小儿骨淋巴管瘤合并脊柱畸形的诊断及治疗

Diagnosis and treatment of bone lymphangioma associated with spinal deformity in children

目的 探讨小儿骨淋巴管瘤合并脊柱畸形的诊断、治疗方法及效果.方法 对北京儿童医院1983至2010年收治的8例骨淋巴管瘤合并脊柱畸形的患儿进行系统分析及随访,对其临床表现、影像学特征、组织病理学特点、治疗方法的选择、治疗结果的评定进行系统总结,尤其是针对该病合并脊柱畸形的发生、发展、预后及早期干预治疗的结果进行评定,结合国内外对该病的治疗经验,总结出小儿骨淋巴管瘤合并脊柱畸形的诊断要点及治疗原则.结果 8例患儿中2例由于治疗不及时导致截瘫,其余6例经过手术刮除病灶植骨、病灶内反复注射平阳霉素及椎弓根钉系统矫正脊柱畸形,经随访10个月至两年,骨淋巴管瘤病灶明显减小或消失,脊柱畸形得到有效控制,脊柱侧弯由术前平均61.3°改善至26.3°,平均矫正率57.0%;脊柱后凸由术前的平均61.6°矫正至25.6°,平均矫正率58.4%.结论 小儿骨淋巴管瘤十分罕见,若侵犯椎体肋骨会导致脊柱畸形进行性加重最终导致截瘫出现.若能早期诊断并采用针对淋巴管瘤的药物治疗及针对脊柱畸形的稳定性手术可以使骨淋巴管瘤得到有效治疗,并能使合并的脊柱畸形得到有效控制避免截瘫的发生.

Objective To investigate the diagnosis, treatment and outcomes of bone lymphangioma associated with spinal deformity in children. Methods Eight cases of children with bone lymphangioma associated with spinal deformity at our hospital from 1983 to 2010 were reviewed and analyzed with the following criteria： clinical manifestations, imaging features, histopathological characteristics and therapeutic options. The outcomes of early intervention treatment were assessed. The key diagnostic points and therapeutic principles were summarized on the basis of our own as well as international and domestic experiences. Results Paraplegia developed in 2 cases because of delayed treatment. The remaining 6 cases were followed up for 10 months to 2 years with satisfactory effects. By curettage lesions, bone grafts,intralesional injection of bleomycin and pedicle screw-stick system maintaining the spinal stability, bone lymphangioma became smaller or disappeared and spinal deformity was effectively controlled. The mean scoliosis and kyphosis correction rates were 57. 0% and 58.4% respectively. Conclusions Bone lymphangioma in children is rare. If costa and vertebrae are involved, spinal deformity will progressively develop and lead eventually to paraplegia. With an early diagnosis and the administration of proper drugs,bone lymphangioma can be effectively controlled. Surgery for the stability of spinal deformity will effectively prevent the occurrence of paraplegia.