Ponseti I V, Maynard J A, Weinstein S L, Ippolito E G, Pous J G
J Bone Joint Surg Am. 1983 Jul;65(6):797-807.
Biopsy specimens of the lateral aspect of the femoral head and neck were obtained from five children with Legg-Calvé-Perthes disease and were studied using histochemistry and electron microscopy. Beneath the normal articular cartilage there was a thick zone of hyaline (epiphyseal) cartilage containing sharply demarcated areas of hypercellular and fibrillated cartilage with prominent blood vessels. The fibrillated cartilage was strongly positive to alcian blue, weakly positive to periodic acid-Schiff, and positive to aniline blue. The interterritorial matrix in the hypercellular areas was weakly positive to both alcian blue and periodic acid-Schiff. Ultrastructural examination of these areas revealed many irregularly oriented large collagen fibrils and variable amounts of proteoglycan granules. These results suggest that in the fibrillar areas there are: (1) a high proteoglycan content, (2) a decrease in structural glycoproteins, and (3) a different size of collagen fibrils from that of normal epiphyseal cartilage. The hypercellular areas had a decrease in proteoglycans, glycoproteins, and collagen. The lateral physeal margin was often irregular, with a marked reduction of collagen and proteoglycan granules, and contained numerous large lipid inclusions.
The abnormal areas in the epiphyseal cartilage of patients with Legg-Calvé-Perthes disease have different histochemical and structural properties from normal cartilage and from fibrocartilage. This suggests that the disease could be a localized expression of a generalized, transient disorder of epiphyseal cartilage that is responsible for delayed skeletal maturation. The cartilage lesions are similar to those seen in the vertebral plates in patients with juvenile kyphosis. Whether the epiphyseal cartilage abnormalities are primary or are secondary to ischemia remains uncertain; however, it appears that the collapse and necrosis of the femoral head could result from the breakdown and disorganization of the matrix of the epiphyseal cartilage, followed by abnormal ossification.
从5名患有Legg-Calvé-Perthes病的儿童获取股骨头和颈部外侧的活检标本，采用组织化学和电子显微镜进行研究。在正常关节软骨下方有一层厚厚的透明（骺）软骨区，其中含有细胞增多和纤维化的软骨区域，界限清晰，并有明显的血管。纤维化软骨对阿尔辛蓝呈强阳性，对过碘酸希夫呈弱阳性，对苯胺蓝呈阳性。细胞增多区域的区域间基质对阿尔辛蓝和过碘酸希夫均呈弱阳性。对这些区域的超微结构检查显示有许多方向不规则的大胶原纤维和数量不等的蛋白聚糖颗粒。这些结果表明，在纤维状区域存在：（1）高蛋白聚糖含量，（2）结构糖蛋白减少，（3）胶原纤维大小与正常骺软骨不同。细胞增多区域的蛋白聚糖、糖蛋白和胶原减少。外侧骨骺边缘通常不规则，胶原和蛋白聚糖颗粒明显减少，并含有大量大的脂质包涵体。
Legg-Calvé-Perthes病患者骺软骨的异常区域具有与正常软骨和纤维软骨不同的组织化学和结构特性。这表明该疾病可能是骺软骨普遍、短暂紊乱的局部表现，导致骨骼成熟延迟。软骨病变与青少年脊柱后凸患者椎板所见病变相似。骺软骨异常是原发性的还是继发于缺血尚不确定；然而，股骨头的塌陷和坏死似乎可能是由于骺软骨基质的破坏和紊乱，随后发生异常骨化所致。
