6年間経過観察中のCronkhite-Canada症候群の1例

Cronkhite-Canada's Syndrome with Right Hemicolectomy and Subtotal Gastrectomy Performed in the Course of Six Years, Report of a Case

要旨　症例は54歳女性．生来健康であったが，1977年（49歳時），右下腹部痛を主訴として入院，回盲部の大腸ポリープによる腸重積症で結腸右半切除を施行した．また，胃・小腸にも多数のポリープを認めた．切除結腸に存在した大腸ポリープの1個に腺腫を認めたが，ほかはすべて異型のない腺管が過形成性の増生を示し大小の囊胞を形成していた．更に手足の爪甲の変形と異常色素沈着，低蛋白血症などの臨床所見よりCronkhite-Canada症候群と診断した．1979年には爪甲の変形は消失し，胃ポリープの数も著明に減少した．1980年には，低蛋白血症と貧血の増悪を認め輸血を施行したが，この時期には再び爪甲の変形が出現し，胃ポリープの数も増加した．1982年には胃ポリープの著明な増加を認め，これが蛋白漏出の一因と考え胃亜全摘を施行した．切除胃には，びまん性に無数のポリープが発生していたが，組織学的にはすべて過形成性のものであった．術後経過良好で，現在外来にてfollow-up中である．

　A 54 year-old woman had been well until January 1977 when she developed atrophy of nails, hyperpigmentation of the extremities and hypoproteinemia. In July of the year, she had an acute onset of abdominal pain and was diagnosed as bowel obstruction in our hospital. Investigation in emergency laparotomy revealed invagination of the ileo-caecal region and diffuse polyposis of the stomach, small intestine and colon. Therefore, right hemicolectomy was performed. By histological examination of the resected colon, all polyps except an adenoma showed hyperplasia of non-atypical glands, cystic dilatation of glands and edematous stroma. She was given a high protein diet and plasma after the operation and was discharged in December of the year.

　In 1979, atrophy of nails was healed and gastric polyps were decreased in number. However, since 1980, the number and size of gastric polyps increased and severe hypoproteinemia appeared again.

　In February 1982, she was admitted to our hospital again because of anorexia and general fatigue. Roentgenographic and endoscopic examinations of the stomach revealed numerous diffuse polyps. It was assumed that severe hypoproteinemia was caused by the gastric polyps, and thus subtotal gastrectomy was performed. Histologically all polyps of the stomach were hyperplastic polyps.