Neurofibromatosis involving the lower urinary tract in children. A report of three cases and a review of the literature

Abstract

Three children with neurofibromatosis involving the lower urinary tract are reported and their clinical, radiological and pathological findings are described. Lower urinary tract involvement in neurofibromatosis has previously been reported in 17 children, 12 of whom had other stigmata of von Recklinghausens disease. Lower urinary tract involvement may be asymptomatic and can be found incidentally. Every case with neurofibromatosis presenting with what may appear to be even only insignificant urinary symptoms should be thoroughly investigated urologically. Symptoms are usually related to urinary tract obstruction or neurogenic bladder dysfunction due to the involvement of the nerves supplying the bladder. Urinary diversion is the treatment of choice as surgical removal of the extensive tumour is seldom feasible. Following treatment the prognosis for survival appears to be good.