Marfan症候群に伴う脊柱後側弯症の治療経験

Surgical Treatment for Kyphosing Scoliosis in Marfan Syndrome. Case Report

　Marfan症候群に対する手術治療は，易出血性で心血管系異常を伴うため合併症発生率も非常に高い．筆者らは高度の脊柱後側弯（kyphosing scoliosis）を呈し，血液型がO型Rh（－）であったため治療に難渋した症例を経験したので報告する．症例は12歳6カ月の男児で胸椎カーブ81°，胸腰椎カーブ106°のダブルメジャーカーブを認め，側面像にて46°の後弯を認めた．手術は二期的に計画したが，術中出血の多さから三期的（前方―後方―前方矯正固定術）にせざるを得なかった．Marfan症候群の側弯手術は固定範囲が広く侵襲も大きい．またそのため術前の全身および脊柱変形の評価，また小児循環器科，麻酔科との連携が非常に重要である．

　Marfan syndrome (MFS) is a one of the most common connective tissue disorders, and increased blood loss and rates of cardiovascular complications are frequently encountered during surgical interventions in MFS. We report a case of MFS and kyphosing scoliosis in which surgical treatment was difficult because of severe deformity and massive bleeding, due to the fact that the patient's blood type was O, Rh (－). Case：The patient was a twelve and a half-year-old male with severe kyphosing scoliosis. The preoperative measurements showed 106°of scoliosis and 46°of kyphosis. Comorbidity included mitral regurgitation and constrictive respiratory dysfunction. A two-stage anterior and posterior combined operation was planned, but because of massive bleeding during the posterior operation, it was interrupted just after correction of the scoliosis, and a third operation after recovery of the patient's general condition was planned. The scoliosis had been corrected from 106°to 23°at the time of the final follow-up examination, and it had been corrected 80.2％. A preoperative cardiac evaluation and carefully planned surgery with the support of a cardiologist and anesthesiologist is recommended to prevent morbidity in MFS patients undergoing surgery for a spinal deformity.