Nitta Hayase, Taira Yusuke, Kinjo Tadatsugu, Chinen Yukiko, Masamoto Hitoshi, Sanabe Naoya, Goya Hideki, Yoshida Tomohide, Sugibayashi Rika, Sumie Masahiro, Wada Seiji, Sago Haruhiko, Aoki Yoichi
Department of Obstetrics and Gynecology, University of the Ryukyus, Okinawa, Japan.
Department of Digestive and General Surgery, University of the Ryukyus, Okinawa, Japan.
AJP Rep. 2017 Jul;7(3):e185-e187. doi: 10.1055/s-0037-1606830. Epub 2017 Sep 22.
We report a case of congenital pulmonary airway malformation (CPAM) with hydrops in which the fetus underwent thoracoamniotic shunting. A 40-year-old (G1P1) woman was diagnosed with a macrocystic CPAM. Thoracoamniotic shunting was performed at 19 weeks of gestation but not well drained and was successfully performed again at 23 weeks. However, the CPAM volume ratio, abdominal circumference, and amniotic fluid index started increasing from 28 weeks and hydrops worsened. The insufficient shunting and the fetal cardiac failure had to be considered. At 32 weeks, a male infant with general edema and massive ascites was born weighing 3,362 g (+4.79 SD) with Apgar scores of 2 and 4. The infant was intubated and high-frequency oscillation and nitric oxide therapies were instituted. The resection of CPAM was performed on day 2. Nasal continuous positive airway pressure was instituted on day 16. The infant was discharged and prescribed with home oxygen therapy (HOT) on day 65. The infant was able to leave the HOT at 30 months and is currently 34 months of age in good condition. Fetal thoracoamniotic shunting may be life-saving in CPAM complicated by hydrops and that this treatment might be sufficient to cure the child.
我们报告一例先天性肺气道畸形（CPAM）合并胎儿水肿并接受胸腔羊膜腔分流术的病例。一名40岁（孕1产1）女性被诊断为大囊型CPAM。妊娠19周时进行了胸腔羊膜腔分流术，但引流不畅，23周时再次成功进行。然而，CPAM体积比、腹围和羊水指数从28周开始增加，水肿加重。必须考虑分流不足和胎儿心力衰竭。32周时，一名全身水肿和大量腹水的男婴出生，体重3362g（+4.协方差），阿氏评分分别为2分和4分。婴儿插管并采用高频振荡和一氧化氮治疗。CPAM在出生后第2天切除。出生后第16天开始鼻持续气道正压通气。婴儿在第65天出院并接受家庭氧疗（HOT）。婴儿在30个月时能够停止家庭氧疗，目前34个月大，状况良好。胎儿胸腔羊膜腔分流术对于合并水肿的CPAM可能是挽救生命的，并且这种治疗可能足以治愈患儿。
