Association between joint hypermobility, scoliosis, and cranial base anomalies in paediatric Osteogenesis imperfecta patients: a retrospective cross-sectional study

BMC Musculoskeletal Disorders

Janna Waltimo-Sirén¹  Outi Mäkitie²  Heidi Arponen¹ 

[1] Department of Orthodontics, Institute of Dentistry, University of Helsinki, PO Box 41, FI-00014 Helsinki, Finland;Center for Molecular Medicine, Karolinska Institutet, and Department of Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden

关键词: Craniovertebral pathology;    Scoliosis;    Joint hypermobility;    Osteogenesis imperfecta;   

Others  :  1090653
DOI  :  10.1186/1471-2474-15-428

 received in 2014-06-10, accepted in 2014-12-08,  发布年份 2014

【 摘 要 】

Background

Joint hypermobility is a common clinical characteristic of patients with Osteogenesis imperfecta (OI), a disorder with serious comorbidities of scoliosis and cranial base anomalies. This study aimed at evaluating how prevalent joint hypermobility is in paediatric OI patients, and to find out whether it serves as a potential predictive marker of the different spinal complications; scoliosis and craniovertebral anomalies (basilar impression and basilar invagination).

Methods

In this cross-sectional one-center study we analysed retrospectively clinical patient records and radiographs of 47 OI patients, aged 1–19 years, some of whom were treated with bisphosphonates. Presence of joint hypermobility, scoliosis, and craniovertebral anomalies was recorded and possible connections between the phenomena were explored with correlation analysis.

Results

Joint hypermobility was found in 70% of the patients. Scoliosis and cranial base anomalies had developed in 26%. The presence of spinal complications was independent of the bisphosphonate treatment status and joint hypermobility.

Conclusions

Scoliosis and craniovertebral anomalies are strongly associated in paediatric OI patients. Joint hypermobility that is much more common appears, however, to be a poor predictor.

【 授权许可】

   
2014 Arponen et al.; licensee BioMed Central.

【 图 表 】

Figure 1.

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