シンケイ センイショウ 1ガタ ノ セキチュウ ヘンケイ ニ トモナウ ロッコツ ズ ノ セキチュウカン ナイ ダッキュウ

2006 年4 月～2010 年3 月の神経線維腫症1 型に伴う脊柱変形手術例10 例中3 例に肋骨頭の脊柱管内脱臼がみられた.症例1 は6 歳の男児でT4-8:74°,T8-11:72° の側弯があり,左第4 と第5 肋骨頭の脊柱管内への陥入を認めた.症例2 は11 歳の男児でT3-7:100° の側弯とT3-T9:90° の後弯があり,左第6 肋骨頭が脊柱管内へ脱臼し,脊髄を圧排していた.症例3 は27 歳の女性でT6～9:73°,T9～12:68° の側弯とT6-10:91°の後弯を認め,右第7 肋骨頭の脊柱管内脱臼がみられた.本病態の報告例は少ないが,決して希な病態ではなくdystrophic change を伴う脊柱変形においては,肋骨頭の脊柱管内脱臼による潜在的リスクに注意する必要があると考える.Spinal deformities are common features in neurofibromatosistype-1( NF-1). Several types of deformities have been reported, however, intraspinal rib head dislocation (IRD)due to dystrophic change is very rare and not well understood. Between April 2006 and March 2010, we experienced3 patients with IRD out of 10 consecutive patients who underwent surgical treatment for dystrophic spinal deformities in NF-1. Case 1:A 6-year-old boy who had 74 ° short angular scoliosis at T4-8 and 72 ° at T8-11 underwent surgery in our institute. CT myelography demonstrated dystrophic changes with for aminal enlargement, vertebral body scalloping, rib penciling and mild IRD. He underwent the Growing Rod techniques and finally underwent posterior spinal fusion (PSF) from T1 to L2 combined with prophylactic rib head resection followed by anterior spinal fusion(ASF) with autologous fibular strut graft. No neurological complications were observed postoperatively and complete bony fusions were achieved. Case 2:Neurologically intact11-year-old boy presented with spinal deformity and caféau-lait spots. Radiography showed 100 ° scoliosis at T3-7and 72 ° at T3-9. CT myelography demonstrated dystrophic changes and IRD and impingement of spinal cord. He underwent PSF with removal of the T6 rib head followed by ASF. Case 3:A 27-year-old woman presented with back pain and spinal deformities. Radiography showed 73 °scoliosis at T6-9 and 91 ° at T9-12. CT myelography demonstrated dystrophic changes and IRD. She also underwentPSF with removal of T7 rib head followed by ASF. AllIRDs were observed at the apex of the convex side of scoliosis.It has a potential risk of spinal cord compression and may cause paraplegia or paraparesis. Therefore, high degree of suspicion is warranted for the treatments of scoliosis with intraspinal rib displacement in NF-1

カンイ ヨウツイ カドウイキ ソクテイキ ノ セイカクセイ ト シンライセイ ノ ケントウ

【目的】腰椎可動性の評価は腰痛性疾患の診断や治療効果判定に際し重要である.簡単な取り付け器具で体幹に装着し, X線撮影なしに腰椎可動域を評価できる簡易腰椎可動域測定器を開発し,測定データの正確性および信頼性を評価したので報告する.【方法】簡易腰椎可動域測定器は,金属製の固定板,その中央に垂直に取り付けられた計測バー,固定板を体幹に確実に固定するゴムバンドより構成されている.第12胸椎と仙骨上の体幹背面に装着した測定器による可動域と実際の腰椎可動域をX線およびCTを用いて計測し,正確性,測定者間および測定者内の一致度および再現性を検討した.【結果】前後屈および回旋可動域に関しては測定値とX線計測値はよく相関し,かつ,その平均値に有意差はないことから正確性は良好であった.一方,前後屈の各体位における測定値とX線計測値には有意差があること,各回旋位の計測値の相関関係にはばらつきが多かったことから,本測定器は腰椎の肢位をX線と同様に捉えていなかった.また,側屈可動域に関しては,測定値とX線計測値の相関は前後屈や回旋より劣り,かつ,その計測値にも有意差が見られたことから,側屈可動域に関する本測定器の正確性は不十分であった.さらに,測定者間および測定者内一致度はすべての項目で高く再現性も認められた.【結論】前後屈および回旋可動域は簡易腰椎可動域測定器により低コストで簡便に測定でき,その正確性や信頼性も優れていた.Purpose : It is important to evaluate mobility of the lumbar spine for assessment of lumbar spinal disorders and their therapeutic effects. We developed a simple measurement device for the lumbar range of motion (SMD-L-ROM) which can be installed on the trunk. SMD-L-ROM allows to evaluate mobility of the lumbar spine without x-ray exposure. Accuracy and reliability of the measurement device was assessed in this article. Method : SMD-L-ROM comprises two metallic immobilization plates (IPs) with a vertically installed bar and a rubber band for fixation of IP to the trunk. Two IPs were installed at the level of Th12 and the sacrum. Range of motion (ROM) of the lumbar spine was measured by using SMD-L-ROM and radiographic modalities (X-ray and CT). Consistency of the measured values by both of SMD-L-ROM and radiographic modalities was evaluated. Furthermore, inter- and intra-observer agreement of measured values by SMD-L-ROM was assessed. Results : Regarding measurement of ROM, there was a high correlation between SMD-L-ROM and X-ray/CT especially in the sagittal and axial plane. There was no significant difference in the average values of ROM between the two methods. On the other hand, SMD-L-ROM did not detect accurate position of the lumbar spine in all dimensions. There were no inter- and intra-observer errors of measured values by SMD-L-ROM. Conclusion : ROM of the lumbar spine was simply and economically measured with SMD-L-ROM. Accuracy and reliability of SMD-L-ROM was good enough for ROM measurement in the lumbar spine