Esmat Habib Ahmad, Naseri Mohammad Wali, Shirzai Asadullah
Department of Radiology, Kabul University of Medical Sciences, Kabul, Afghanistan.
Department of Internal Medicine, Division of Endocrinology, Metabolism, and Diabetes, Kabul University of Medical Sciences, Kabul, Afghanistan.
Radiol Case Rep. 2021 Feb 24;16(5):1080-1084. doi: 10.1016/j.radcr.2021.02.015. eCollection 2021 May.
Heterotaxy syndrome is a rare condition characterized by the abnormal arrangement of thoracoabdominal organs across the left-right axis of the body. It is generally classified as right and left atrial isomerism or asplenia and polysplenia syndrome, even though there are overlaps and uncertainties. The diagnosis of isomerism is typically made by echocardiography. However, multidetector computed tomography and MRI can help in obtaining detailed data on the morphology of the heart, great vessels, the anatomy of the internal organs, and their mutual arrangement that make an accurate diagnosis of heterotaxy syndrome. The authors present here the imaging findings of the heterotaxy polysplenia syndrome in a 21-year-old female with a complete endocardia cushion defect and a duplicated right renal vein.
内脏异位综合征是一种罕见的病症，其特征是胸腹部器官在身体的左右轴线上排列异常。尽管存在重叠和不确定性，但它通常被分类为右心房异构和左心房异构或无脾和多脾综合征。异构的诊断通常通过超声心动图进行。然而，多排计算机断层扫描和磁共振成像有助于获取有关心脏、大血管形态、内部器官解剖结构及其相互排列的详细数据，从而准确诊断内脏异位综合征。作者在此展示了一名21岁女性内脏异位多脾综合征的影像学表现，该女性患有完全性心内膜垫缺损和右肾静脉重复畸形。
