Di Luigi G, D'Emilio I, Marcozzi A, Gennaccaro L, Palermo P, Carta G
Department of Obstetrics and Gynecology, University of L'Aquila, Italy.
Clin Exp Obstet Gynecol. 2011;38(3):280-2.
Syndactyly is an unusual condition in humans where two or more digits are fused together. In our report we present a case of prenatal diagnosis of simple, complete, bilateral syndactyly as the only ultrasonographic anomaly in a fetus with Down's syndrome. The mother, a 30-year-old, gravida 2, was referred to our hospital with an abnormal triple-test at 17 weeks of gestation, with a final biochemical risk for Down's syndrome more than 1:50. In this pregnancy neither the NT test nor early morphological exam showed typical findings of any chromosomal disorder. The patient underwent amniocentesis. We performed an accurate second level scan at 21 weeks while waiting for genetic results, and we suspected simple, complete, bilateral syndactyly between the third and fourth finger of the hands (rapper sign). The result of the invasive test was 47,XY,+21 and the mother opted for termination of pregnancy; the baby showed simple, complete, bilateral syndactyly of the two digits as suspected during sonography. In presenting our case report, we want to stress the importance of the accuracy of observation of fetal hand morphology, attitude, movements and reactivity. When the observation of fetal hands is not satisfactory (e.g., when the fetus does not open the fist), we recommend external stimulation of fetal reactivity through probe movements on the maternal abdomen (dynamic scan). This approach can make the identification of subtle hand anomalies easier and improve the detection rate of both structural and genetic fetal disorders.
并指畸形是人类一种罕见的情况,即两个或更多手指融合在一起。在我们的报告中,我们呈现了一例产前诊断单纯、完全性双侧并指畸形的病例,这是一名患有唐氏综合征胎儿唯一的超声异常表现。这位母亲30岁,孕2产,在妊娠17周时因三联筛查异常转诊至我院,最终唐氏综合征的生化风险高于1:50。此次妊娠中,颈部透明带(NT)检查和早期形态学检查均未显示任何染色体疾病的典型表现。患者接受了羊水穿刺。在等待基因检测结果期间,我们于孕21周进行了一次精确的二级超声扫描,怀疑双手第三和第四指之间存在单纯、完全性双侧并指畸形(拉普征)。侵入性检测结果为47,XY,+21,母亲选择终止妊娠;婴儿出生后显示出超声检查时所怀疑的两个手指单纯、完全性双侧并指畸形。在呈现我们的病例报告时,我们想强调准确观察胎儿手部形态、姿势、活动及反应性的重要性。当对胎儿手部的观察不令人满意时(例如,胎儿未张开拳头),我们建议通过在孕妇腹部移动探头来对外界刺激胎儿反应性(动态扫描)。这种方法可以使识别细微的手部异常更容易,并提高胎儿结构和基因疾病的检出率。