Search

The reasons for referral and treatment strategies in patients who underwent complete primary repair of bladder exstrophy elsewhere in the newborn period were evaluated.An institutionally approved database identified patients who underwent complete primary repair of exstrophy and were subsequently referred for continued care.A total of 10 females and 55 males were referred for treatment between 1996 and 2010. Six females and 23 males were referred for complications following initial complete primary repair of exstrophy. Female complications included dehiscence (3 patients), prolapse (2) and stricture (1). Male complications included dehiscence (10 patients), prolapse (9), pubic separation (1) and stricture (3). Nine males had posterior urethral loss and 13 had major penile soft tissue injuries. Grade V vesicoureteral reflux and severe hydronephrosis were seen in the 4 patients with urethral strictures. Patients with dehiscence or prolapse underwent successful reclosure with osteotomy. Cases with posterior urethral loss or strictures were repaired with grafts. Four females and 32 males were referred for incontinence. Nine of these patients had poor bladder capacity, of whom 5 underwent bladder augmentation with continent stoma and 4 are awaiting further bladder growth. A total of 27 patients underwent bladder neck repair, with 15 (56%) subsequently continent, 5 (19%) daytime continent with nocturnal incontinence and 7 (26%) continuously incontinent. At initial closure osteotomy had been performed in all patients who were continent following bladder neck reconstruction and in 4 of 5 who were daytime continent.Complications of complete primary repair of bladder exstrophy can result in undesired upper urinary tract changes and penile soft tissue loss. Surgical reconstruction of such complications and acceptable continence are attainable in select cases.

Left tibial agenesis, polysyndactyly with talipes equinovarus deformity and Grade IV vesicoureteral reflux of the right kidney are described in a 40-day-old male and an unrelated 1-month-old male, is also reported with right tibial agenesis, polysyndactyly with talipes equinovarus deformity and right kidney agenesis and left Grade V vesicoureteral reflux. No other pathology was recorded. Follow up at 1 year and 3 years, respectively, revealed normal motor and mental development. As this combination has been unpublished before, we believe that this a new syndrome.

Augmentation cystoplasty is a commonly used method to treat patients with inadequate bladder capacity. Calculus formation within the altered urinary tract has been a frequent complication. We report the unique finding of calculi within a retained ureteral stump in a patient who had undergone enterocystoplasty. CASE REPORT A 29-year-old female presented with recurrent urinary tract infections that were refractory to treatment. The patient had been born with sacral agenesis and imperforate anus. Neurogenic bladder and vesicoureteral reflux had subsequently developed, and the patient underwent ileal conduit formation for management of progressive bilateral hydroureteronephrosis as a young child. At age 15 years she underwent urinary undiversion with ileocecoplasty to the native bladder. Videourodynamics were repeated at age 28 years and revealed a volume capacity of 700 ml., and bilateral grade V vesicoureteral reflux and reflux into the retained ureteral stumps. The patient performed clean intermittent catheterization every 2 to 4 hours to minimize the post-void residual urine volume. Recurrent urinary tract infections were managed with outpatient oral antibiotic therapy. In addition, in 1999 a bladder calculus had developed in the bowel segment of the bladder. The stone was managed with cystolithotripsy, with complete stone removal. In 2001 urinary tract infections recurred despite appropriate antibiotic coverage for each recurrent infection and antibiotic prophylaxis. Symptoms consisted of painful urethral catheterizations, small urethral catheterized volumes and pyuria. Plain x-ray of the kidneys, ureters and bladder demonstrated multiple calcified densities in the pelvis (see figure). In preparation for cystolithotripsy, cystoscopy of the native and augmented bladder was performed, which showed an absence of bladder calculi. However, a dilated and patulous right ureteral orifice was identified. Retrograde ureterogram revealed several filling defects in the right ureteral stump that correlated with the calcifications seen on x-ray. The ureteral stump measured approximately 8 cm. The calculi were fragmented via semirigid ureteroscopy with holmium laser lithotripsy and basket stone extraction. The bladder was copiously irrigated. The patient was discharged home on the same day with postoperative instructions to perform bladder irrigation twice daily in addition to clean intermittent catheterization. Stone analysis demonstrated calcium phosphate. DISCUSSION