Ishigaki Tatsuya, Akita Shinsuke, Suzuki Hiroyuki, Udagawa Akikazu, Mitsukawa Nobuyuki
Department of Plastic Surgery, Chiba Children's Hospital, 579-1, Heta-cho, Midori-ku, Chiba-city, Chiba, Japan.
Department of Plastic, Reconstructive, and Aesthetic Surgery, Chiba University Graduate School of Medicine, 1-8-1, inohana, Chuo-ku, Chiba-city, Chiba, Japan.
J Plast Reconstr Aesthet Surg. 2019 Jul;72(7):1170-1177. doi: 10.1016/j.bjps.2019.02.030. Epub 2019 Mar 2.
The incidence of postaxial polydactyly of the hand is rare in Japan. This study aimed to compare the clinical presentation of postaxial polydactyly between a cohort of patients from Japan and those from other racial and ethnic backgrounds.
In this retrospective study, we included 30 patients who were treated at our hospital during a 25-year study period (1990-2015). Based on the clinical records of these patients, we characterized the Japanese presentation of the condition. We searched for studies that included other racial and ethnic groups and characterized the clinical presentations. Then, we compared the clinical presentations between Japanese patients and other racial and ethnic groups.
A total of 19 male and 11 female patients were treated in our hospital, and bilateral and unilateral involvements (right side: 4 patients; left side: 4 patients) were observed in 22 and 8 patients, respectively. Moreover, 22 postaxial polydactylies were type A and 28 polydactylies were type B, which were classified using the Temtamy-McKusick classification system. In addition, 4 patients had a family history of hand postaxial polydactylies; 18, 6, 4, and 3 patients presented with polydactyly of the foot, syndactyly, systemic abnormalities, and related syndromes, respectively.
Japanese patients had two distinguishing characteristics: (1) when the condition was unilateral, left side and right side involvement was almost equal with regard to incidence and (2) associated polydactylies of the foot were more common (60%) than those in other cohorts (less than 31%). To better understand postaxial polydactyly of the hand, guidelines to record the clinical presentations in patients with such a condition must be developed.
在日本，手部轴后多指畸形的发病率很低。本研究旨在比较日本患者队列与其他种族和族裔背景患者队列中轴后多指畸形的临床表现。
在这项回顾性研究中，我们纳入了在25年研究期间（1990 - 2015年）在我院接受治疗的30例患者。根据这些患者的临床记录，我们描述了该疾病在日本患者中的表现。我们检索了包含其他种族和族裔群体的研究，并描述了其临床表现。然后，我们比较了日本患者与其他种族和族裔群体的临床表现。
我院共治疗了19例男性和11例女性患者，分别在22例和8例患者中观察到双侧和单侧受累情况（右侧：4例患者；左侧：4例患者）。此外，根据Temtamy - McKusick分类系统，22例轴后多指畸形为A型，28例为B型。另外，4例患者有手部轴后多指畸形的家族史；分别有18例、6例、4例和3例患者出现足部多指畸形、并指畸形、全身异常及相关综合征。
日本患者有两个显著特征：（1）当病情为单侧时，左侧和右侧受累的发生率几乎相等；（2）足部合并多指畸形比其他队列更常见（60%）（其他队列低于31%）。为了更好地了解手部轴后多指畸形，必须制定记录此类疾病患者临床表现的指南。
