Panchalee Tachjaree, Ruangvutilert Pornpimol, Limsiri Pattarawan, Sutcharitpongsa Pavit
Department of Obstetrics and Gynecology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
BMJ Case Rep. 2018 Oct 25;2018:bcr-2018-224362. doi: 10.1136/bcr-2018-224362.
A 29-year-old nulliparous woman with a dichorionic diamniotic (DCDA) twin pregnancy was referred to our hospital at 16 weeks' gestation for prenatal diagnosis. She was diagnosed of Haemoglobin H Constant Spring (Hb H CS; --/αα) and her husband of alpha thalassemia-1 trait (--/αα). Detailed ultrasound showed that left twin had fetal anaemia and early signs of hydrops while the right one was normal. Both twins were female. Amniocentesis in each sac was performed for prenatal diagnosis of thalassemia after a proper counselling with the couple. DNA analysis confirmed that the left fetus was affected with haemoglobin Bart's hydrops fetalis (--/--) while the right one was alpha thalassemia-1 trait (--/αα). Selective feticide with intracardiac injection of KCl was successfully performed on the hydropic fetus. Identification of the affected fetus is crucial for selective termination. Family counselling about the procedure and complications is also necessary.
一名29岁未生育的双绒毛膜双羊膜囊(DCDA)双胎妊娠女性在妊娠16周时因产前诊断转诊至我院。她被诊断为血红蛋白H Constant Spring(Hb H CS;--/αα),其丈夫为α地中海贫血1特征(--/αα)。详细超声检查显示,左胎有胎儿贫血和早期水肿迹象,而右胎正常。两个胎儿均为女性。在与这对夫妇进行适当咨询后,对每个羊膜囊进行了羊膜腔穿刺术以进行地中海贫血的产前诊断。DNA分析证实,左胎患有巴氏水肿胎儿血红蛋白病(--/--),而右胎为α地中海贫血1特征(--/αα)。对水肿胎儿成功进行了心内注射氯化钾的选择性减胎术。识别受影响的胎儿对于选择性终止妊娠至关重要。关于该手术及并发症的家庭咨询也是必要的。