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Developmental defects of the urinary bladder and abdominal wall

Publication at Second Faculty of Medicine |


Objective: The aim is to evaluate the results of treatment of patients with penopubic epispadias, exstrophy of the urinary bladder and cloacal exstrophy. Material and methods: Between 1994-2013 there were 3 patients with epispadias, 25 with bladder exstrophy and 6 with cloacal exstrophy operated on at our institution. 94 surgical procedures were performed.

Reconstruction of the urinary bladder was done 24-48 hours postnatally, 3x with pelvic osteotomy. In cloacal exstrophy the bladder was reconstructed from two hemibladders together with omphalocele plasty and colostomy of the large intestine stump.

Reconstruction of the epispadiac urethra was performed in toddler age. 3rd phase of reconstruction was shifted to prepubertal and pubertal age with reimplantation of the ureters to eliminate vesicoureteral reflux and Young-Dees-Leadbetter procedure to increase bladder outlet resistance so as to achieve socially acceptable state of continence. Results: We have faced following complications: one death of intractable sepsis in a child with cloacal exstrophy and other associated developmental defects.

One decreased function on the kidney on renal scan (30%) despite succesfull surgical correction of VUR. One recurrent bladder dehiscence + pyohydronephrosis bilaterally.

One mechanical intestinal obstruction in a girl with cloacal exstrophy and duplex uterus the right one with dystopic ureter. Five times urethrocutaneous fistula in the bladder neck.

Conclusion: Bladder reconstruction is technically possible even with very small bladder targets. The bladder tends to expand and enlarge its capacity where the initial capacity was a few cc., despite initial incontinence.

Socially acceptable continence has been reached in our cohort of patients in 40% of cases with bladder exstrophy. In case of permanent incontinence the solution is bladder augmentation and catheterisable stoma.

Good preservation of renal function is the rule, fertility is rather exceptional.