Becker母斑症候群に伴った側弯症の1例

Becker's Nevus Syndrome Associated with Scoliosis. Case Report

　症例は14歳の女性で主訴は体幹変形であった．受診の2カ月前から体幹部変形を自覚し，脊柱側弯症と診断された．脊柱の側弯変形の他に左乳房の低形成，左肩・左腰部に有毛性の広範な褐色母斑を認めた．単純X線像では主胸椎部に右凸の74°のカーブを認めた．臨床所見および皮膚病理所見から，本症例をBecker母斑症候群と診断した．手術は椎弓根スクリューを用いた後方矯正固定術を施行した．術後胸椎カーブは13°となり，矯正率は82.4％であった．1995年にHappleらは，Becker母斑に側弯症・二分脊椎などの脊柱変形を認める病態をBecker母斑症候群として提唱したが，現在までに側弯症に対する手術を要した症例の報告はない．本症例は母斑の出現も広範であり，強い表現型であったため側弯が進行した可能性があった．術後経過は順調であり経過観察中である．

　Introduction：Patients with Becker's nevus syndrome are known to often have scoliosis, but there have been no reports of cases in which corrective surgery has been performed. Case：A 14-year-old female was referred to our hospital for treatment of scoliosis. Physical examination revealed brown patces with hair over a wide area extending from the left shoulder to the left thigh. The left breast was hypoplastic. Radiograms revealed a right thoracic curve T5-T11) and a left dorsolumbar curve (T11-L4) with a Cobb' angle of 74 degrees and 51 degrees, respectively. A diagnosis of scoliosis associated with Becker's nevus syndrome was made, and posterior correction and fusion with a pedicle screw system were performed. Postoperatively, the thoracic curve and dorsolumbar curve were reduced to 13 degrees and 7 degrees, respectively. Discussion：The scoliosis that occurs in association with Becker's nevus syndrome is usually mild, and surgical treatment is unnecessary. This is the first report of a case in which the patient was treated by posterior correction and fusion. The surgical result at ten months was favorable, but further follow-up is necessary.