Related Experiment Videos
[Neurofibromatosis of the bladder in infancy. 10 years' follow-up]
F Kempter1, K Möhring, D E Müller-Wiefel
1Urologische Klinik des Chirurgischen Zentrums, Universität, Heidelberg.
Insights
Urinary tract issues in neurofibromatosis are uncommon, particularly in children. This study reviews 51 cases, highlighting upper urinary tract obstruction and successful surgical management for bladder neurofibromatosis.
Area of Science:
- Urology
- Pediatric Urology
- Oncology
Background:
- Neurofibromatosis (NF) is a genetic disorder that can affect various organs, including the urinary tract, though involvement is rare.
- Urinary tract complications in NF, especially in pediatric populations, require careful consideration due to potential for significant morbidity.
Observation:
- A literature review identified 51 documented cases of urinary tract involvement in neurofibromatosis.
- The majority of affected individuals were children (31 cases), with a notable incidence of upper urinary tract obstruction (18 cases).
- A specific case involved a 10-month-old boy with bladder neurofibromatosis.
Findings:
- Upper urinary tract obstruction was observed in over half of the pediatric cases.
- Radical surgery, including cystoprostatectomy and uretero-transureterocutaneostomy, was performed on a 10-month-old patient with bladder neurofibromatosis.
- A 10-year follow-up of this surgical case indicated a positive outcome.
Implications:
- Early recognition and management of urinary tract complications in pediatric neurofibromatosis are crucial.
- Surgical intervention can be effective in treating severe manifestations like bladder neurofibromatosis.
- Further research into optimal treatment strategies for urinary tract involvement in neurofibromatosis is warranted.
Abstract:
Urinary tract involvement in neurofibromatosis is rare. We found 51 cases documented in the literature, the majority of these (n = 31) were children. More than half of these (n = 18) developed upper urinary tract obstruction. A report is given of a 10-month-old boy with neurofibromatosis of the bladder. Treatment consisted in radical surgery with cystoprostatectomy and uretero-transureterocutaneostomy. Follow-up of 10 years presents a good result. The different modalities of treatment are reviewed in the literature.