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Horizontal pelvic osteotomies for bladder exstrophy. A preliminary report.

We have treated 11 patients aged three days to 15 years with bladder exstrophy by horizontal osteotomies of the innominate bones. The operation was originally used for older patients with severe deformity or failed previous surgery but is now applied as a primary procedure in the first week of life. The osteotomies enable the complex malformations to be corrected in a single operation without turning the patient: the pubic bones can be brought together, the abdominal wall repaired and the bladder closed with reconstruction of the urethra and external genitalia. The early results have been very satisfactory in all cases with only minor complications; we felt that a preliminary report should be made, despite a mean follow-up of only seven months.

Adolescent↗

Long-term results of surgical treatment of bladder exstrophy.

A review of the long-term results of the treatment of 18 cases of bladder exstrophy is reported. Five cases have been operated on before 1961 by ureterosigmoidostomy; 4 (80%) patients have good renal function and have not had any complications, although 1 patient has anal incontinence; 1 patient presented bilateral renoureteral complications, requiring several corrective operations, and has anal incontinence too. Out of 13 cases operated on after 1961, 10 have been followed up from 3 to 14 years. In 1 patient an ileal conduit was performed because of the failure of an ureterosigmoidostomy performed at another hospital 10 years before. In 9 patients a personal technique based on the bladder substitution by a colonic loop plus creation of a sphincteric mechanism by trigonal tubulization was used (enterotrigonal urethroplasty). Morbidity has been high (66.6%), but the end results are very satisfactory: 7 patients (77.7%) are currently living a normal life, with normal renal function and good continence; 1 patient is incontinent and has bilateral reflux and a left retracted kidney; 1 patient has been secondarily diverted by means of a cutaneous Y transuretero-ureterostomy due to gross lreteral dilatation, renal damage and incontinence.

Adolescent↗

Ureterosigmoidostomy in bladder exstrophy.

Experience with ureterosigmoidostomy (US) using a reflux prevention technique in 38 of 48 cases of bladder exstrophy is reviewed. During a follow-up period of 14 years, the results were excellent as to morphological changes of the upper urinary tract, incontinence, psychological development and social behavior. Within their families the patients are considered healthy. Even in children with benign disease, US with reflux prevention performed during the second year of life is the operation of choice for urinary diversion. In the postoperative follow-up it is recommended that sigmoidoscopy be performed once a year in addition to routine controls of the upper urinary tract and metabolic balance.

Adolescent↗

Nephrogenic adenoma with bladder exstrophy and immunosuppression.

Nephrogenic adenoma, an unusual proliferative lesion, often occurs in association with chronic inflammation of the bladder and is rarely found in children. Two cases are reported, one occurring in a young child born with bladder exstrophy, and one in a young female, with both interstitial cystitis and chronic use of immunosuppressive therapy. We believe these are the first case reports of nephrogenic adenoma occurring in this specific clinical setting.

Adenoma↗

Pregnancy in women after repair of bladder exstrophy. Two case reports.

Improved techniques in urinary diversion operations have enabled a large number of patients with bladder exstrophy to achieve urinary control. They can live fairly normal lives and some have achieved pregnancy. Case reports of such exist in the literature. Two more cases are reported in this paper, the pregnancies and their outcome are discussed.

Adult↗

[One stage reconstruction of bladder exstrophy and epispadias with abdominal wall skin flap and rectus abdominis muscle flap: report of 5 cases].

5 cases of complete bladder exstrophy were treated by one stage reconstruction with abdominal wall skin flap and rectus abdominis muscle flap. Closure of the abdominal wall and bladder defect were achieved satisfactorily in all the patients. 4 patients have been followed up for 1.5-10.5 years. 3 patients whose bladder neck had been augmented by rectus abdominis muscle flap were continent but 1 patient whose bladder neck had not been, had giggle incontinence. The renal functions were all normal in these patients.

Adult↗

Primary closure of bladder exstrophy: long-term functional results in 137 patients.

Between 1945 and 1985, 207 patients were treated at our institution for exstrophy of the urinary bladder. Primary anatomical bladder closure was performed in 137 patients. In 97 patients treated prior to 1975, bladder closure was performed at a median age of 1 year and continence in this group was only 16%. Forty patients treated after 1975 underwent closure at a median age of 72 hours followed by staged reconstruction of the bladder neck. Continence in this group was 82%; however, the average number of surgical procedures was five as compared with two in the first group. Early bladder closure and staged reconstruction can achieve acceptable urinary continence, but multiple surgical procedures may be required.

Bladder Exstrophy↗

Epidermolysis bullosa junctionalis associated with urinary bladder exstrophy: a case report.

We report the second infant of nonconsanguineous parents with epidermolysis bullosa junctionalis associated with urinary bladder exstrophy, epispadias, anteriorized anus, and bilateral inguinal hernias. The family history also included the death of a maternal cousin due to epidermolysis bullosa. Our diagnosis was based on electron microscopy and immunofluorescence evidence. This patient is reported because of the rarity of this constellation of findings.

Abnormalities, Multiple↗

[Experience with reconstructive-plastic operations in bladder exstrophy in children].

The author analyzes the experience with reconstructive-plastic operations in her modification mad in 22 children with exstrophy of the urinary bladder. Long-term results within 3-17 years were observed in 15 children. Good outcomes (complete retaining of urine, normal capacity of the bladder, good morphofunctional state of the kidneys) were noted in 8 children. Based on the results obtained the author recommends the surgical treatment of exstrophy of the urinary bladder to be started with reconstructive-plastic operations at the age of 1-3 years.

Bladder Exstrophy↗

Results of umbilicoplasty for bladder exstrophy.

PURPOSE: The umbilicus is an important aesthetic landmark and its absence or deformity may be associated with poor self-image. In patients born with bladder exstrophy the umbilicus is attached to the upper margin of the bladder and reconstructive surgery often removes the navel. The umbilicus marks the waistline and serves to complete the harmony of the curved lines above and below the waist. We present our experience with children born with exstrophic anomalies during the last 2 decades. MATERIALS AND METHODS: Our database included 61 children born with classic bladder and 8 born with cloacal exstrophy treated between 1980 and 1998. We performed primary reconstruction in 35 children, while 34 children and young adults were referred for secondary surgical repair, including bladder augmentation, continent diversion, genitoplasty and so forth. Neoumbilicoplasty was done in all of the former and in 30 of the 34 latter cases. Early in the series a V-shaped flap was raised and buried subcutaneously. The flap eventually became a tube around the cystotomy tube and the cicatrix formed the umbilical dimple. This method necessitated packing with iodoform gauze for 4 weeks with weekly dressing. The technique evolved into a tubularized U-shaped flap. A rubber tube was placed indwelling as a stent to maintain inward projection of the neoumbilicus. RESULTS: In 66 of the 69 cases the early results of umbilicoplasty were described by the surgeon as excellent or satisfactory. In 3 cases the neoumbilicus appeared flat, lost depth and was described as unsatisfactory. Long-term followup of more than 1 year was available in 48 patients, of whom 2 underwent umbilical repositioning for an off center or low umbilicus and 3 underwent repeat umbilicoplasty for a flat umbilicus that had lost depth. The best cosmetic results were achieved in patients with a relatively thick layer of subcutaneous fat, whereas cosmesis was suboptimal in thin children. Nevertheless, the patients and parents were generally pleased with the umbilical appearance even when the surgeon was not. CONCLUSIONS: Although the navel is a functionless depressed scar, it represents an important and pleasing landmark. Umbilical construction should be attempted early during functional closure or urinary diversion.

Adolescent↗