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普拉德-威利综合征患者的脊柱侧弯保守治疗

Conservative scoliosis treatment in patients with Prader-Willi syndrome.

作者信息

Weiss H-R, Bohr S

机构信息

Asklepios Katharina Schroth Spinal Deformities Rehabilitation Centre, Korczakstr. 2, 55566 Bad Sobernheim, Germany.

出版信息

Stud Health Technol Inform. 2008;140:314-7.

Abstract

UNLABELLED

Patients with Prader-Willi syndrome often suffer from scoliosis of major degrees. Due to current literature surgical intervention seems the gold standard of treatment although the rate of complications in this condition are reported to be significantly higher than in patients with Adolescent Idiopathic Scoliosis. Purpose of this study was to reveal the effects of conservative treatment in this rare patient population.

MATERIALS AND METHODS

A case series of patients with this condition has been investigated to estimate as to whether Prader-Willi patients with scoliosis may benefit from conservative scoliosis management. 9 Patients with this condition have been found in our out-patient database. 5 of these retarded patients (3 girls, two boys) today are 19 years and older and therefore are without any significant residual growth. Average Cobb angle was 47 degrees (34 - 66 degrees) at 12 years, average observation time was 6.4 years.

RESULTS

Two of the five patients progressed. Average Cobb angle after follow-up was 52 degrees. No progression beyond 70 degrees has been found after cessation of growth. In one patient the curve deteriorated clearly after reducing brace wearing time and therefore was due to non-compliance.

CONCLUSIONS

Stabilisation of scoliosis due to Prader-Willi syndrome is possible by means of conservative management. To expose this patient population to the risks of surgical management seems not to be justified.

摘要

未标注

普拉德-威利综合征患者常患有严重程度的脊柱侧弯。根据现有文献,手术干预似乎是治疗的金标准,尽管据报道这种情况下的并发症发生率明显高于青少年特发性脊柱侧弯患者。本研究的目的是揭示保守治疗对这一罕见患者群体的效果。

材料与方法

对一组患有这种疾病的患者进行了调查,以评估患有脊柱侧弯的普拉德-威利患者是否能从保守的脊柱侧弯治疗中获益。我们在门诊数据库中发现了9例患有这种疾病的患者。其中5例发育迟缓患者(3名女孩,2名男孩)如今已年满19岁,因此没有任何显著的剩余生长。12岁时平均Cobb角为47度(34 - 66度),平均观察时间为6.4年。

结果

5例患者中有2例病情进展。随访后平均Cobb角为52度。生长停止后未发现超过70度的进展。1例患者在减少支具佩戴时间后曲线明显恶化,因此是由于不依从所致。

结论

通过保守治疗可以稳定普拉德-威利综合征所致的脊柱侧弯。让这一患者群体承受手术治疗的风险似乎不合理。

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