摘要目的 探讨Poland综合征患儿患侧手部不同类型骨骼的骨龄发育状况及其发育特征.方法 2013年2月至2014年8月北京积水潭医院手外科共收治诊断明确且行双侧手部标准正位X线检查的Poland综合征患者32例,男性24例,女性8例;年龄1～15岁,中位年龄2.4岁,畸形位于右侧23例,位于左侧9例.分别选取每例患者患侧手部和健侧手部的20块目标骨(尺骨、桡骨、7块腕骨、11块掌指骨),应用Greulich-Pyle图谱法逐一检测骨龄,并行两侧对比,从而评定患侧手部不同类型骨骼的发育状况;同时按畸形程度将32例病例的手部畸形分为Ⅰ～Ⅴ型.取各骨龄平均数作为个体骨龄,取尺、桡骨骨龄平均数作为长骨骨龄,取腕骨骨龄平均数作为腕骨骨龄,取掌指骨骨龄平均数作为短骨骨龄;骨龄落后指骨龄与生活年龄的差值为负值.采用独立样本差异性分析患侧手部各骨骨龄与健侧是否存在差距,以及该差距与性别、患侧及手部畸形程度的相关性,应用3次方回归曲线分析骨龄差值与年龄的相关性.结果 与健侧相比,患侧手部长骨骨龄落后0 ～1.9岁,平均(0.5±0.5)岁,腕骨骨龄落后0～2.2岁,平均(0.7±0.5)岁,短骨骨龄落后0.5 ～2.0岁,平均(0.6±0.4)岁,总体骨龄落后0.1～1.7岁,平均(0.6±0.4)岁.32例患者中,Ⅱ型手部畸形12例、Ⅲ型15例、Ⅳ型5例.男性患者患侧手部与健侧手部各骨骨龄差值与女性患者相比差异无统计学意义(P值均＞0.05);患侧为左侧患者各骨骨龄差值与患侧为右侧患者相比差异无统计学意义(P值均＞0.05).各骨骨龄差值与手部畸形程度呈正相关(F=3.663 ～ 12.971,P=0.000 ～0.038),且随年龄的增长呈先增后减的特征.结论 Poland综合征患儿患侧手部与健侧相比,各类型骨的骨龄发育落后,且该骨龄差异与年龄和手部畸形程度相关,与性别、患侧并无相关性.

abstractsObjective To explore development status in different types of the hand bone and its developmental characteristics with Poland syndrome.Methods There were 32 cases with Poland's syndrome who accepted bilateral hand X-ray examination in Department of Hand Surgery,Beijing Jishuitan Hospital from February 2013 to August 2014.There were 24 male and 8 female patients aged from 1.0 to 15.0 years with median age of 2.4 years.Right hand deformity was 23 cases and left hand deformity was 9 cases.According to Tanner-Whitehouse skeletal age scoring system,20 bones (radius and ulna,7 carpal bones,11 metacarpal and phalangeal bones) selected from the affected and contralateral limb respectively,were evaluated.Besides,hand deformity of the cases was classified into 5 types based on relevant literature.Each bone was given an individual age using the references of Greulich-Pyle chart.The average of all individual ages was taken as gross bone age,the average of individual ages of radius and ulna was taken as bone age of long bones,the average of individual ages of carpal bone was taken as bone age of carpal bones,and the average of individual age of metacarpal and phalangeal bones was taken as bone age of short bones.The delay of bone age was evaluated by correlation test,while the curve of cubic equation was used for analyzing the variance of skeletal development with age.Results The delay of long bone age of patients with Poland's syndrome in this study were 0-1.9 years ((0.5 ±0.5) years),0-2.2 years ((0.7 ±0.5)years) for carpal bone,0.5-2.0 years((0.6 ±0.4) years)for short bone and 0.1-1.7 years((0.6 ±0.4)years) for gross bone.Twelve cases in type Ⅱ hand deformity,15 cases in type Ⅲ and 5 cases in type Ⅳ.The delay of bone ages,including long bone age,carpal bone age,short bone age and gross bone age,was not related with gender and side(all P ＞ 0.05),but related with degree of deformity(F =3.663-12.971,P =0.000-0.038).Conclusion Compared with normal upper limb,the bone age in the affected limb in Poland's syndrome is delayed and it is correlated with gender,age and the extent of hand deformity and negative with side.