Thomas S, Winter R B, Lonstein J E
Minnesota Spine Foundation, Minneapolis, USA.
Spine (Phila Pa 1976). 1997 Jun 15;22(12):1330-7. doi: 10.1097/00007632-199706150-00010.
This study evaluated the different forms of treatment of camptomelic dysplasia, a rare form of short-limbed dwarfism.
To determine the most efficacious form of management of spinal deformities in camptomelic dysplasia.
The literature on treatment of spinal deformities in camptomelic dysplasia is sparse. One report advocates aggressive surgical treatment to prevent curve progression and prevent already compromised respiratory function.
Eight patients with camptomelic dysplasia and progressive spinal deformity underwent a retrospective chart and radiographic review by an independent observer. Follow-up averaged 3 years and 9 months.
Five of eight patients initially were treated with bracing and six of eight patients eventually required surgery. Average initial kyphosis was 114 degrees and scoliosis 61 degrees, compared with 99 degrees kyphosis and 52 degrees scoliosis at follow-up. Complications included pseudarthrosis (50%) and neurologic problems (33%).
The authors advocate anterior/posterior uninstrumented fusion and halo cast immobilization postoperatively to prevent curve progression and avoid the potentially fatal sequelae associated with this disorder.
本研究评估了坎普梅尔发育异常（一种罕见的短肢侏儒症）的不同治疗方式。
确定坎普梅尔发育异常中脊柱畸形最有效的管理方式。
关于坎普梅尔发育异常中脊柱畸形治疗的文献稀少。一份报告主张积极的手术治疗以防止畸形进展并预防已受损的呼吸功能进一步恶化。
八位患有坎普梅尔发育异常和进行性脊柱畸形的患者接受了一位独立观察者的回顾性病历及影像学检查。平均随访时间为3年9个月。
八位患者中有五位最初接受支具治疗，八位患者中有六位最终需要手术。初始平均后凸为114度，脊柱侧凸为61度，随访时后凸为99度，脊柱侧凸为52度。并发症包括假关节形成（50%）和神经问题（33%）。
作者主张术后采用前路/后路非内固定融合及头环石膏固定以防止畸形进展，并避免与该疾病相关的潜在致命后遗症。
