日本新生児成育医学会雑誌　第34巻　第2号　55 ～ 60頁（2022年）

日本新生児成育医学会雑誌　34(2)：176-181；2022　

受付日：2021．09．03

受理日：2022．03．30

術後に消化管通過障害と腸管不全合併肝障害を認めたApple peel 型先天性小腸閉鎖症

A Case of a Surviving Male Infant with Apple-peel Intestinal Atresia Who Had Progressive Liver Dysfunction During Postoperative Gastrointestinal Transit Disturbance

＊ 1 山梨大学医学部　小児科，＊ 2 山梨大学医学部　第二外科

＊ 1 Department of Pediatrics, Faculty of Medicine, University of Yamanashi，＊ 2 Division of Pediatric Surgery, Second Department of Surgery, Faculty of Medicine, University of Yamanashi

鎌田康弘＊ 1・矢ヶ崎英晃＊ 1・深尾俊宣＊ 1・成澤宏宗＊ 1・村上　寧＊ 1・蓮田憲夫＊ 2・中島博之＊ 2・犬飼岳史＊ 1

Yasuhiro KAMATA ＊ 1，Hideaki YAGASAKI ＊ 1，Toshimichi FUKAO ＊ 1，Hiromune NARUSAWA ＊ 1， Yasushi MURAKAMI ＊ 1，Norio HASUDA ＊ 2，Hiroyuki NAKAJIMA ＊ 2，Takeshi INUKAI ＊ 1

Key Words：Apple-peel intestinal atresia，cyclic total parenteral nutrition（cyclic TPN），intestinal failure-associated liver disease（IFALD）

　Apple peel 型先天性小腸閉鎖症は死亡率が14 ～ 54％と高く，手術後の胆汁うっ滞，敗血症，短腸症候群の合併が予後に影響を与える。症例は在胎34 週6 日，出生体重2,346g の男児。日齢1 に消化管ガスの異常分布を認め，消化管造影検査で腸管通過障害を確認した。日齢2 に開腹手術を行い，Grosfeld 分類Ⅲb 型のApple peel 型先天性小腸閉鎖症と診断し，小腸閉鎖部の切除と端々吻合術を実施した。手術後，消化管通過不良による中心静脈栄養管理の長期化とMRSA 敗血症を認め，腸管不全合併肝障害を併発した。ウルソデオキシコール酸，十二指腸チューブ管理，間欠的高カロリー輸液を併用し，日齢56 に経静脈栄養から離脱し経腸栄養を確立できた。Apple peel 型先天性小腸閉鎖症は，手術後の腸管不全合併肝障害への対策を念頭にした管理により長期予後の改善が見込めるため，症例の蓄積と術後栄養管理法のさらなる検討が期待される。

Apple-peel intestinal atresia is a rare disease, with a postnatal mortality rate of 14 ～ 54％. Complications, such as postoperative bile stasis, sepsis, and short bowel syndrome, have a significant impact on the prognosis. We report a male infant who was born at 34 weeks and 6 days gestation with a birth weight of 2,346 grams. Abdominal radiographs on day 1 showed an abnormal distribution of gastrointestinal gas, and gastrointestinal enema led to suspicion of jejunal atresia. On the 2nd day, we performed an end-to-end anastomosis of the small intestine and diagnosed Grosfeld type Ⅲb apple-peel intestinal atresia. Although hepatic dysfunction（ intestinal failure-associated liver disease：IFALD） due to prolonged central venous hyperalimentation that had been necessary due to poor gastrointestinal transit after surgery was observed, we were able to wean the patient from transvenous nutrition and establish enteral feeding at 56 days of age by using ursodeoxycholic acid, duodenal tube management, and intermittent high-calorie infusion. In cases of intestinal atresia, the long-term prognosis can be improved by transvenous and enteral nutritional strategies for hepatic injury complicated by intestinal insufficiency after surgery.
Accumulation of further cases and studies of appropriate postoperative nutritional strategies are expected.