Résumé
Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition. It is a multisystem disorder. Abdominal involvement in patients with NF1 has been described in the form of neurofibromas within the liver, mesentery, retroperitoneum and gastrointestinal (GI) tract. Involvement of bowel is rarely seen. Malignant peripheral nerve sheath tumour of small intestine is a rare entity with very few cases reported in literature. Surgery remains the only curative option. Chemotherapy may be used as an adjuvant for advanced diseases. We report a case of malignant peripheral nerve sheath tumour of small intestine which presented as acute intestinal obstruction.
Résumé
An internal hernia is a protrusion of bowel through a normal or abnormal orifice in the peritoneum or mesentery. Although they are considered as a rare cause of intestinal obstruction, para-duodenal hernias are the most common type of congenital internal hernias. We report one such case of a left para-duodenal hernia presented as acute intestinal obstruction.