Pellengahr C, Krödel A, Müller-Höcker J, Pongratz D
Orthopädische Klinik und Poliklinik, Ludwig-Maximilians-Universität München, Klinikum Grosshadern, Germany.
Arch Orthop Trauma Surg. 1998;117(6-7):411-4. doi: 10.1007/s004020050282.
Multicore disease is a rare congenital myopathy that normally stops or makes slow progress. It starts with delayed motor development and frequent falls. Especially the proximal muscles are involved. Other symptoms include contractures of joints, clubfoot, high arched palate, chest deformities, involvement of eye-muscle, cardiac abnormalities, hyperlordosis and scoliosis that normally does not require surgery. We report a rare case of advanced scoliosis in multicore disease requiring operative treatment in a 15-year-old girl. In this case the scoliosis took a rapidly progredient course in puberty close to the end of skeletal growth. A dorsal spondylodesis with USS instrumentation from T9 to L3 was performed. A significant reduction of the scoliosis was obtained. Scoliosis in multicore disease may take a rapidly progredient course. Frequent checks will lead to the initiation of surgical treatment in time.
多核病是一种罕见的先天性肌病，通常病情停止进展或进展缓慢。其起病表现为运动发育延迟和频繁跌倒。尤其近端肌肉受累。其他症状包括关节挛缩、马蹄内翻足、高腭弓、胸廓畸形、眼肌受累、心脏异常、腰椎前凸过度和脊柱侧弯，脊柱侧弯通常无需手术治疗。我们报告一例15岁女孩患多核病并出现严重脊柱侧弯需手术治疗的罕见病例。该病例中，脊柱侧弯在青春期骨骼生长接近尾声时呈快速进展病程。实施了T9至L3节段的后路脊柱融合内固定术，脊柱侧弯得到显著改善。多核病中的脊柱侧弯可能呈快速进展病程。定期检查将有助于及时启动手术治疗。
