Abdominal signs of von recklinghausenrsquo;s disease a rare case report and revue of literature

Abstract

Von recklinghausen's disease is a dominant genetic disease whose incidence is 1 in 4000 of all cases are spontaneous mutations, it belongs to phacomatoses and is part of neurocristopathy. The disease manifestations are extremely variable. The abdominal manifestations during this disease is well described in the literature, but of relatively rare occurrence. We report in this article the case of a patient aged 2 years, recently admitted to the pediatric surgery department for a retro-vesical pelvic mass discovered on the abdomino-pelvic ultrasound performed in the etiological assessment of chronic diarrhea in von recklinghausen disease. The patient underwent a surgery with favorable post-operative follow up, histopathological evaluation of the piece returned to a bladder wall ganglioneuroma associated with diffuse neurofibroma. Association of neurofibromatosis 1 and ganglioneuroma is rarely reported in the literature. The follow-up of patients with nf1 is clinical and radiological in the quest for complications that could involve the patient's prognosis, hence the urgency of the surgical management of complications of benign or malignant tumor.