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KMID : 0371320060710030238
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Journal of the Korean Surgical Society
2006 Volume.71 No. 3 p.238 ~ p.240
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Plexiform Neurofibroma Involving the Ascending Colon and its Mesentery in a Child
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박재정/Park JJ
최금자/이선화/성순희/Choi KJ/Lee SW/Sung SH
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Abstract
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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. (J Korean Surg Soc 2006;71:238-241)
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