Miyake Ryuta, Ichikawa Mayuko, Naruse Katsuhiko
OB/GYN, Nara Medical University Hospital, Kashihara, Japan
OB/GYN, Nara Medical University Hospital, Kashihara, Japan.
BMJ Case Rep. 2021 Mar 1;14(3):e237904. doi: 10.1136/bcr-2020-237904.
Congenital contractural arachnodactyly (CCA) is a rare disease with the clinical features of limited extension of multiple joints, arachnodactyly, camptodactyly, thin and long extremities, and so on. In the point of long extremities, CCA resembles Marfan syndrome (MFS). CCA is easily differentiated from MFS after birth due to the flexion of multiple joints, including elbows, knees, hips and fingers. During the fetal period, observation of arachnodactyly and folded fingers by fetal ultrasound is the means of differential diagnosis between these two diseases. We report on a case of CCA diagnosed with prenatal symptoms of long extremities, and introduced physiotherapy in early childhood for a better physical prognosis.
先天性挛缩性蜘蛛指(CCA)是一种罕见疾病,具有多个关节伸展受限、蜘蛛指、屈曲指、四肢细长等临床特征。就四肢细长而言,CCA与马凡综合征(MFS)相似。由于包括肘部、膝盖、臀部和手指在内的多个关节屈曲,CCA在出生后很容易与MFS区分开来。在胎儿期,通过胎儿超声观察蜘蛛指和手指折叠是这两种疾病鉴别诊断的方法。我们报告一例产前诊断为四肢细长症状的CCA病例,并介绍了幼儿期的物理治疗以获得更好的身体预后。