小児例を含む家族性大腸ポリポージスの上部消化管病変についての考察

Familial Polyposis of the Colon: A study on upper gastrointestinal lesions and infantile onset

　家族性大腸ポリポージスは，大腸にびまん性に無数の腺腫性ポリープが発生する比較的稀な疾患として知られている．高率に大腸癌が発生することもあって，従来の研究は主に大腸病変に限られたものであった．そのため本症における上部消化管についての検索は，ほとんどなされていなかったといえる．1974年宇都宮ら¹⁾は胃の精密検査を行ない得た6家系15症例中10例（66.7％）に胃ポリープを合併していたと報告し，上部消化管に対する精査の必要性を強調した．引き続いて大里ら²⁾，牛尾ら³⁾もほぼ同様の頻度で胃病変の合併をみたと報告している．しかし，十二指腸・小腸病変の合併に関しては，同部位が胃に比して診断困難なためか，ほとんどその報告をみない．したがって，これら上部消化管病変の発生時期および発育過程などに関しては，ほとんど知られていないのが現状である．

　そこでわれわれは胃・十二指腸に微細な多発性腺腫を認めた本症2家系5症例（小児3例を含む）を報告し，合併十二指腸病変の特異性，小児例における病像の特徴，および本症の自然経過などについて，若干の考察を加えてみたい．

　This report is based on two families in which 2 adults (41 and 38 years old) and 3 children (11, 8 and 8 years old) developed polyposis of the colon and upper gastrointestinal tract.

　In all of 5 cases numerous small sessile polyps were noted in the duodenum. Histological examination performed on 3 cases revealed all polyps to be adenoma. Multiple sessile polyps were found in the gastric antrum in 4 cases. Histological examination on 2 cases showed all gastric polyps to be adenoma as well. Careful upper gastrointestinal survey including hypotonic duodenography and fiberscopy is necessary to find these small lesions in the duodenum.

　The polyps of the colon in 3 children, histologically adenoma, were found as tiny sessile lesions scattered in the sigmoid and descending colon. But polyps were hardly recognized in the rectum. These results suggest that these iufantile cases could be missed by sigmoidoscopy alone, which has been described to be an adequate examination for this purpose.

　Two adult cases had the characteristic findings of familial polyposis of the colon; diffuse polyposis and colonic cancers, quite distinct from those seen in the children. On the other hand, upper gastrointestinal lesions in adult cases consisted of small sessile polyps, similar to those seen in the infantile cases and showed no malignant change. Therefore, the associated upper gastrointestinal lesions may develop as early as colonic lesions, but the former may have slower growth and fewer malignat changes.

　Gardner's stigmata were also recognized in 3 cases. These findings suggest that this disease should not be regarded as a localized disease of the colon, but as a systemic disease with a predisposition to multiple tumor formation.