对先天性桡尺骨融合患者流行病学特征的分析

Analysis of the epidemiological features in congenital radioulnar synostosis cases

目的 探讨先天性桡尺骨融合(CRUS)患者的流行病学特征及其发病危险因素。方法2020年1至3月,通过网络问卷调查收集2017年1月至2019年12月北京积水潭医院手外科收治的CRUS患者100例的资料,并进行回顾性分析。其中男75例,女25例,平均年龄5.21岁(0~29岁)。收集患者的一般信息,民族,籍贯,疾病特点及其父母相关孕产期致畸因素和母亲孕产史。采用Cleary与Omer分型方法对CRUS进行分型。依据患者父亲和母亲接触射线及有毒化合物情况分别将患者分为低危险、中危险和高危险组。采用χ~2检验对不同侧别发病患者中男、女所占比例以及3组中各型CRUS进行比较;采用Kruskal-Wallis检验对3组患者的双侧患病率进行比较。结果 100例患者(155个患肢)中双侧患病55例(110个患肢);Cleary与Omer 3型患肢最多见(125个,80.6%)。男性患者双侧患病较多(χ~2=13.968,P=0.003)。1型和2型更多见于双侧患病。3组双侧患病率差异无统计学意义(依据父亲分组P=0.441,依据母亲分组P=0.548)。患者及其父母的出生地、籍贯、民族以及患者父母吸烟史与发病率均无显著相关性。各项孕产期因素与CRUS发病均无显著相关性。患者及其父母最关注的治疗结果是前臂旋转功能的改进以及消除患者的心理障碍。结论 CRUS主要累及男性,双侧发病多见,Cleary与Omer 3型多见;患者父母孕产期吸烟史、射线和有毒化合物接触程度与CRUS发病率无确切相关性;患者母亲孕早期病史可能与CRUS发病相关。

Objective To explore the epidemiological characteristics of patients with congenital radioulnar synostosis(CRUS) and possible risk factors for CRUS.Methods From January to March 2020,a internet questionnaire was conducted on 100 patients with CRUS treated in the Department of Hand Surgery,Beijing Jishuitan Hospital from January 2017 to December 2019.Seventy-five males and 25 females aged 5.21(0-29)years were included.The following data were collected:the general information(age,gender,birthplace,ethnicity),contact history of teratogenic factors of patient′s parents(smoking,radioactive rays,toxic compounds),history of gestation of mothers(age of gestation,abnormal pregnancy-labor history,history of smoking,drinking,infection,chronic disease,medication,trauma and contact of teratogenic factors),the information of affected limbs,classification and concomitant diseases,the family history and the expectation of treatment.CRUS was classified by Cleary-Omer classification.All patients were divided into 3 groups based on their fathers' and mothers′ exposure to teratogenic factors(radiation and toxic compounds):high risk,middle risk and low risk,respectively.The χ~2 test was engaged in the bilateral involved cases between male and female.The Kruskal-Wallis test was engaged in the bilateral involved cases among 3 groups,respectively.Results Of the 100 patients(155 affected limbs),55 patients(110 affected limbs) were bilaterally affected.Affected limbs in type 3 were the most common(125 affected limbs,80.6%).Male patients had more bilateral affected limbs(χ~2=13.968,P=0.003).Type 1 and type 2 were more common in bilateral affected limbs.There was no significant difference in the bilateral prevalence among the 3 groups(P=0.441 and 0.548 for the 3 groups divided according to patients′ father and mother,respectively).There was no significant correlation between the incidence and birthplace,place of origin,nationality,and smoking history of the patient's parents.The forearm function and psychological issue were the most concerned therapeutic goal according to the patients and their patients.Conclusion CRUS mainly affects males and bilaterally affected limbs and type 3 limb are more common.Smoking history,exposure to radiation and toxic compounds of the patient′s parents are not reliably associated with disease severity.The maternal history of illness in early pregnance may be related to the onset of CRUS.