Diastematomyeliaの2症例

TWO CASES OF DIASTEMATOMYELIA

I．はじめに

　Diastematomyeliaは神経軸neural axis，と脊椎骨の先天性の発生異常によるもので，脊髄が骨性，軟骨性，繊維性の中隔によつて縦てに2分され，それぞれ別々のクモ膜や時に硬膜に包まれている状態をいう。

　Diastematomyeliaはしばしば皮膚，骨，中枢神経系の先天性の異常を伴い，進行性の脊髄障害をおこすことがある。欧米での報告は多いが，本邦での報告例は少く著者らの探し得たかぎり前田^1）らの1例，Ii^2）らの1剖検例，石上^3）らのこれを疑う1例が報告されているにすぎない。最近著者らは進行性の側彎を主訴として来院した2例のdiastematornyeliaを経験したのでここに報告する。

Diastematomyelia is a bifid state of the spinal cord due to the presence of a septum of bone, cartilage, or fibrous tissue. We reported here two cases of diastematomyelia presenting as a progres-sive scoliosis.

Case 1 was a 7-year-old boy, his abnormal spinal curvature was found soon after the birth. Because of progressive scoliosis and limping gait, he was admitted in Chiba University Hospital. On ad-mission following findings were noted, thoracic scoliosis with convexity to the right, short right leg, foot, and sacral dimple. X-ray examination of the spine revealed extensive spina bifida, increased interpedicular distances, abnormal segmentation of vertebral bodies, and midline bony spurs. Lami-nectomy confirmed the presence of two spinal cords within the separate dural sacs. Three midline bony spurs were located at Tv8, Tv10, Lv2, re-spectively. Unfortunately complete removal of the septa resulted in paraplegia.

Case 2 was 7-year-old girl, her abnormal spinal curvature and hypertrichosis were found soon after the birth. Just like the Case 1, progressive scoliosis and unstable gait brought her to the Hospital. On admission lumbar hypertrichosis, sacral dimple, and small left foot were noted. X-ray of the spine revealed 65 degree of thoracic scoliosis convexity to the left, extensive spina bifida, increased inter-pedicular distances, and multiple abnormal seg-mentation of vertebral columns.

Myelography demonstrated filling defect at Lv2. Operation confirmed the presence of bifid state of the spinal cord by fibrous tissue for 8 cm long. Conus medullaris was situated at Lv5.

Complete removal of the septum was successful without any complication.