摘要目的:对照分析胎儿多发关节挛缩症(arthrogryposis multiplex congenita，AMC)的产前超声表现和产后病理类型，总结AMC受累关节的产前声像图特征。方法:收集2015年1月至2020年6月于中山大学附属第一医院及开平市中心医院产前超声发现、产后病理证实的AMC病例，对比产前超声表现、受累关节类型与产后病理特征的差异，并比较合并与不合并其他系统异常病例的关节受累情况。结果:共纳入AMC 31例，合并其他系统异常11例，不合并其他系统异常20例。合并与不合并其他系统异常两组受累关节的差异无统计学意义( P>0.05)。产前超声提示与产后病理诊断完全符合21例(21/31，67.7%)。31例中受累关节占比依次为指间(23/31，74.2%)、膝(20/31，64.5%)、踝(19/31，61.3%)、下颌(11/31，35.5%)、腕(11/31，35.5%)、肘(10/31，32.3%)、趾间(6/31，19.4%)、脊柱(2/31，6.5%)、肩(1/31，3.2%)和髋(1/31，3.2%)。病例受累部位关节的产前超声符合率分别为指间(100%)、踝(100%)、脊柱(100%)、髋(100%)、腕(90.9%)、膝(75.0%)、肘(70.0%)、下颌(54.5%)、趾间(50.0%)和肩(0)。 结论:当产前超声筛查发现胎儿上、下肢姿势异常，尤其是重叠指、伸膝和足内翻等畸形时，应警惕AMC，需仔细扫查胎儿其他关节，以提高AMC的产前检出率。

abstractsObjective:To summarize the prenatal sonographic characteristics of affected joints of fetal arthrogryposis multiplex congenita (AMC) by comparing the ultrasonographic features and the postnatal pathological manifestations.Methods:The cases of AMC detected by antenatal ultrasound and confirmed by postnatal pathology were collected in the First Affiliated Hospital of Sun Yat-Sen University and Kaiping Central Hospital from January 2015 to June 2020. The differences between prenatal ultrasonic manifestations, types of affected joints and postnatal pathological features were analyzed. And the different involvements of joints in AMC cases with or without other system abnormalities were also explored separately.Results:A total of 31 cases of AMC were included, in which 11 cases were with other system abnormalities and 20 cases without. No significant difference was observed in number of affected joints between these two groups ( P>0.05). The prenatal sonogram features were completely consistent with the postnatal pathological manifestations in 21 (21/31, 67.7%) cases. Among 31 cases, the involvement rates of joints were: interphalangeal joints of fingers (23/31, 74.2%), knee joints (20/31, 64.5%) and ankle joints (19/31, 61.3%), temporomandibular joint (11/31, 35.5%), wrists (11/31, 35.5%), elbow joints (10/31, 32.3%), interphalangeal joints of toes (6/31, 19.4%), spinal joints (2/31, 6.5%), shoulder joint (1/31, 3.2%) and hip joint (1/31, 3.2%), respectively. The coincidence rates of prenatal ultrasound in involved joints were: interphalangeal joints of fingers (100%), ankles (100%), spines (100%), hips (100%), wrists (90.9%), knees (75.0%), elbows (70.0%), jaws (54.5%), interphalangeal joints of toes (50.0%), and shoulders (0), respectively. Conclusions:When postural abnormalities of fetal upper and lower extremities are detected by prenatal ultrasound screening, especially overlapping fingers, extended knee and club foot, AMC should be kept on alert. Simultaneously, other joints should be carefully scanned to improve the prenatal detection rate of AMC.