Sponseller P D, Bhimani M, Solacoff D, Dormans J P
Johns Hopkins Hospital, Baltimore, Maryland; and the Children's Hospital of Philadelphia, Pennsylvania, USA.
Spine (Phila Pa 1976). 2000 Sep 15;25(18):2350-4. doi: 10.1097/00007632-200009150-00013.
Retrospective review of a defined Marfan population with traditional indications for bracing.
To determine the success rate of brace treatment in keeping curves from progressing by more than 5 degrees or exceeding 45 degrees.
Few studies exist regarding brace treatment of Marfan syndrome, and they include many patients with curves of more than 45 degrees, as well as some who are near maturity. All of the prior studies risk the possibility of some selection bias.
Patients were selected from support groups and several institutions. Inclusion criteria were: Definite diagnosis of Marfan syndrome, curve of 45 degrees or less, Risser sign 2, 1, or 0 at inception of bracing, recommended wear of 18 hours or more per day, and follow-up until maturity or surgery (minimum, 2 years). Success was defined as curve progression of 5 degrees or less and final curve remaining 45 degrees or less. Failure was a final curve of more than 45 degrees. Twenty-four patients met the criteria. There were 15 girls and 9 boys. Twenty-two patients wore a brace as recommended. Two additional patients were unable to tolerate it.
Mean age at inception of bracing was 8.7 years (range, 4-12 years). There were 14 double major, 6 thoracic, and 4 thoracolumbar curves with a mean size of 29 degrees at the beginning of bracing. The stated wearing time averaged 21 hours per day. Five patients had significant pain over bony prominences. Although correction of the curve in brace was good (45%), only 4 of the patients had success, and in 20 of the 24 treatment was considered a failure. Mean progression was 6 degrees +/- 8 degrees per year, for a final mean curve of 49 degrees. Sixteen of the patients had, or were advised to have, surgical correction. The difference in age and degree of curvature were not statistically significant between the success and nonsuccess groups.
The success rate for brace treatment of Marfan scoliosis is 17%, which is lower than that reported for idiopathic scoliosis. Possible reasons include increased progressive forces, altered transmission of corrective pressure to the spine, and younger age at inception of bracing. Because there was no control group, it is unknown whether bracing slowed curve progression. Physicians should understand that most patients with Marfan syndrome who have a curve of more than 25 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.
对有传统支具治疗指征的特定马凡氏综合征患者群体进行回顾性研究。
确定支具治疗在防止侧弯进展超过5度或超过45度方面的成功率。
关于马凡氏综合征支具治疗的研究很少，且这些研究纳入了许多侧弯超过45度的患者以及一些接近成熟年龄的患者。所有先前的研究都存在一定选择偏倚的可能性。
从支持小组和多个机构中选取患者。纳入标准为：明确诊断为马凡氏综合征，侧弯45度或以下，开始支具治疗时Risser征为2级、1级或0级，建议每天佩戴18小时或更长时间，随访至成熟或手术（至少2年）。成功定义为侧弯进展5度或以下且最终侧弯保持在45度或以下。失败定义为最终侧弯超过45度。24名患者符合标准。其中有15名女孩和9名男孩。22名患者按建议佩戴支具。另外两名患者无法耐受。
开始支具治疗时的平均年龄为8.7岁（范围4 - 12岁）。有14例双主弯、6例胸弯和4例胸腰段侧弯，支具治疗开始时平均角度为29度。规定的佩戴时间平均每天21小时。5名患者在骨突处有明显疼痛。尽管支具内侧弯矫正效果良好（45%），但只有4例患者成功，24例治疗中有20例被认为失败。平均每年进展6度±8度，最终平均侧弯为49度。16例患者已经或被建议进行手术矫正。成功组和未成功组在年龄和侧弯程度上的差异无统计学意义。
马凡氏综合征脊柱侧弯支具治疗的成功率为17%，低于特发性脊柱侧弯的报道成功率。可能的原因包括进展力增加、矫正压力向脊柱的传递改变以及开始支具治疗时年龄较小。由于没有对照组，尚不清楚支具是否减缓了侧弯进展。医生应明白，大多数侧弯超过25度且Risser征为2级或以下的马凡氏综合征患者即使接受支具治疗也会达到手术范围。
