Avcu Serhat, Akdeniz Hüseyin, Unal Ozkan, Kurdoğlu Mertihan
Department of Radiology, Yüzüncü Yil University, Van, Turkey.
Fetal Pediatr Pathol. 2010;29(5):353-8. doi: 10.3109/15513815.2010.494704.
We present a case of the Goldston syndrome which is the association of polycystic kidneys with Dandy-Walker malformation. The diagnosis was made by ultrasound in twenty second week of gestation. Obstetric ultrasound and fetal MRI studies showed hydrocephalus, agenesis of the cerebellar hemispheres, vermian hypoplasia, cystic dilatation of the 4(th) ventricle, enlargement of the posterior fossa, abdominal distension, and oligohydramnios.. The kidneys were symmetrically enlarged and multicystic. To our knowledge this is the third reported case of Goldston syndrome which was diagnosed during intrauterine life.
我们报告一例戈德斯顿综合征,该综合征表现为多囊肾合并丹迪-沃克畸形。诊断是在妊娠22周时通过超声做出的。产科超声和胎儿磁共振成像研究显示脑积水、小脑半球发育不全、蚓部发育不良、第四脑室囊性扩张、后颅窝增大、腹胀和羊水过少。双肾对称增大且呈多囊性。据我们所知,这是第三例在宫内诊断出的戈德斯顿综合征病例。