Poland综合征合并畸形的形态学特征及治疗策略

Clinical presentations and treatment strategy of associating deformities in Poland syndrome

目的 研究和总结Poland综合征手和胸大肌以外的畸形临床形态学特征及治疗策略.方法 回顾分析自2000年2月至2014年4月我院收治的91例Poland综合征患者资料,对Poland综合征手和胸大肌以外的畸形进行定性的研究和总结,并探讨各类畸形的治疗方法.结果 91例中有85例发现存在除手部和胸部以外的畸形,其中71例存在2种或2种以上合并畸形.共发现12种合并畸形,前三位者分别为乳头发育异常、肋骨发育异常及锁骨短小畸形.结论 Poland综合征并非一局限性先天畸形,除手部和胸大肌畸形外,还有很多其他部位的畸形,严重影响患者的外观及功能.要求手外科医师对Poland综合征可能出现的形态学特征有更全面的了解,进行更为细致的检查,才能及时发现这些畸形,从而联合相应科室针对不同性别的患者在不同年龄段及时制定个体化治疗方案.

Objective To study and review the morphological characteristics of and treatment strategy for malformations other than hand and pectoralis major deformities in Poland syndrome.Methods Ninety-one cases of Poland syndrome treated from February 2000 to April 2014 were retrospectively reviewed.The clinical morphological characteristics of malformations other than hand and pectoralis major deformities were systemically analyzed,and the treatment methods of various malformations were also reviewed and summarized.Results Eighty-five out of the 91 cases had associating deformities other than the hand and chest.Seventy-one cases had 2 or more than 2 additional deformities.A total of 12 types of deformity existed,the top three being nipple,rib and clavicle deformities.Conclusion Morphological changes of patients with Poland syndrome are complex.Besides deformities in the hands and pectoralis major,there are deformities in other parts of the body,which can seriously affect the appearance and function.Hand surgeons should have a more comprehensive understanding of the morphological manifestations of Poland syndrome and thorough physical examination of the patient to make an individualized treatment plan teamed with relevant departments and based on gender and age of the patient.