日本未熟児新生児学会雑誌　第26巻第1号　124～130頁（2014年）

日本未熟児新生児学会雑誌　26(1)：124-130；2014　

受付日：平成24．09．25

受理日：平成25．10．01

Milk curd syndrome 関連の腸管合併症を来たした早産低出生体重児6例

Intestinal Obstruction, Perforation and Necrotizing Enterocolitis in Premature Infants due to Milk Curd Syndrome

神奈川県立こども医療センター　新生児科

Department of Neonatology, Kanagawa Children’s Medical Center

新関昌枝・大山牧子・山口直人・斎藤朋子・石川　淑・柴崎　淳・豊島勝昭・星野陸夫・川滝元良・猪谷泰史

Masae NIIZEKI，Makiko OHYAMA，Naoto YAMAGUCHI，Tomoko SAITO，Shuku ISHIKAWA, Jun SHIBASAKI，Katsuaki TOYOSHIMA，Rikuo HOSHINO，Motoyoshi KAWATAKI，Yasufumi ITANI

Key Words：milk curd, intestinal obstruction, premature, human milk fortifier

　経腸栄養確立後に糞便性腸閉塞に続いて壊死性腸炎・腸穿孔を発症した早産低出生体重児6例について，経過と手術所見よりmilk curd syndrome（MCS）と診断し，その危険因子および診断・治療に関して検討した。
　症例は妊娠23～35週，体重436～2,004gで出生した早産低出生体重児で，腹部膨満，便秘（浣腸によっても排便がみられない状態），胆汁性胃内容液で発症した。発症日齢は中央値36（24～59）で，栄養内容は強化母乳3例，母乳1例，混合栄養2例であった。6例中5例は，禁乳，抗菌薬投与，注腸造影などの内科的治療が無効で，開腹手術を受けた。3例は初回手術時，2例は再手術時に腸管内に凝乳塊（milk curd）が充満し，それによる腸管損傷と考えられる限局性の壊死性腸炎・腸穿孔を認め，回腸人工肛門造設を要した。1例は注腸後にmilk curdの排泄を認め，腸閉塞は解除された。
　MCSの危険因子として，本症例では腸管の未熟性，胆汁うっ滞による脂肪吸収障害，消化管手術後の癒着，経十二指腸チューブ栄養，母乳添加物質などが考えられた。早産低出生体重児の慢性期の腸管合併症として，MCSも念頭に置きながら，早期診断と迅速な治療を進めることが重要である。

　We experienced 6 preterm infants with milk curd syndrome（MCS）with 23 to 35 weeks of gestation and 436 to 2,004 grams at birth. Four were breast fed with or without additional breast-milk fortifier, and two were both breast and formula fed. The initial symptoms were observed on day 36（range from 24 to 59）with abdominal distention, constipation and bile-stained aspirates or vomitus. Five infants soon developed sepsis and coagulopathy. Conservative therapy such as cessation of feeding and antibiotic therapy failed in all of 6 patients. GastrografinⓇ contrast enemas relieved the obstructions in 1 patient but failed in 2 patients. Laparotomy was performed in 5 patients. Three patients had localized necrotizing enterocolitis（NEC）with perforation and required ileostomy. Two patients did not show intestinal perforation or NEC and received only lavage of milk curd followed by second-look laparotomy and ileostomy.
　The pathogenesis of MCS is considered to be multifactorial such as intestinal immaturity, calcium-rich breast milk fortifier, cholestasis, and previous abdominal surgery. We would like to add possible risk factors, i.e.；transpyloric feeding, and antiapneic drugs, with decrease peristalsis.
　We suggest considering MCS in all previously stable premature infants who shows signs of intestinal obstruction. Early surgical intervention with ileostomy might be life saving and prevent secondary events such as intestinal perforation or NEC.