Motono Nozomu, Kawaguchi Masahito, Kawahara Norio, Uramoto Hidetaka
Department of Thoracic Surgery, Kanazawa Medical University, Ishikawa, Japan.
Department of Orthopedic Surgery, Kanazawa Medical University, Ishikawa, Japan.
Int J Surg Case Rep. 2018;53:168-170. doi: 10.1016/j.ijscr.2018.10.071. Epub 2018 Nov 1.
Neurofibromatosis type 1 (NF-1) is often associated with various orthopedic disorders, especially scoliosis. Spinal deformity in patients with NF-1 can be induced by localized neurofibromas.
Scoliosis can be induced by a localized neurofibroma. A 12-year-old boy diagnosed with neurofibromatosis type 1 had severe scoliosis caused by a neurofibroma located in the posterior mediastinum. We performed two-stage procedure involving the extirpation of the neurofibroma in the lateral position, and posterior spinal fusion with segmental spinal instrumentation for the scoliosis in the prone position.
The optimum surgical approach for the extirpation of neurofibroma located in the posterior mediastinal remains to be established. It may be difficult to extirpate a tumor in this position via a posterior approach alone, as the tumor is surrounded by several tissue, e.g. azygos vein, trachea and esophagus. In our case, we first extirpated the tumor located in the posterior mediastinum via a posterolateral incision in the right lateral position. With this approach, it may be easy to confirm the safe relationship between the tumor and surrounding tissue.
We safely performed a two-stage procedure involving the extirpation of a neurofibroma in the lateral position, and posterior spinal fusion with segmental spinal instrumentation for scoliosis in the prone position.
1型神经纤维瘤病(NF-1)常与多种骨科疾病相关,尤其是脊柱侧弯。NF-1患者的脊柱畸形可由局部神经纤维瘤引起。
脊柱侧弯可由局部神经纤维瘤引起。一名12岁被诊断为1型神经纤维瘤病的男孩患有严重脊柱侧弯,由位于后纵隔的神经纤维瘤所致。我们分两阶段进行手术,包括在侧卧位切除神经纤维瘤,以及在俯卧位对脊柱侧弯进行后路脊柱融合并使用节段性脊柱内固定。
位于后纵隔的神经纤维瘤切除的最佳手术入路仍有待确定。仅通过后路入路切除该部位的肿瘤可能困难,因为肿瘤被多种组织包围,如奇静脉、气管和食管。在我们的病例中,我们首先通过右侧卧位的后外侧切口切除位于后纵隔的肿瘤。采用这种入路,可能更容易确认肿瘤与周围组织之间的安全关系。
我们安全地进行了分两阶段的手术,包括在侧卧位切除神经纤维瘤,以及在俯卧位对脊柱侧弯进行后路脊柱融合并使用节段性脊柱内固定。