Beneux J, Rigault P, Pouliquen J C, Geffroy J, Padovani J P, Merckx J, Guyonvarch G
Rev Chir Orthop Reparatrice Appar Mot. 1978 Sep;64(6):471-85.
The authors have studied twenty cases of spine deformity in children suffering from Marfan's syndrome. They describe the main features of the disease and point out the difficulty of accurate diagnosis in children with minor involvement. The deformities are those of a dysplastic scoliosis as shown by the frequency of vertebral inversion on the lateral view: dorso-lumbar kyphosis, decreased lumbar lordosis. The treatment should be surgical because of the danger of a fatal outcome without treatment. The incidence of non-union is high and the authors recommend systematic revision of the fusion double curve scolioses with a moderate kyphosis. In cases of severe kyphosis, the addition of anterior grafting is recommended.
作者研究了20例患有马凡氏综合征的儿童脊柱畸形病例。他们描述了该疾病的主要特征,并指出了轻度受累儿童准确诊断的困难。畸形表现为发育异常性脊柱侧凸,如侧位片上椎体反转的频率所示:胸腰段后凸、腰椎前凸减小。由于不治疗有致命后果的风险,治疗应采用手术。不愈合的发生率很高,作者建议对中度后凸的双弯脊柱侧凸融合进行系统性翻修。对于严重后凸的病例,建议增加前路植骨。