ABNORMAL EXTRACELLULAR MATERIAL IN THE LEVATOR PALPEBRAE SUPERIORIS COMPLEX IN CONGENITAL PTOSIS
ABNORMAL EXTRACELLULAR MATERIAL IN THE LEVATOR PALPEBRAE SUPERIORIS COMPLEX IN CONGENITAL PTOSIS
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DOI：
10.1001/archopht.1995.01100110074028
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发表时间：
1995-11-01
影响因子：
--
通讯作者：
LEE, WR
中科院分区：
文献类型：
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作者：
CLARK, BJ;KEMP, EG;LEE, WR
目的：目的：探讨先天性上睑下垂提上睑肌的病理改变。方法：应用光镜、电镜及免疫组织化学方法，对15例先天性上睑下垂提上睑肌行常规上睑提肌切除术后的前提上睑肌组织标本进行观察。此外，在15例患者中的4例中，存在异常细胞外物质。通过光学显微镜，这似乎是一种不寻常的无定形物质，排列在团块和条带中，电子显微镜显示它由平行的粗纤维颗粒物质束组成。通过免疫组织化学在该材料中鉴定了III型胶原和纤连蛋白。有没有可检测到的胶原蛋白I，II，IV，V，VI，或VII或层粘连蛋白，和材料没有染色肌动蛋白，肌球蛋白，肌红蛋白，淀粉样蛋白P成分，或淀粉样蛋白A.结论：在15例先天性上睑下垂患者的提上睑肌样本中，我们确定了一个不寻常的无定形细胞外材料，染色阳性的III型胶原蛋白和纤连蛋白的免疫组化。这种新的材料，我们称之为“amorphocollagenoid”，可能是提上睑肌组织发育不良的产物，这种材料的来源和全部成分值得进一步研究。
Objective: To determine the pathologic abnormalities of the levator palpebrae superioris in congenital ptosis.Methods: By means of light and electron microscopy and immunohistochemistry, anterior levator tissue specimen from 15 patients with congenital ptosis excised during routine levator resections were examined.Results: All specimens showed lack of muscle fibers with endomysial and perimysial fibrosis and thickening of the aponeurosis. In addition, in four of the 15 patients, an abnormal extracellular material was present. By light microscopy this appeared as an unusual amorphous material arranged in clumps and bands, and electron microscopy showed it to consist of parallel coarse bundles of fibrillogranular material. Collagen type III and fibronectin were identified within this material by immunohistochemistry. There was no detectable collagen types I, II, IV, V, VI, or VII or laminin, and the material did not stain for actin, myosin, myoglobin, amyloid P component, or amyloid A.Conclusions: In four of 15 samples of levator palpebrae superioris from patients with congenital ptosis, we identified an unusual amorphous extracellular material that stained positively for collagen type III and fibronectin on immunohistochemistry. This novel material, which we call ''amorphocollagenoid,'' may represent a product of dysgenesis of the levator tissues, The source and full composition of this material merit further study.