Cervical rotation, chest deformity and pelvic obliquity in patients with spinal muscular atrophy

Agnieszka Stępień,Katarzyna Maślanko,Witold Rekowski,Łucja Mazurkiewicz,Maria Jędrzejowska

doi:10.1186/s12891-020-03710-7

Abstract

BackgroundMusculoskeletal disorders are often observed in patients with spinal muscular atrophy (SMA). The aim of the study was to assess passive ranges of rotation in the cervical spine, chest deformity and pelvic obliquity in SMA patients, and to compare these results to the norms obtained in the group of healthy individuals. The second aim was to review these measurements and Cobb angle values for correlations in SMA patients.MethodsThe study included 74 patients with SMA and 89 healthy individuals aged 2 to 18 years. Cervical Rotation (CR), Supine Angle of Trunk Rotation (SATR) and Pelvic Obliquity (PO) tests were carried out.ResultsCervical rotation ranges were significantly higher in the control group than in SMA patients (p < 0.05). Differences between cervical rotation ranges to the left and to the right were significantly larger in SMA I and SMA II groups than in healthy individuals (p = 0.000). Chest asymmetry and pelvic obliquity were bigger in SMA groups than in the control (p < 0.05). Significant correlations between cervical rotation measurements, chest deformity, pelvic obliquity and Cobb angle were found in SMA individuals, depending on the type.ConclusionsThe results of the study suggest that CR, SATR and PO tests may assist in the assessment of SMA patients in addition to the radiographic evaluation of the spine. Biomechanical relationships between disorders located in various skeletal structures should be taken into account in the treatment of SMA patients. Special attention should be given to assessing postural parameters in non- sitters and sitters. Treatment of patients with SMA and associated musculoskeletal disorders requires a multi-specialist approach.

Highlights

Musculoskeletal disorders are often observed in patients with spinal muscular atrophy (SMA)
The analysis showed significant differences between CRleft, Sum of cervical spine rotation on the left (CR left) and CR right (CRS), Supine Angle of Trunk Rotation Upper test (SATRU), Supine Angle of Trunk Rotation Lower test (SATRL) and Pelvic Obliquity (PO) values obtained by SMA patients with scoliosis and without scoliosis
This study has shown that Cervical Rotation (CR), Supine Angle of Trunk Rotation (SATR) and PO tests provide relevant information about body posture in SMA patients

Summary

Introduction

Musculoskeletal disorders are often observed in patients with spinal muscular atrophy (SMA). Spinal muscular atrophy may lead to many complications: musculoskeletal (scoliosis, chest deformities, joint contractures, hip dislocation), respiratory The spinal deformity is usually accompanied by lateral flexion and rotation of the cervical spine and head, chest deformity and pelvic obliquity [8, 13, 17,18,19,20] (Fig. 1a). These musculoskeletal impairments may affect the daily functioning of patients with SMA. Distortion of the rib cage, associated with scoliosis, contributes to respiratory disorders occurring in SMA patients [9, 10, 20, 22], while pelvic obliquity may affect the ability to maintain balance in a sitting position [13, 17, 20]

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