Fauchet R, Stagnara P
Rev Chir Orthop Reparatrice Appar Mot. 1981;67(7):647-53.
The authors describe three cases of megspondylodysplasia, an original syndrome first described in 1972. This congenital lesion is associated with severe kyphoscoliosis, multiple hemimelic enchondromatoses of the spine, hands, feet, knees and hips and hypertrophy of half of the body. In the discussion, differentiation between this syndrome and other diseases such as Ollier's or Mafucci's disease is described. Surgical correction on such a severely deformed spine is dangerous to the spinal cord.
作者描述了三例巨脊椎发育不良病例，这是一种1972年首次被描述的原发性综合征。这种先天性病变与严重的脊柱侧凸、脊柱、手、脚、膝盖和臀部的多发性半肢内生软骨瘤以及身体半侧肥大有关。在讨论中，描述了该综合征与其他疾病如奥利尔病或马富西病之间的鉴别。对如此严重畸形的脊柱进行手术矫正对脊髓有危险。
