Fargen Kyle M, Anderson Richard C E, Harter David H, Angevine Peter D, Coon Valerie C, Brockmeyer Douglas L, Pincus David W
Department of Neurosurgery, University of Florida College of Medicine, Gainesville, Florida 32610, USA.
J Neurosurg Pediatr. 2011 Jul;8(1):57-62. doi: 10.3171/2011.4.PEDS10450.
Although rarely encountered, pediatric patients with severe cervical spine deformities and instability may occasionally require occipitocervicothoracic instrumentation and fusion. This case series reports the experience of 4 pediatric centers in managing this condition. Occipitocervical fixation is the treatment of choice for craniocervical instability that is symptomatic or threatens neurological function. In children, the most common distal fixation level with modern techniques is C-2. Treated patients maintain a significant amount of neck motion due to the flexibility of the subaxial cervical spine. Distal fixation to the thoracic spine has been reported in adult case series. This procedure is to be avoided due to the morbidity of complete loss of head and neck motion. Unfortunately, in rare cases, the pathological condition or highly aberrant anatomy may require occipitocervical constructs to include the thoracic spine.
The authors identified 13 patients who underwent occipitocervicothoracic fixation. Demographic, radiological, and clinical data were gathered through retrospective review of patient records from 4 institutions.
Patients ranged from 1 to 14 years of age. There were 7 girls and 6 boys. Diagnoses included Klippel-Feil, Larsen, Morquio, and VATER syndromes as well as postlaminectomy kyphosis and severe skeletal dysplasia. Four patients were neurologically intact and 9 had myelopathy. Five children were treated with preoperative traction prior to instrumentation; 5 underwent both anterior and posterior spinal reconstruction. Two patients underwent instrumentation beyond the thoracic spine. Allograft was used anteriorly, and autologous rib grafts were used in the majority for posterior arthrodesis. Follow-up ranged from 0 to 43 months. Computed tomography confirmed fusion in 9 patients; the remaining patients were lost to follow-up or had not undergone repeat imaging at the time of writing. Patients with myelopathy either improved or stabilized. One child had mild postoperative unilateral upper-extremity weakness, and a second child died due to a tracheostomy infection. All patients had severe movement restriction as expected.
Occipitocervicothoracic stabilization may be employed to stabilize and reconstruct complex pediatric spinal deformities. Neurological function can be maintained or improved. The long-term morbidity of loss of cervical motion remains to be elucidated.
尽管小儿严重颈椎畸形和不稳的情况很少见，但偶尔也需要进行枕颈胸内固定和融合术。本病例系列报告了4个儿科中心处理这种情况的经验。枕颈固定是治疗有症状或威胁神经功能的颅颈不稳的首选方法。在儿童中，现代技术最常见的远端固定水平是C-2。由于下颈椎的灵活性，接受治疗的患者仍保持大量颈部活动。在成人病例系列中曾报道过远端固定至胸椎的情况。由于会导致头颈部活动完全丧失，应避免该手术。不幸的是，在极少数情况下，病理状况或高度异常的解剖结构可能需要枕颈固定结构包括胸椎。
作者确定了13例行枕颈胸固定术的患者。通过回顾4家机构的患者记录收集人口统计学、放射学和临床数据。
患者年龄从1岁至14岁不等。有7名女孩和6名男孩。诊断包括克-费二氏综合征、拉森综合征、黏多糖贮积症Ⅳ型和VATER综合征，以及椎板切除术后驼背和严重骨骼发育不良。4例患者神经功能完好，9例有脊髓病。5名儿童在手术前接受了术前牵引；5例行前后路脊柱重建。2例患者的固定范围超过胸椎。前路使用同种异体骨移植，后路关节融合术大多使用自体肋骨移植。随访时间为0至43个月。计算机断层扫描证实9例患者融合；其余患者失访或在撰写本文时未进行重复影像学检查。患有脊髓病的患者病情改善或稳定。1名儿童术后出现轻度单侧上肢无力，另1名儿童因气管造口感染死亡。所有患者均如预期出现严重活动受限。
枕颈胸固定可用于稳定和重建复杂的小儿脊柱畸形。神经功能可得到维持或改善。颈椎活动丧失的长期发病率仍有待阐明。
