Leet Arabella I, Sampath Jayanth S, Scott Charles I, MacKenzie William G
Department of Orthopedic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA.
J Pediatr Orthop. 2006 May-Jun;26(3):347-52. doi: 10.1097/01.bpo.0000214923.95314.ad.
Metatropic dysplasia is a rare skeletal dysplasia characterized by rapid collapse of the thoracolumbar spine into kyphoscoliosis. Other spinal anomalies associated with metatropic dysplasia include odontoid hypoplasia and atlantoaxial instability leading to cervical myelopathy. Children with metatropic dysplasia evaluated at our institution for spinal deformity showed evidence of cervical stenosis with or without associated cord compression. Magnetic resonance imaging was found to demonstrate these changes. The association of cervical spinal stenosis and metatropic dysplasia has not been previously described. This has significant treatment implications, because decompression over the stenotic segments should be considered in conjunction with spinal fusion for treatment of odontoid hypoplasia or atlantoaxial instability. A retrospective review of 13 cases of metatropic dysplasia was performed. Despite the challenges provided by this patient population, the chance to halt or reverse neurological dysfunction and improve deformity necessitates prompt surgical intervention.
变异性发育不良是一种罕见的骨骼发育不良，其特征是胸腰椎迅速塌陷成脊柱侧凸。与变异性发育不良相关的其他脊柱异常包括齿状突发育不全和寰枢椎不稳，可导致颈髓病。在我们机构接受脊柱畸形评估的变异性发育不良儿童显示有颈椎管狭窄的证据，伴或不伴有相关的脊髓压迫。磁共振成像显示了这些变化。颈椎管狭窄与变异性发育不良的关联此前尚未见报道。这具有重要的治疗意义，因为在治疗齿状突发育不全或寰枢椎不稳时，应考虑对狭窄节段进行减压并结合脊柱融合术。对13例变异性发育不良病例进行了回顾性研究。尽管这类患者群体带来了诸多挑战，但阻止或逆转神经功能障碍并改善畸形的机会使得必须及时进行手术干预。
