Plexiform Neurofibroma Involving the Ascending Colon and its Mesentery in a Child.
- Author:
Jae Jeong PARK
1
;
Kum Ja CHOI
;
Sun Wha LEE
;
Sun Hee SUNG
Author Information
1. Department of Surgery, College of Medicine, Ewha Womans University, Seoul, Korea. kumchoi@ewha.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis type 1 (von Recklinghausen's disease);
Plexiform neurofibroma;
Mesocolon;
Child
- MeSH:
Abdominal Cavity;
Abdominal Pain;
Adolescent;
Cafe-au-Lait Spots;
Child*;
Colon, Ascending*;
Female;
Gastrointestinal Tract;
Genetic Diseases, Inborn;
Hemorrhage;
Humans;
Incidence;
Mesentery*;
Mesocolon;
Neurofibroma;
Neurofibroma, Plexiform*;
Neurofibromatosis 1;
Skin
- From:Journal of the Korean Surgical Society
2006;71(3):238-241
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The neurofibromatosis type 1 (NF-1) is a rare hereditary disease of autosomal dominant fashion with the overall incidence of one in 3,000. It is characterized by cafe-au-lait spots of skin, multiple cutaneous neurofibromas and a broad spectrum of clinical finding. Plexiform neurofibroma is a frequent complication of NF-1 but symptomatic involvement of the gastrointestinal tract in children with NF-1 is rare. It may present with complications such as obstruction, dysfunction, pain and hemorrhage. We report that a 13-year-old female diagnosed with NF-1 at the age of 10 years had abdominal pain and showed huge plexiform neurofibromas of right mesocolon in abdominal cavity. She also presented with multiple cafe-au-lait spots and axillary freckling. She was performed a right hemicolectomy with complete excision of the mass and had an uneventful course.
