面偏侧萎缩症145例临床分析

Clinical analysis of 145 cases Progressive facial hemiatrophy

目的 探讨面偏侧萎缩症的临床特点及诊治。方法 通过检索截至到2014年2月以来国内文献报道的126例临床病例及我院2010年以来我院收治的19例面偏侧萎缩症临床资料,总结这些患者的发病机制、临床表现、影像学检查及治疗。结果 共纳入145例患者,男∶女=1∶1.96,年龄范围3~53岁,中位年龄17.3岁,病程6-40 y,平均7.9 y。17.3%患者可找到诱因,最常见为外伤（7.6%）,其次为感染（6.2%）。部位左侧∶右侧=1.04∶1,差异无统计学意义（P〈0.05）。145例均出现受累面部瘦小,颧部91例（62.7%）,额部80例（55.2%）,颊部69例（47.6%）,脱发及眉毛脱落62例（42.8%）,下颌部43例（29.7%）,面部感觉异常33例（22.7%）,刀痕征24例（16.5%）,自主神经系统症状14例（9.7%）,肌力稍下降14例（9.7%）。20例（13.8%）伴发硬皮病。33例（22.8%）出现影像学（X线、CT或MRI）异常。14例（9.7%）肌电图异常。治疗有封闭、针灸及整形。结论 面偏侧萎缩症目前尚无明确的诊断标准,主要依据临床表现进行诊断。研究发现偏侧萎缩肢体肌力及咀嚼肌力量受到影响,但均轻微,不影响患者日常生活,但与认为面偏侧萎缩症肌力不受影响的观点不一致。影像学、肌电图可发现异常以辅助诊断,该病仍无有效的治疗方法。

Objective To investigate the clinical features, treatments of Progressive facial hemiatrophy. Methods 126 cases from the chinese published literatures and 19 cases from the first affiliated hospital of Zhengzhou university were reviewed and analyzed. 145 cases were inclued totally, their mechanism, clinical manifestations, imaging examinations and treatments data were collected and summarized. Results We indentified 145 cases,among which the male to female ratio was 1/1.96. The patient ages ranged from 3 - 53 years, the median year was 17.3. The course of the disease was from 6 month to 40 years, which the median course was 7.9 year. 17.2% patients could find cause and the most commonst was injuries （7.6%）,followed by infection （6.2%）. The left to right ratio was 1.04：1, which had no statistically significant difference （ P 〈 0.05）. 145 cases were all affected with thin face, zygomatic atrophy cases were 91 cases （62.7%）, frontal atrophy 80 cases （55.2%）, buccal atrophy 69 cases （47.6%）, hair and eyebrows off 62 cases （42.8%）, submandibular 43 cases （29.7%） ,facial paresthesia 33 cases （22.7%） ,scar sign 24 cases （ 16.5% ） and 14 cases （9.7%） were affected by the autonomic nervous system. The strength slightly decreased cases were 14 cases（9.7% ）. 20 cases （ 13.8% ） suffered from scleroderma. Imaging in 33 patients （22.8%） （X-ray, CT or MRI） found anomalies. Abnormal elemyograpctrohies were found in 14 cases. Treatments included blocking, accpuncture and plastic surgery. Conclusion The mechanism of Romberg Sydrome is unknown, clinical manifestation are much apparent and the diagnosis is not difficult. The strengh of Chewing muscles and limbs are decresed in 9.7% patients, but the changes are mild, and they don＇ t influence the patients＇ daily life. Imaging and abnormal electromyography could be found in auxiliary diagnosis, there still has no effective treatment to the disease.