Neurofibromatosis of the urinary tract occurs infrequently: fewer than 70 cases with bladder involvement have been reported to date and of these, only 25 cases occurred in the pediatric population. We describe an unusual case of plexiform neurofibroma and diffuse ganglioneuromatosis of the bladder in a small infant, diagnosed with neurofibromatosis 1 (NF1) at 3 months of age. At the moment, there are no established criteria for the treatment of this rare lesion. Due to the high morbidity, although not very common, bladder involvement should be considered in infants with NF1.
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Published online: February 04, 2013
Accepted: January 7, 2013
Received: June 7, 2012
© 2013 Journal of Pediatric Urology Company. Published by Elsevier Inc. All rights reserved.
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- Re: ‘Diffuse ganglioneuromatosis and plexiform neurofibroma of the urinary bladder: An uncommon cause of severe urological disease in an infant’. J Pediatr Urol. 2013 Apr; 9(3):e131–e133Journal of Pediatric UrologyVol. 10Issue 1
- PreviewWe read with interest the article by Salvitti et al. . As stated by the authors, visceral involvement is typically insidious, progressive, and difficult to treat. It has been theorized that the plexiform neurofibroma involving the bladder is derived from the vesicoprostatic plexus in males and the urethrovaginal plexus in females . Variable involvement of different segments of the plexus translates into a variable constellation of symptoms and signs that arise as neurofibromas enlarge, compressing and distorting local structures.