Segmental spinal dysgenesisの1例

Segmental Spinal Dysgenesis. Case Report

　Segmental spinal dysgenesis（分節性脊椎形成異常症）は主に胸椎，腰椎に発生する脊椎無形成症もしくは形成不全症である．今回われわれは本症の1例を経験したので報告する．症例は3歳の男児．歩行障害，排尿障害を主訴に当院を受診した．画像にてL3椎体の前方低形成を認め，L4椎体は欠損し，リング状の椎弓のみが遺残していた．著明な局所後弯変形による脊髄の圧迫を認めたため，一期的に腰椎前後方合併除圧固定術を施行し，良好な結果を得た．本症例では脊髄空洞症と脂肪腫による脊髄係留の合併があるため，今後も注意深い経過観察が必要である．

　Segmental spinal dysgenesis is characterized by focal agenesis or dysgenesis of the lumbar or thoracolumbar spine, with abnormality of the underlying spinal cord and nerve roots. Here, we report a case of segmental spinal dysgenesis in the lumbar spine. A 3-year-old boy developed gait disturbance and neurogenic bladder and was referred to our clinic. CT demonstrated hypoplasia of the anterior L3 body and absence of the L4 body with a remnant ring-shaped lamina. MRI revealed severe local kyphosis and compression of the spinal cord that was tethered by a spinal lipoma. A favorable outcome was obtained by an anterior decompression and fibula strut graft followed by a posterior fusion with instrumentation. Because of the associated syringomyelia and tethering of the spinal cord, further careful observation is necessary.