duplications and collateral varieties. We report a case of an accessory urethra associated with complete bladder exstrophy in which the accessory urethra was integrated into the epispadias repair. To our knowledge this is the first case reported of urethral duplication associated with bladder exstrophy.

Abnormalities, Multiple↗

PubMed1999

The Mainz II pouch: experience in 5 patients with bladder exstrophy.

PURPOSE: We report our experience with 5 patients with bladder exstrophy who underwent creation of a Mainz II pouch. MATERIALS AND METHODS: We retrospectively reviewed the results of the Mainz II pouch as a primary urinary diversion in 2 and a secondary urinary diversion in 3 patients. Each patient underwent multiple surgeries, including osteotomy in 1. All patients were followed postoperatively on a yearly basis. RESULTS: All patients are continent and the upper urinary tract is stable. CONCLUSIONS: The Mainz II pouch is appropriate for children born with a small fibrotic bladder, and as a salvage procedure for those who have endured multiple reconstructive procedures and remain incontinent. Furthermore, this procedure deserves serious consideration in children residing in developing countries.

Adult↗

PubMed1995

[Reconstructive-plastic surgery in bladder exstrophy].

Among 234 children with exstrophy of the urinary bladder 112 were subjected to reconstructive-plastic operation after G. A. Pair. A good result was produced in 71 cases. In 27 children a satisfactory result was attained only after repeated operations. Reconstructive-plastic interventions had no positive effect in 14 children. By means of electrophysiological and morphological methods three degrees of underdevelopment of the muscular coat of an urinary bladder affected by exstrophy were distinguished. In I degree plastics of the bladder with local tissues is indicated, in II degree the indications are relative, in III degree a plastic operation is not indicated. Choice of the operative method on basis of the morphofunctional condition of the bladder with exstrophy, the use of microsurgical techniques, the formation of a closed pelvic ring, and antireflux operations on the ureters improve the immediate and late-term results of reconstructive-restorative surgical interventions.

Age Factors↗

PubMed1992

Results of surgical treatment in children with bladder exstrophy.

A series of 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy between 1972 and 1989. Primary bladder closure was performed in 11 patients and was successful in 6. Four of these children are about 2 years old and so it is not yet possible to assess their continence. Ureterosigmoidostomy was performed in 35 children, one of whom was converted to cutaneous ureterostomy. Follow-up ranges from 2 to 20 years (average 9 years 3 months) and 50% of the patients are symptom-free. The most frequent problems were acidosis and urinary tract dilatation. Other complications, such as hypokalaemia and pyelonephritis, were seldom seen. Although ureterosigmoidostomy has some disadvantages, our patients have adapted well and lead a normal life.

Adolescent↗

PubMed1991

Surgical management of incontinence in bladder exstrophy.

Between 1978 and 1990, 86 patients with previously closed classical bladder exstrophy and 10 patients seeking undiversion have presented for continence management and have undergone selective reconstruction designed for voiding and/or intermittent urethral catheterisation. The reconstruction in these 96 patients has been reviewed. Eight of the 10 patients undergoing undiversion achieved a satisfactory state of continence but 4 required Mitrofanoff procedures to enable catheterisation. Of the other 86 patients, 2 reached a satisfactory state of continence without further surgery; 79 underwent bladder neck surgery for continence either without augmentation (n = 32) or with augmentation (n = 47). Twenty of the 32 patients who were treated by bladder neck reconstruction alone were later found to require augmentation. Five patients had very early augmentation either to facilitate neonatal closure or on account of severe upper tract dilatation. Of these, 1 became continent without further surgery and 4 demonstrated the need for bladder neck reconstruction. Thus 12 children achieved successful continence (n = 6) or are evolving satisfactorily with potential success (n = 6) as a result of bladder neck reconstruction. Of the 71 patients requiring bladder neck reconstruction and augmentation, 68 have completed their surgery. The current status of these patients is: satisfactory in 57 (80%) (42 void/urethral clean intermittent catheterisation (CIC), 7 waiting to learn CIC, 5 Mitrofanoff, 3 artificial urinary sphincter (AUS]. Of the remaining 11 patients (20%), 8 are unsatisfactory to varying degrees and the status of the other 3 is unknown.

Bladder Exstrophy↗

PubMed1977

Variations in the bladder exstrophy complex associated with large omphalocele.

Two newborns with a large omphalocele associated with the bladder exstrophy complex are presented. In 1 case the omphalocele was combined with a musculoskeletal defect and imperforate anus with an anovulvar fistula. The other patient had a musculoskeletal deformity and a duplicated bladder with exstrophy. Surgical treatment was successful in both cases.

Bladder Exstrophy↗