Spontaneous Vesicovaginal Fistula in Neurofibromatosis: A Case Report

Background: Neurofibromatosis type 1 is an autosomal dominant transmitted disease with various forms of clinical presentation. It commonly affects the skin and the nervous system. Involvement of the genitourinary system is rare. The bladder is the most commonly affected organ in the urinary system. Vesicovaginal fistula presenting as continuous leakage of urine per vagina is a rare urogenital manifestation of neurofibromatosis which has not been previously reported to the best of our knowledge. Vesicovaginal fistula is not a usual complication of urethral catheterization. Our aim is to report a rare case of vesicovaginal fistula in a patient with neurofibromatosis. Case presentation: We report a case of a 36 year-old nullipara with eighteen years history of multiple features of type 1 neurofibromatosis who presented with continuous leakage of urine per vagina of spontaneous onset. Patient had urinary incontinence following which she was catheterized for 18 years but subsequently started leaking urine despite catheterization five months prior to presentation. There were no other known associated predisposing factors. Important findings on examination were paraplegia, multiple neurofibroma, café-au-lait spot and bony deformities including scoliosis. Examination in theatre showed vesicovaginal (juxta-urethral) fistula. Abdominal computerized tomography scan showed left diaphragmatic crural cyst, dextroscoliosis and cholelithiasis. Pelvic CT scan revealed irregular thickening of the bladder wall, uterine leiomyomata and left hemi pelvic deformity/dysplasia with resultant hip dislocation suggestive of neurofibromatosis of bone. Chest x-ray noted a soft tissue mass in the posterior mediastinum. She had surgical repair of vesicovaginal fistula using the vaginal approach. Conclusion: The cause of vesicovaginal fistula in this patient with neurofibromatosis is uncertain. However, it may have resulted from bladder neurofibromatosis or prolonged urethral catheterization or both.