The Journal of Bone and Joint Surgery, Vol 57, Issue 3 358-368, Copyright © 1975 by Journal of Bone and Joint Surgery, Inc
Scoliosis in Marfan's syndrome. Its characteristics and results of treatment in thirty-five patients
PR Robins, JH Moe and RB Winter
Of sixty-four patients with Marfan's syndrome, thirty-five had scoliosis,
which in 44 per cent began in the infantile and juvenile age periods. The
curve patterns were double major right thoracic-left lumbar in 48 per cent
and single right thoracic in 33 per cent. At the start of treatment the
curves were severe (average, 72 degrees) and quite structural (average
bending correction, 36 per cent). Seventy-four per cent of the thirty-five
patients had back pain. Inadequately treated and untreated curves usually
progressed to severe and painful deformity. Milwaukee brace treatment was
generally unsuccessful because of the severity and rigidity of the curves
or the poor maintenance of correction obtained. Spine fusion combined with
Harrington rod instrumentation (fourteen patients) gave an average
correction of 41 per cent with an average loss of 7 degrees (average
follow-up, 2.3 years). Operative treatment of scoliosis is indicated in
most patients with Marfan's syndrome.