Department of Orthopaedic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Republic of Korea.
PLoS One. 2021 Mar 9;16(3):e0248105. doi: 10.1371/journal.pone.0248105. eCollection 2021.
This study aimed to analyze the epidemiology of congenital upper limb anomalies (CULA) in Korea. We evaluated the incidence of each type of CULA, the presence of coexisting anomalies and the surgical treatment status in CULA patients. We conducted a retrospective cohort study of patients aged < 1 year between 2007 and 2016 who were registered with CULA in the Health Insurance Review and Assessment Service of Korea. In total, 10,704 patients had CULA, including 6,174 boys (57.7%) and 4,530 girls (42.3%). The mean annual incidence of CULA was 23.5 per 10,000 live births; it was significantly higher in boys than in girls (26.3 vs. 20.5, p < 0.001). Among the four categories of CULA-polydactyly, syndactyly, limb deficiency, and other anomalies-polydactyly was the most common. In total, 4,149 patients (38.8%) had other congenital anomalies and coexisting anomalies of the circulatory system (24.9%) were the most common. In total 4,776 patients (44.6%) underwent operative treatment for CULA within minimum three years of the diagnosis. The proportion of patients who underwent surgical treatment was significantly higher for polydactyly (73.4% vs. 16.8%, p < 0.001) and syndactyly (65.3% vs. 41.5%, p < 0.001), but it was significantly lower in limb deficiency (27.6% vs. 45.4%, p < 0.001) and other anomalies (10.0% vs. 69.8%, p < 0.001) than rest of CULA patients. Among the patients who had operations, 21.5% underwent multiple operations. The proportion of patients who underwent multiple operations was significantly higher in syndactyly (35.6% vs. 18.1%, p < 0.001), but it was significantly lower in polydactyly (4.0% vs. 95.5%, p < 0.001) and other anomalies (17.9% vs. 21.9%, p < 0.001) than rest of CULA patients. These results could provide a basis for estimating the national healthcare costs for CULA and the required number of CULA specialists.
本研究旨在分析韩国先天性上肢畸形（CULA）的流行病学。我们评估了每种 CULA 类型的发病率、共存畸形的存在以及 CULA 患者的手术治疗状况。我们对 2007 年至 2016 年间在韩国健康保险审查和评估服务中心登记的年龄<1 岁的 CULA 患者进行了回顾性队列研究。共有 10704 名患者患有 CULA，其中 6174 名男性（57.7%）和 4530 名女性（42.3%）。CULA 的年平均发病率为每 10000 例活产儿 23.5 例；男孩明显高于女孩（26.3 比 20.5，p<0.001）。在 CULA-多指、并指、肢体缺失和其他畸形四类中，多指最为常见。共有 4149 名患者（38.8%）患有其他先天性畸形，循环系统共存畸形最常见（24.9%）。共有 4776 名患者（44.6%）在诊断后至少三年内接受了 CULA 的手术治疗。接受手术治疗的患者比例在多指畸形（73.4%比 16.8%，p<0.001）和并指畸形（65.3%比 41.5%，p<0.001）中明显较高，但在肢体缺失（27.6%比 45.4%，p<0.001）和其他畸形（10.0%比 69.8%，p<0.001）中明显较低。在接受手术的患者中，21.5%接受了多次手术。在并指畸形（35.6%比 18.1%，p<0.001）中，接受多次手术的患者比例明显较高，但在多指畸形（4.0%比 95.5%，p<0.001）和其他畸形（17.9%比 21.9%，p<0.001）中明显较低。这些结果可为估计 CULA 的国家医疗保健费用和所需的 CULA 专家数量提供依据。
