Katz K, Sabato S, Horev G, Cohen I J, Yosipovitch Z
Department of Orthopedic Surgery, Beilinson Medical Center, Petah Tiqva, Israel.
Spine (Phila Pa 1976). 1993 Mar 1;18(3):332-5. doi: 10.1097/00007632-199303000-00004.
Nineteen patients with type I Gaucher disease with spinal involvement first diagnosed during childhood, were followed for 2 to 24 years (average 9 years), and their clinical and radiologic history reviewed. Patients presented with three types of pain: mild pain that was defined as nonspecific, severe pain due to bone crisis, and pain associated with vertebral collapse. Collapse of vertebra occurred gradually anywhere along the thoraco-lumbar spine, and usually more than one vertebra was involved. In three patients, rectangular collapse was noted. Six patients suffered from central vertebra collapse, and two from anterior wedge compression. Further vertebral collapse with signs of root and cord compression developed in three patients as they grew. Awareness to the possibility of progressive vertebral collapse and periodic follow-up might prevent severe complications.
19例童年期首次诊断为伴有脊柱受累的I型戈谢病患者，随访2至24年（平均9年），并回顾其临床和放射学病史。患者出现三种疼痛：定义为非特异性的轻度疼痛、因骨危象引起的重度疼痛以及与椎体塌陷相关的疼痛。椎体塌陷沿胸腰椎任何部位逐渐发生，通常累及一个以上椎体。3例患者出现矩形塌陷。6例患者发生椎体中央塌陷，2例发生椎体前缘楔形压缩。3例患者随年龄增长出现进一步的椎体塌陷并伴有神经根和脊髓受压体征。认识到进行性椎体塌陷的可能性并定期随访可能预防严重并发症。
