Spontaneous bladder rupture in non-augmented bladder exstrophy. Issue 6 (December 2016)

• Record Type: Journal Article
• Authors: Giutronich, Sarah; Scalabre, Aurélien; Blanc, Thomas; Borzi, Peter; Aigrain, Yves; O'Brien, Mike; Mouriquand, Pierre D.E.; Heloury, Yves
• Is Part Of: Journal of pediatric urology. Volume 12:Issue 6(2016)
• Page Start: 400.e1
• Page End: 400.e5
• Publication Date: 2016-12
• Subjects: Bladder exstrophy–epispadias complex -- Bladder rupture -- Urodynamics -- Long-term follow-up; Pediatric urology -- Periodicals; Urologic Diseases -- Periodicals; Urogenital Diseases -- Periodicals; Urologic Surgical Procedures -- Periodicals; Child; Infant; Urologie pédiatrique -- Périodiques; Appareil urinaire -- Maladies -- Périodiques; Pédiatrie; Urologie; Pediatric urology; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); Electronic journals; Periodicals; Electronic journals; 618.926
• DOI: 10.1016/j.jpurol.2016.04.054
• Languages: English
• ISSNs: 1477-5131

Abstract

Summary: Objective: 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. Methods: 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). Results: 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 cmH2 O 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. Conclusions: 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. Table Patients with bladder perforation. Pt Pre-rupture imaging Pre-rupture continence Age, years Management Outcome 1 Small right kidney with high-grade VUR and high PVR 90 min voiding with episodes of retention 13 Laparotomy IDC 3 weeks Refused augmentation and Mitrofanoff. Recurrent bladder rupture 2 Small left kidney (15% df) with high-grade VUR and high PVR. Poorly compliant bladder on urodynamics Straining to void with frequency 8 Laparotomy and bladder neck closure with SPC Augmentation and Mitrofanoff 3 Trabeculated bladder Straining to void. High PVR. Incontinent 5 Laparotomy SPC 3 weeks Refused Mitrofanoff. Ongoing straining and lost to f/u 4 Trabeculated bladder, low-grade VUR bilaterally and urethral stenosis 2–3 hourly voiding with straining. High PVR 20 SPC with prolonged drainage No further surgery or recurrences known 5 Bilateral HUN 3 hourly voiding with high PVR 15 Laparotomy IDC 3 weeks No further surgery or recurrences known 6 Normal USS Voiding and dry on desmopressin and oxybutynin 11 Laparotomy IDC 3 weeks Augmentation with Mitrofanoff 7 Bilateral HUN with high-grade VUR. Poorly compliant bladder on urodynamics CIC 3 hourly 9 Laparotomy SPC Augmentation with Mitrofanoff. Bladder stone post augment 8 Bilateral HUN with high-grade VUR and reduced function on left Voiding with day and night wetting. Occasional CIC via Mitrofanoff 8 Laparotomy. Drainage via Mitrofanoff Augmentation. Recurrent rupture near the anastomosis of the augment 9 Normal USS. Bladder capacity 100 mL. Low compliance Voiding with day and night leaking. Occasional CIC via Mitrofanoff 10 Laparotomy. Drainage via Mitrofanoff Augmentation, redo Mitrofanoff (Monti) CIC, clean intermittent catheterization; HUN, hydroureteronephrosis; IDC, indwelling catheter; Pt, patient; PVR, post void residual; R, rupture; SPC, suprapubic catheter; USS, ultrasound; VUR, vesicoureteric reflux.