大腿骨転子下骨折を生じたEhlers-Danlos症候群の1例

Subtrochanteric Fracture of the Femur in Ehlers-Danlos Syndrome. Case Report

　症例は41歳の男性である．生後まもなくEhlers-Danlos症候群(以下，EDS)と診断された．今回玄関先で転倒し，左大腿骨転子下骨折を受傷した．左大腿骨遠位部には骨折後の変形治癒が認められた．また，骨粗鬆症性変化が著しく，骨密度も著明に低下していた．髄内釘にCCGバンドを併用した骨接合術を行った．術後10カ月時点で骨癒合は良好である．骨脆弱性や変形治癒のため内固定方法や，内固定材料の選択に注意を要した．また，関節弛緩性，血管や皮膚の脆弱性などEDSの特徴に対する配慮が必要であった．EDSの骨脆弱性については様々な報告があるが，骨折リスクは決して低くないと考えるべきである．

　We report the case of a 41-year-old male with Ehlers-Danlos syndrome (EDS) who suffered a subtrochanteric fracture of the left femur.　He was diagnosed with EDS soon after birth.　His bone mineral density was very low, and he had a history of frequent fractures and malunion of a distal fracture of the left femur.　Intramedullary nailing was performed to treat the subtrochanteric fracture, and bony union was achieved by five months postoperatively.　Fixation devices had to be selected with care because of the malunion of the femoral fracture and bone fragility.　Close attention also had to be paid to joint hypermobility and soft tissue vulnerability.　Although no association between EDS and bone fragility has been demonstrated, careful surgical planning is required to prevent complications related to this congenital condition.