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Duplicated exstrophy in one of identical twins.

The characteristic of duplicated exstrophy is the presence of a patch of exstrophic bladder mucosa in the infraumbilical region with a low-set umbilicus, in addition to the normal bladder. Musculoskeletal defects, diastasis pubis, and rectus abdominis can also be found in these patients. We report the first case of a female twin baby with appearance of a low-set umbilicus and diastasis pubis associated with a pubic sinus. The external genitalia and urinary continence were normal. Duplicated exstrophic mucosal remnant was excised, and the histopathologic study of the specimen confirmed urothelium.

Abnormalities, Multiple↗

The fate of the upper urinary tract in exstrophy.

PURPOSE: We evaluated the impact of reconstructive bladder procedures on the upper urinary tract in bladder/cloacal exstrophy. MATERIALS AND METHODS: A total of 57 patients underwent bladder neck procedures for urinary incontinence due to bladder exstrophy between 1988 and 2001. We correlated evidence of renal injury to urinary continence and type of surgery. Renal scarring and moderate/severe hydronephrosis were considered significant upper tract changes. RESULTS: Overall 27 patients (47%) underwent bladder neck closure, 38 (67%) augmentation cystoplasty and 44 (77%) a Mitrofanoff procedure. Mean interval since last continence procedure was 4.5 years and mean patient age at last followup was 11.7 years. Hydronephrosis was noted in 18 patients (32%) at last followup (after bladder neck reconstruction in 15). Renal scarring in 14 cases on dimercapto-succinic acid scan was evenly distributed regardless of the type of bladder neck procedure or presence of augmentation. Of 6 patients with grade 3 to 4 hydronephrosis 5 had renal scarring versus only 1 in the 39 without hydronephrosis (p <0.0001). Serum creatinine remained normal in 55 patients, mild renal insufficiency developed in 1 and renal transplantation was performed in 1. Two patients with persistent incontinence are anticipating an augmentation. CONCLUSIONS: Of our 57 patients with exstrophy 14 (24%) had significant upper tract damage in the form of renal scarring and/or moderate or severe hydronephrosis. The surgical method of achieving continence was not predictive of hydronephrosis or scarring. Bladder neck closure appears to provide better urinary continence and was equally safe with respect to hydronephrosis or scarring compared to patent forms of bladder neck reconstruction.

Bladder Exstrophy↗

Transurethral bladder eversion concurrent with uterovaginal prolapse.

BACKGROUND: Transurethral bladder eversion is rare. A case of transurethral bladder eversion with concurrent uterovaginal prolapse is presented. CASE: The patient was postmenopausal, had a long history of uterovaginal prolapse, and developed bladder eversion. Examination under anesthesia revealed full-thickness transurethral bladder eversion and complete uterine procidentia. Temporary reduction of the prolapse was done initially because of her unstable medical condition. After medical stabilization, definitive surgical reconstruction was completed by a vaginal approach. CONCLUSION: Full-thickness, transurethral bladder eversion can be surgically treated by a vaginal approach.

Bladder Exstrophy↗

A pseudoexstrophy with penile anomaly.

Although classical exstrophy of the bladder is not a rare anomaly, variants of exstrophy are extremely uncommon. We report a male pseudoexstrophy case with appearance of a low set umbilicus associated with umbilical hernia and penile anomaly. Although this mild exstrophy variant does not normally require surgical management, penile reconstruction was necessary in our patient.

Abnormalities, Multiple↗