目的:描述一名患有1型神经纤维瘤病的胸椎部分omega畸形患者的手术治疗。
方法:患者是一名55岁的男性,患有欧米茄畸形,它被定义为弯曲,其中末端椎骨位于的水平,上面,或根尖椎骨以下(即,将其平分的水平线)。我们进行了7周的光环重力牵引(HGT),随后从T2-L5进行了几乎相等的脊柱后路融合,并进行了三个同种异体股骨头移植物和一个局部自体骨移植物。由于他的严重肺功能障碍,我们避免了胸前脊柱的重建。
结果:HGT将%肺活量从32.5提高到43.5%,并将后凸的Cobb角从牵引前的>180°改善到牵引后的144°。后凸和脊柱侧凸的Cobb角由术前>180°变化为155°和146°,分别,术后,和167°和156°,分别,在最后的后续行动。由于双侧胸腔积液和压迫性肺不张,他的术后呼吸功能短暂恶化。通过频繁的位置变化和鼻腔高流量成功治疗1周。在最后的后续行动中,在VC中,他的肺功能从0.86L改善到1.04L,从32.5%到37.9%的VC。然而,手术后的术前痛苦没有总体改善,尽管他的改良Borg量表从术前的3提高到术后的0.5。出院后一个月,他感到呼吸窘迫恶化(SpO2:75%),并因肺动脉高压再次入院2个月。他通过无创正压通气(双相气道正压通气)1周得到改善,药物治疗和每日肺部理疗。此后,他一直在家中接受永久性的白天(0.5L/min)和夜间(2L/min)氧疗。在计算机断层扫描上证实了通过融合区域的固体关节固定术。然而,后凸矫正损失为12°(即155°-167°),而脊柱侧弯矫正损失为10°(即,146°-156°),恢复2年。
结论:我们建议几乎相等的原位融合是防止畸形进一步恶化和避免致命并发症的有效选择。
OBJECTIVE: To describe the surgical treatment in a patient with a partial omega deformity in the thoracic spine with neurofibromatosis type 1.
METHODS: The patient was a 55-year-old man with an omega deformity, which is defined as a curvature in which the end vertebra is positioned at the level of, above, or below the apical vertebra (i.e., a horizontal line bisecting it). We performed halo gravity traction (HGT) for 7 weeks, followed by posterior spinal instrumented nearly equal in situ fusion from T2-L5 with three femoral head allografts and a local bone autograft. We avoided reconstruction of the thoracic anterior spine because of his severe pulmonary dysfunction.
RESULTS: HGT improved the % vital capacity from 32.5 to 43.5%, and improved the Cobb angle of the kyphosis from > 180° before traction to 144° after traction. The Cobb angle of kyphosis and scoliosis changed from > 180° preoperatively to 155° and 146°, respectively, postoperatively, and 167° and 156°, respectively, at final follow-up. His postoperative respiratory function deteriorated transiently due to bilateral pleural effusions and compressive atelectasis, which was successfully treated with a frequent change of position and nasal high flow for 1 week. At final follow-up, his pulmonary function improved from 0.86 to 1.04 L in VC, and from 32.5 to 37.9% in %VC. However, there was no overall improvement in preoperative distress following surgery, although his modified Borg scale improved from 3 preoperatively to 0.5 postoperatively. One month after discharge, he felt worsening respiratory distress (SpO2:75%) and was readmitted for pulmonary hypertension for 2 months. He was improved by non-invasive positive pressure ventilation (biphasic positive airway pressure) for 1 week, medication and daily lung physiotherapy. Thereafter, he has been receiving permanent daytime (0.5 L/min) and nighttime (2 L/min) oxygen therapy at home. A solid arthrodesis through the fusion area was confirmed on computed tomography. However, the kyphosis correction loss was 12° (i.e., 155°-167°), while the scoliosis correction loss was 10° (i.e., 146°-156°) at 2 years of recovery.
CONCLUSIONS: We suggest that nearly equal in situ fusion is a valid option for preventing further deformity deterioration and avoiding fatal complications.