Weiss Hans-Rudolf
Orthopedic Rehabilitation Services, Alzeyerstr, Germany.
Stud Health Technol Inform. 2012;176:383-6.
Little information exists about successful brace treatment of progressive early onset scoliosis. Even less information is available about the early treatment of scoliosis patients with Marfan's syndrome at age < 6 years. Purpose of this case report is to demonstrate the possibility of successful brace treatment in a patient with early onset scoliosis due to Marfan's syndrome.
A two year old girl diagnosed with Marfan's syndrome presented with a double major scoliosis of 20°. After a follow-up of 6 months she showed a rapid progression to 46° (November 2008) and was braced immediately. In-brace correction in the first Chêneau brace (RSC TM) was moderate due to the stiffness mainly of the lumbar curve. A new brace was made after significant growth (Gensingen braceTM in October 2009). An in-brace correction to 12° thoracic and 12° lumbar has been achieved. In October 2010 she also has outgrown her second brace to some extent. Due to clinical overcorrection (ATR lumbar -5°) brace wearing time has been reduced to 12 hrs. / day at first. In January 2011 at the age of 4 and a half she presented again with an ATR lumbar of -6° still overcorrected clinically, so we decided to leave off the brace for 3 months time. The deformity returned and we had to make a new brace in April 2011. For brace construction a new x-ray has been made showing the curve meanwhile has been reduced to 24° Cobb, however still with significant wedging of the apical vertebra.
(1) Successful brace treatment in infantile / juvenile patients with scoliosis is possible. (2) When treated during periods of rapid growth corrections can be achieved with high correction braces. (3) Before early surgery is performed high quality conservative management seems indicated.
关于成功使用支具治疗进行性早发性脊柱侧弯的信息很少。关于6岁以下患有马凡氏综合征的脊柱侧弯患者的早期治疗信息更少。本病例报告的目的是证明对一名因马凡氏综合征导致早发性脊柱侧弯的患者成功进行支具治疗的可能性。
一名2岁被诊断为马凡氏综合征的女孩出现了20°的双主弯脊柱侧弯。随访6个月后，她的侧弯迅速进展至46°（2008年11月），并立即佩戴支具。由于主要是腰椎曲度的僵硬，在第一个Chêneau支具（RSC TM）中的支具内矫正效果一般。在显著生长后制作了一个新的支具（2009年10月的Gensingen支具TM）。已实现支具内矫正至胸椎12°和腰椎12°。2010年10月，她也在一定程度上长得超过了第二个支具。由于临床过度矫正（腰椎ATR -5°），起初支具佩戴时间减少至12小时/天。2011年1月，4岁半的她再次出现腰椎ATR为-6°，临床仍为过度矫正，所以我们决定停用支具3个月。畸形复发，我们不得不在2011年4月制作一个新的支具。为制作支具拍摄了新的X光片，显示此时侧弯已降至24° Cobb，但顶椎仍有明显楔形变。
（1）对婴儿/青少年脊柱侧弯患者成功进行支具治疗是可能的。（2）在快速生长期间进行治疗时，使用高矫正支具可以实现矫正。（3）在进行早期手术之前，似乎需要高质量的保守治疗。
