Management of bladder dysfunction in Wolfram syndrome with Mitrofanoff appendicovesicostomy: long-term follow-up

Abstract Purpose To present the long-term outcomes of appendicovesicostomy using the Mitrofanoff principle for end-stage Wolfram bladder dysfunction as an alternative to clean intermittent self-catheterization (CIC) per urethra mainly following blindness. Methods Twelve Wolfram patients presenting w...

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Bibliographic Details
Published in:Journal of pediatric surgery 2015-07, Vol.50 (7), p.1201-1204
Main Authors: Mozafarpour, Sarah, Kajbafzadeh, Abdol-Mohammad, Mojtahed, Ali, Mojtahed, Mohammad, Mahboubi, Hossein, Shalileh, Keivan
Format: Article
Language:English
Subjects:
Adult
Appendix - surgery
Bladder dysfunction
Cystostomy - methods
Diabetes insipidus
Diabetes mellitus
Disease Management
Female
Follow-Up Studies
Humans
Male
Middle Aged
Mitrofanoff principle
Nephrotic Syndrome - therapy
Pediatrics
Renal Insufficiency - surgery
Surgery
Surgical Stomas
Urethra - surgery
Urinary Bladder - abnormalities
Urinary Bladder - surgery
Urinary Catheterization
Urinary Diversion - methods
Urologic Surgical Procedures - methods
Wolfram (DIDMOAD) syndrome
Wolfram Syndrome - complications
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Summary:Abstract Purpose To present the long-term outcomes of appendicovesicostomy using the Mitrofanoff principle for end-stage Wolfram bladder dysfunction as an alternative to clean intermittent self-catheterization (CIC) per urethra mainly following blindness. Methods Twelve Wolfram patients presenting with bilateral hydroureteronephrosis and advanced bladder dysfunction were included in this study. All patients were managed initially by CIC per urethra. All of these patients became blind during follow-up and were unable to perform urethral CIC independently. Out of these patients, six patients agreed to proceed to appendicovesicostomy. Appendicovesicostomy urinary diversion using the Mitrofanoff principle was performed in these six blind patients. The rest of the patients stopped CIC or performed CIC irregularly. Results Severe hydroureteronephrosis and large bladders were found in all patients prior to intervention. All patients were able to conduct CIC independently through the stoma and maintained overnight bladder free drainage. In all patients with urinary diversion and CIC, the hydroureteronephrosis was reduced and renal function returned to normal. However, the non-intervention group ended with different degrees of progressive renal failure with three mortalities during the follow-up. Conclusions We suggest appendicovesicostomy as a safe and lifesaving procedure for long-term management of bladder dysfunction in Wolfram syndrome particularly after progression to blindness
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2014.09.058