Lepinard C, Coutant R, Boussion F, Loisel D, Delorme B, Biquard F, Bonneau D, Guichet A, Descamps P
Department of Obstetrics and Gynecology, CHU Angers, 90 bis rue de Létanduère, 49000 Angers, France.
Ultrasound Obstet Gynecol. 2005 Jan;25(1):73-5. doi: 10.1002/uog.1807.
Septo-optic dysplasia (SOD; De Morsier syndrome) is a rare congenital disorder characterized by the absence of the septum pellucidum (SP), hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. We report on two fetuses with absence of the SP diagnosed by ultrasound examination at 29 and 30 gestational weeks. In the first case the diagnosis of SOD was suspected in utero and confirmed postnatally; to the best of our knowledge this is the first report of the prenatal diagnosis of SOD. In the second case absence of the SP appeared to be isolated and no visual or endocrine impairment were detected after birth.
视隔发育不良(SOD;德莫西埃综合征)是一种罕见的先天性疾病,其特征为透明隔缺如、视交叉和视神经发育不全,以及各种类型的下丘脑 - 垂体功能障碍。我们报告了两例在孕29周和30周时经超声检查诊断为透明隔缺如的胎儿。第一例在宫内怀疑为视隔发育不良,出生后得以确诊;据我们所知,这是视隔发育不良产前诊断的首例报告。第二例透明隔缺如似乎是孤立存在的,出生后未检测到视觉或内分泌功能损害。