観血的内視鏡的ポリペクトミーを施行したPeutz-Jeghers症候群の1例

Report of a Case of Peutz-Jeghers Syndrome Treated with Intraoperative Endoscopic Polypectomy

　Peutz-Jeghers症候群は口唇指趾等の色素沈着と遺伝傾向を有する腸管ポリポージスで，本邦では200例以上の報告がある．Peutz-Jeghers症候群の揚合，ポリープの癌化は比較的稀であるが，ポリープの誘因となる腸重積の合併が多く，全消化管に発生したポリープの処置が問題となる．ポリペクトミーの手技としてはendoscopic surgeryの応用が侵襲も少なくすぐれているが，ポリープが全消化管に多数存在する場合，あるいは腸重積合併例等では観血的手術が必要となる．最近われわれは急性膵炎の経過中に発見されたPeutz-Jeghers症候群の1症例（腸重積合併例）に開腹のうえ内視鏡的ポリペクトミーを施行し，全消化管にわたって存在した計50コのポリープを切除し，きわめて良好な結果を得たので報告する．

　A case of Peutz-Jeghers Syndrome complicated with acute pancreatitis is reported. The patient was a 17 years old high school girl admitted for the chief complaints of epigastrial pain, nausea and vomiting. Numerous small spots with brown pigmentation were observed on her lips, oral mucosa and fingers. Laboratory data on admission showed marked leucocytosis and increased amylase level in both serum and urine. Roentogenographic and endoscopic examination revealed diffuse polyposis in the stomach, duodenum and both in the small and large bowels. The findings of invagination was not observed. She was diagnosed to have Peutz-Jeghers Syndrome with acute pancreatitis. Acute pancreatitis was treated with fasting and intravenous hyperalimentation. Three weeks after therapy, her complaints lessened and laboratory data improved.

　The patient, however, developed invagination soon after she began eating at the eighth week. At laparotomy invagination was reposed manually. Then intraoperative endoscopic polypectomy was performed through a small incision in the jejunum, and fifty polyps were resected. Eight months after the treatment, the patient was quite well.

　Since patients with Peutz-Jeghers Syndrome are at a high risk of death due to invagination and for its prevention polypectomy is necessary, endoscopic polypectomy is recommended when they are operated on for reposition of invagination.