高度後側弯を呈した神経線維腫症の1例

Dystrophic Spinal Neurofibromatosis with Hyperkyphoscoliosis : Case Report

　抄録：神経線維腫症に伴う高度後側弯に対し二期的前後合併手術を行った症例を報告する．症例は1歳時に神経線維腫症と診断された14歳男児で，単純X線上T4-10で後弯120度，側弯56度と高度の胸椎後側弯が認められた．手術はまずT4-8の前方解離術を行い，2週間の頭蓋直達牽引後に，インストゥルメントを用いたT1-L1後方固定術，および前方遊離腓骨移植術を行った．後弯は65度，側弯32度まで矯正され，神経麻痺，血管損傷など合併症はなかった．術後4年10カ月の現在，後弯は75度で，良好な骨癒合と矯正が得られている．神経線維腫症に伴う高度な脊柱変形に対する矯正固定術では，脊髄麻痺や大血管損傷などの重大な合併症が報告されている．われわれは確実な前方解離と直達牽引による整復，高さの低いインストゥルメントの選択により，合併症を防ぎ良好な整復・骨癒合を得ることができた．

　We present a case of dystrophic spinal deformity associated with neurofibromatosis treated with two staged combined anterior and posterior spinal fusion. A 14-year-old boy who had been diagnosed as neurofibromatosis when he was one year old, presented severe thoracic deformity with the kyphosis measured 120°and the scoliosis 56°between T4-10. He underwent anterior resection of the disc and ligament as a first stage operation. After the direct head traction for two weeks, combined anterior bone grafting and posterior instrumentation using sublaminar wiring and lamina hooks were performed. There were no major complications related to the surgery, such as iatrogenic spinal cord injury and fatal vascular injury. At the 4-year follow-up, a successful correction of the deformity in the radiographs was maintained. Sufficient anterior release, direct head traction, and using low profile instrumentation contributed to sufficient correction with solid fusion and no major complications.