Intra-abdominal manifestations of von Recklinghausen's neurofibromatosis.
Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition with an approximate incidence of one per 3000 births. NF1 involves multiple systems of the body. Abdominal involvement occurs in the form of neurofibroma and tumour growth in the liver, mesentery, retroperitoneum, gastric and bowel. Gastrointestinal neoplasms have a reported occurrence of 2-25%. Two cases are reported herein as well as a review of the literature of the intra-abdominal manifestations of NF1, including a discussion on the radiological appearance and diagnosis. The article provides an insight into the intriguing variety of clinical problems that such patients may present.
Rastogi
Rajul
R
Yash Diagnostic Center, Yash Hospital and Research Center, Civil Lines, Kanth Road, Moradabad, India. eesharastogi@gmail.com