Tom P C Schlösser1, Tom Semple2, Siobhán B Carr3, Simon Padley2, Michael R Loebinger4, Claire Hogg1,2,3,4, René M Castelein5. 1. Department of Orthopaedic Surgery, G05.228, University Medical Center Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands. 2. Department of Radiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK. 3. Department of Pediatric Respiratory Medicine, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, UK. 4. Host Defence Unit, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, UK. 5. Department of Orthopaedic Surgery, G05.228, University Medical Center Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands. r.m.castelein@umcutrecht.nl.
Abstract
PURPOSE: Primary 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. METHODS: Chest 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. RESULTS: A 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. CONCLUSIONS: 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 PCD patients that were 'randomized' to normal organ anatomy and to the left in patients with situs inversus totalis.
PURPOSE:Primary 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 PCDpatients with and without situs inversus. METHODS: Chest radiographs of PCDpatients 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 PCDpatients with situs inversus and normal anatomy. RESULTS: A total of 198 PCDpatients 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 PCDpatients 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. CONCLUSIONS: 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 PCDpatients that were 'randomized' to normal organ anatomy and to the left in patients with situs inversus totalis.
Entities:
Keywords:
Curve convexity; Primary ciliary dyskinesia; Scoliosis; Situs inversus totalis; Situs solitus
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