Spontaneous bladder rupture in non-augmented bladder exstrophy

Bladder perforation is not commonly described in bladder exstrophy patients without bladder augmentation. The goal of this study was to identify the risk factors of spontaneous perforation in non-augmented exstrophy bladders.

The study was a retrospective multi-institutional review of bladder perforation in seven male and two female patients with classic bladder exstrophy-epispadias (E-E).

Correction of E-E was performed using Kelly repair in two and staged repair in seven (Table). Bladder neck repair was performed in eight patients at a mean age of 6 years. Three patients had additional urethral surgery. Before rupture, six patients were voiding only per urethra. Two patients were voiding urethrally but were also performing occasional CIC via a Mitrofanoff. One patient was performing CIC 3 hourly per urethra. Six were dry during the day. Six of the patients had lower urinary tract symptoms: five had frequency and four were straining to void. Two had suffered episodes of urinary retention. Pre-rupture ultrasound showed that the upper urinary tract was dilated in four patients. Micturating cystourethrogram was performed in six showing vesico-ureteral reflux in five. Two had urethral stenosis. Nuclear medicine was done in three patients with two abnormal differential function. Urodynamics was performed in two patients with low capacity (100 mL) and hypocompliant (<10) bladders. Both had high leak point pressures: 60 cmH₂O at 100 mL. The mean age at rupture was 11 years, with a range of 5-20 years. Patients presented with abdominal pain, associated with signs of intestinal obstruction in seven and fever in two. Eight patients underwent laparotomy and one prolonged drainage via SPC. Simple closure was performed in seven and bladder neck closure in one, because of extension of the rupture inferiorly. All patients recovered well. Following rupture, five underwent augmentation and Mitrofanoff. One of these suffered a recurrent rupture. Two other patients refused augmentation and Mitrofanoff and one of these has since had a subsequent rupture.

The limitations of this series include the small number of patients and its retrospective nature, without knowledge of the incidence. Bladder rupture is a risk even in non-augmented bladder exstrophy. It is potentially life-threatening and most often requires laparotomy. Rupture occurs because of poor bladder emptying and/or high pressure. Urodynamics may identify those at risk. CIC with or without augmentation should not be delayed once poor bladder emptying and/or high pressure are identified.