先天性上睑下垂患者提上睑肌腱膜的病理改变

背景先天性上睑下垂是临床常见的眼睑运动功能障碍性疾病，可导致患者视功能异常。国外研究表明，提上睑肌的发育异常与该病的发生明显相关，但中国人群该病患者提上睑肌的形态研究缺乏。目的对先天性上睑下垂患者提上睑肌腱膜组织进行组织病理学检查，探讨其发育异常的具体表现。方法先天性上睑下垂患者（年龄14～19岁，平均17岁）2l例，根据上睑下垂的程度分为轻度组（3例）、中度组（14例）和重度组（4例），在提上睑肌缩短术过程中获取所有患眼截除的提上睑肌腱膜组织标本，分别进行苏木精一伊红染色、Masson三色染色、胶原纤维染色，对标本中的Ⅲ型胶原蛋白和肌球蛋白行免疫组织化学染色，患者标本的染色结果与取自北京同仁医院眼库的9例正常供体的新鲜提上睑肌腱膜组织进行对照。结果不同程度先天性上睑下垂患者随着下垂程度的增加，提上睑肌腱膜肌纤维数量减少，间质中结缔组织增加，肌内膜的完整性下降的例数均增加，各组间比较差异均有统计学意义（Z=-0．702，P=0．002；Z=0．738，P‘0．001；Z=0．746，P〈O．001）。4例（占19％）先天性上睑下垂患者标本中发现肌间质中有脂肪细胞增生。免疫组织化学染色发现，先天性上睑下垂患者组提上睑肌腱膜肌纤维中肌球蛋白的表达较正常对照组明显减弱，而Ⅲ型胶原蛋白的表达明显增强。先天性上睑下垂患者组标本中肌动蛋白、肌红蛋白、纤维连接蛋白、Ⅳ型胶原纤维及层黏连蛋白的表达强度与正常对照标本比较差异均无统计学意义（P〉O．05）。结论先天性上睑下垂患者提上睑肌腱膜组织肌纤维、结缔组织及其相关蛋白均有发育异常，其病理改变的程度与症状一致。

Histopathological change of levator aponeurosis in patients with congenital blepharoptosis

Background Congenital blepharoptosis is a common disorder of eyelid movement. Overseas research showed that the most likely mechanism of congenital blepharoptosis is the hypoplasia of levator. But the study on Chinese is still lack. Objective This study was to investigate the pathological features of hypoplasia in levator aponeurosis in Chinese congenital blepharoptosis patients. Methods Twenty-one patients with congenital blepharoptosis were divided into mild group （3 cases） , moderate group （ 14 cases） and severe group （4 cases）. Samples of the levator aponeurosis were obtained during the levator palpebrae superioris muscle shortening surgery. Hematoxylin-eosin,special staining and immunohistochemistry were performed to analyze the characteristics of the samples. Normal samples of fresh ]evator aponeurosis were obtained from the donors in the eye bank of Beijign Tongren Hospital. Results Hematoxylin-eosin staining and special staining showed that with the increase of severity,the cases of levator fibers sparse, fibrous tissue hyperplasia and endomysium defect were gradually increased, showing significant differences among the different groups （ Z = -0. 702, P = 0. 002; Z = 0. 738, P〈0. 001 ; Z = 0. 746, P〈 0. 001 ）. Four samples （ 19% ） presented with adipose in the interstitial tissue. Immunohistochemistry showed that the expression of muscle proteins myosin was weaker in the levator aponeurosis of patients with congenital blepharoptosis than that in the normal samples, and the expression intensity of collagen type m in the samples enhanced in comparison with the normal samples. However, there were no significant differences in the expression of actin, myoglobin,fibronection,collagen type VI and laminin among various groups （all at P〉0.05 ）. Conclusions The levator aponeurosis appears developing abnormality in Chinese patients with congenital blepharoptosis. The histopathological change degree is parallel with the severity of congenital blepharoptosis.