Abstract
PurposePrimary ciliary dyskinesia (PCD) is a respiratory syndrome in which ‘random’ organ orientation can occur; with approximately 46% of patients developing situs inversus totalis at organogenesis. The aim of this study was to explore the relationship between organ anatomy and curve convexity by studying the prevalence and convexity of idiopathic scoliosis in PCD patients with and without situs inversus.MethodsChest radiographs of PCD patients were systematically screened for existence of significant lateral spinal deviation using the Cobb angle. Positive values represented right-sided convexity. Curve convexity and Cobb angles were compared between PCD patients with situs inversus and normal anatomy.ResultsA total of 198 PCD patients were screened. The prevalence of scoliosis (Cobb >10°) and significant spinal asymmetry (Cobb 5–10°) was 8 and 23%, respectively. Curve convexity and Cobb angle were significantly different within both groups between situs inversus patients and patients with normal anatomy (P ≤ 0.009). Moreover, curve convexity correlated significantly with organ orientation (P < 0.001; ϕ = 0.882): In 16 PCD patients with scoliosis (8 situs inversus and 8 normal anatomy), except for one case, matching of curve convexity and orientation of organ anatomy was observed: convexity of the curve was opposite to organ orientation.ConclusionsThis study supports our hypothesis on the correlation between organ anatomy and curve convexity in scoliosis: the convexity of the thoracic curve is predominantly to the right in PCD patients that were ‘randomized’ to normal organ anatomy and to the left in patients with situs inversus totalis.
Highlights
Idiopathic scoliosis is a three-dimensional (3-D) deformity of the spine and trunk that primarily affects previously healthy children during the adolescent growth spurt, without any underlying known pathology [1]
This study supports our hypothesis on the correlation between organ anatomy and curve convexity in scoliosis: the convexity of the thoracic curve is predominantly to the right in Primary ciliary dyskinesia (PCD) patients that were ‘randomized’ to normal organ anatomy and to the left in patients with situs inversus totalis
Information about curve convexity in situs inversus patients could increase our understanding of the factors that are involved in the convexity of idiopathic scoliosis
Summary
Idiopathic scoliosis is a three-dimensional (3-D) deformity of the spine and trunk that primarily affects previously healthy children during the adolescent growth spurt, without any underlying known pathology [1]. Incidental reports describe that, in patients with situs inversus totalis or dextrocardia and scoliosis, the curve pattern is the opposite of that found normally: a left convex thoracic curve instead of right convex [5,6,7,8]. These reports generate the hypothesis that the convexity of the curve in scoliosis is to the right in patients with normal organ anatomy (situs solitus) and to the left in patients with situs inversus totalis. Information about curve convexity in situs inversus patients could increase our understanding of the factors that are involved in the convexity of idiopathic scoliosis
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