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近年,家族性大腸腺腫症における大腸以外の消化管病変が注目されてきている.本邦においては,既に,胃病変の合併頻度は60%以上1)~6),十二指腸病変のそれは100%7)8)と報告され,更に最近では小腸病変の検索も重要視されてきている.飯田らは術中小腸内視鏡検査により半数以上に腺腫が認められたと報告している8)9).われわれも,十二指腸・上部空腸に多数の腺腫と考えられる病変が認められ,他合併病変として,下顎骨潜在性骨腫・歯牙異常,皮下血管腫がみられた1例を経験したので報告する.
A case of colo-rectal adenomatosis associated with multiple lesions at the duodenum and jejunum, occult osteomas at the mandible and dental anomalies, and a subcutaneous hemangioma, is reported.
A 19-year-old man was first seen at our hospital on May 9, 1976, because of shortness of breath and bloody stool.
Barium enema demonstrated numerous polyps and the specimen excised by endoscopic polypectomy showed adenoma, therefore he was admitted under the diagnosis of colo-rectal adenomatosis on May 30, 1976.
There was a negative family history, though upper G.I. series with small bowel follow-through, bariumenema, roentgenogram of the general bones and dental examination were performed for his patients and sister, and only history-taking from paternal and maternal grandparents. Therefore this case is thought to be nonfamilial case at present.
As gastro-intestinal lesions besides colon and rectum, there was no evidence of esophageal and gastric lesions, but hypotonic double contrast study of the duodenum and upper jejunum demonstrated multiple minute protruded lesions, and endoscopic examination of the second portion of the jejunum revealed multiple small yellow-white protruded lesions. Biopsy specimen from these lesions showed an evidence of adenoma.
Only 32 cases, associated with lesions of the small bowel, has been reported so far.
Total colectomy with ileostomy was performed on Oct. 27, 1977. Macroscopical findings of the resected specimen showed non-carpeted type, and microscopically 3 adenocarcinomas were noted.
As other associated lesions, so-called occult Osteomas at the mandible and dental anomalies, subcutaneous capillary hemangioma were noted. In addition, lymphfollicular hyperplasia of the terminal ileum was also found.
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