Severe urological involvement in junctional epidermolysis bullosa justifying bladder enlargement by sigmoidocystoplasty and Mitrofanoff appendicovesicostomy

The urological complications of junctional epidermolysis bullosa (JEB) are difficult to manage because of the fragility of the skin and mucosa, and are not the subject of any recommendations. We report on 2 cases of chronic lower urinary tract dysfunction causing chronic kidney disease, treated by b...

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Published inArchives de pédiatrie : organe officiel de la Société française de pédiatrie Vol. 32; no. 4; pp. 281 - 284
Main Authors Ehilé-Kacou, Marielle, Mure, Pierre Yves, Demede, Delphine, Phan, Alice, Bacchetta, Justine, Bidault, Valeska, Ranchin, Bruno
Format Journal Article
LanguageEnglish
Published France Elsevier Masson SAS 01.05.2025
Subjects
Appendix - surgery
Bladder dysfunction
Child
Chronic kidney disease
Colon, Sigmoid - surgery
Cystostomy - methods
Epidermolysis bullosa junctionalis
Epidermolysis Bullosa, Junctional - complications
Humans
Mitrofanoff
Urinary Bladder - surgery
Epidermolysis bullosa junctionalis
Chronic kidney disease
Bladder dysfunction
Mitrofanoff
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Summary:The urological complications of junctional epidermolysis bullosa (JEB) are difficult to manage because of the fragility of the skin and mucosa, and are not the subject of any recommendations. We report on 2 cases of chronic lower urinary tract dysfunction causing chronic kidney disease, treated by bladder enlargement with sigmoidocystoplasty and Mitrofanoff appendicovesicostomy after failure of medical treatment and suprapubic catheterisation several times a day. Follow-up was favourable in both children, with kidney function preservation and significant improvement of quality of life.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:0929-693X
1769-664X
1769-664X
DOI:10.1016/j.arcped.2025.01.002