Castro Pedro Teixeira, Matos Ana Paula Pinho, Werner Heron, Daltro Pedro, Fazecas Tatiana, Nogueira Renata, Araujo Júnior Edward
Department of Obstetrics and Gynecology, Escola de Medicina Souza Marques, Rio de Janeiro, Brazil.
Department of Maternal and Child, Fluminense Federal University (UFF), Niteroi, Brazil.
J Obstet Gynaecol Can. 2017 Dec;39(12):1176-1179. doi: 10.1016/j.jogc.2017.04.041. Epub 2017 Jun 29.
Caroli disease is a very rare congenital anomaly characterized by non-obstructive saccular or fusiform dilatation of the intrahepatic bile ducts. It is associated with bile stagnation and hepatolithiasis, which explain the recurrent cholangitis and portal hypertension as a consequence of congenital liver fibrosis. Although there are several reports of diagnosis in childhood and adult life, the prenatal diagnosis using conventional 2-D ultrasound is rare, with few reports in the literature.
We present a case of a 26-year-old primigravid woman at 24 weeks of gestation which 3-D ultrasound in the rendering mode clearly revealed the enlarged fetal kidneys and the increased abdominal volume, confirming the diagnosis of autosomal recessive polycystic kidney disease. The MRI was essential to the prenatal diagnosis of Caroli disease, identifying the congenital saccular dilations of intrahepatic bile ducts.
卡罗里病是一种非常罕见的先天性异常，其特征为肝内胆管的非阻塞性囊状或梭形扩张。它与胆汁淤积和肝内胆管结石有关，这解释了先天性肝纤维化导致的复发性胆管炎和门静脉高压。尽管有关于儿童期和成年期诊断的多篇报道，但使用传统二维超声进行产前诊断却很罕见，文献中报道较少。
我们报告一例26岁初产妇，妊娠24周，三维超声渲染模式清晰显示胎儿肾脏增大和腹腔容积增加，确诊为常染色体隐性多囊肾病。磁共振成像对于卡罗里病的产前诊断至关重要，可识别肝内胆管的先天性囊状扩张。
