Abdulian Michael H, Liu Raymond W, Son-Hing Jochen P, Thompson George H, Armstrong Douglas G
Division of Pediatric Orthopaedic Surgery, Rainbow Babies and Children's Hospital, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, OH, USA.
J Pediatr Orthop. 2011 Jan-Feb;31(1):6-10. doi: 10.1097/BPO.0b013e3182032029.
Rib head penetration into the spinal canal in patients with severe kyphoscoliosis secondary to neurofibromatosis type-1 (NF-1) is extremely rare. Double rib head penetration has only been reported once earlier.
We are reporting on an adolescent male with NF-1 and severe thoracic kyphoscoliosis with adjacent double rib head penetration into the spinal canal without neurological deficits. Only one was recognized on the initial radiographic imaging.
A 14-year-old with NF-1 and 74 degrees left thoracic scoliosis and 75 degrees kyphosis was treated at our institution. Preoperative computed tomography (CT) demonstrated protrusion of the left T6 rib head into the spinal canal on the convexity of the curve, compressing the spinal cord. Staged surgical procedures for resection of the rib head and correction of the spinal deformity were planned. After presumed successful resection of the penetrated rib head, a postoperative CT revealed the presence of a second adjacent left T7 rib head in the spinal canal. This was not initially recognized owing to the severe deformity and image obliquity of the CT gantry. Another procedure was performed to remove this rib head. He was then placed in halo traction until anterior and posterior spinal fusion and segmental spinal instrumentation were performed. He achieved good deformity correction and had no neurological deficits throughout his treatment.
Rib head protrusion into the spinal canal can occur with spine deformity in NF-1. If present, the imaging should be carefully reviewed for the possibility of an adjacent rib head penetration that may have been obscured by the limitations of CT in the context of a dysplastic spinal deformity.
Level V. Case study.
1型神经纤维瘤病（NF-1）继发严重脊柱侧凸患者中，肋骨头部穿入椎管极为罕见。双肋骨头部穿入此前仅报道过1次。
我们报告1例患有NF-1的青少年男性，其患有严重的胸椎后凸畸形，相邻的双肋骨头部穿入椎管且无神经功能缺损。初次影像学检查仅发现1处穿入。
一名14岁患有NF-1的患者，左胸弯74度，后凸75度，在我院接受治疗。术前计算机断层扫描（CT）显示，在侧弯凸侧，左侧第6肋骨头部突入椎管，压迫脊髓。计划分阶段进行肋骨头部切除术及脊柱畸形矫正手术。在假定成功切除穿入的肋骨头部后，术后CT显示椎管内存在相邻的左侧第7肋骨头部。由于严重畸形及CT扫描架的图像倾斜，最初未识别出此处穿入。遂再次手术切除该肋骨头部。随后对其进行头环牵引，直至行前路和后路脊柱融合及节段性脊柱内固定术。整个治疗过程中，患者畸形矫正良好，无神经功能缺损。
NF-1患者脊柱畸形时可发生肋骨头部突入椎管。若存在这种情况，应仔细复查影像学检查，以排除因发育不良性脊柱畸形导致CT检查受限而可能被漏诊的相邻肋骨头部穿入。
V级。病例研究。
