Hussain Zainab, Masroor Imrana, Haider Qurrat-Ul-Ain, Alam Tariq
Department of Radiology, The Aga Khan University Hospital, Karachi.
J Coll Physicians Surg Pak. 2011 Apr;21(4):242-4.
Goldston syndrome is a rare entity describing the association of polycystic kidneys and Dandy Walker malformation with or without hepatic fibrosis. A 28 years old pregnant woman came to radiology department for the first antenatal scan. Ultrasound showed a single fetus corresponding to 27 week of gestation. The fetal brain revealed deficient vermis with a posterior fossa cyst communicating with the 4th ventricle suggestive of Dandy-Walker malformation (DWM). The kidneys were enlarged, and echogenic containing small cysts with associated amniotic fluid index of 5 cm suggestive of oligohydramnios. A diagnosis of Goldston syndrome was suggested. These findings were confirmed on post natal head and renal ultrasound.
戈德斯顿综合征是一种罕见的病症，描述了多囊肾与丹迪-沃克畸形的关联，可伴有或不伴有肝纤维化。一名28岁的孕妇前来放射科进行首次产前扫描。超声显示单胎，孕周为27周。胎儿脑部显示小脑蚓部缺失，后颅窝囊肿与第四脑室相通，提示丹迪-沃克畸形（DWM）。双肾增大，回声增强，含有小囊肿，羊水指数为5厘米，提示羊水过少。初步诊断为戈德斯顿综合征。产后头部和肾脏超声检查证实了这些发现。
