Schlösser Tom P C, Semple Tom, Carr Siobhán B, Padley Simon, Loebinger Michael R, Hogg Claire, Castelein René M
Department of Orthopaedic Surgery, G05.228, University Medical Center Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands.
Department of Radiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
Eur Spine J. 2017 Jun;26(6):1595-1599. doi: 10.1007/s00586-017-4970-5. Epub 2017 Feb 8.
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.
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.
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.
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.
原发性纤毛运动障碍(PCD)是一种呼吸综合征,其中可能会出现“随机”的器官定向;约46%的患者在器官发生时会出现全内脏转位。本研究的目的是通过研究有无内脏转位的PCD患者特发性脊柱侧凸的患病率和凸侧,探讨器官解剖结构与脊柱侧凸凸侧之间的关系。
使用Cobb角系统筛查PCD患者的胸部X线片,以确定是否存在明显的脊柱侧方偏移。正值表示右侧凸。比较有内脏转位和正常解剖结构的PCD患者的脊柱侧凸凸侧和Cobb角。
共筛查了198例PCD患者。脊柱侧凸(Cobb>10°)和明显脊柱不对称(Cobb 5-10°)的患病率分别为8%和23%。内脏转位患者和正常解剖结构患者两组内的脊柱侧凸凸侧和Cobb角均有显著差异(P≤0.009)。此外,脊柱侧凸凸侧与器官定向显著相关(P<0.001;ϕ=0.882):在16例患有脊柱侧凸的PCD患者(8例内脏转位和8例正常解剖结构)中,除1例病例外,观察到脊柱侧凸凸侧与器官解剖结构定向相匹配:脊柱侧凸的凸侧与器官定向相反。
本研究支持我们关于脊柱侧凸中器官解剖结构与脊柱侧凸凸侧之间相关性的假设:在“随机”为正常器官解剖结构的PCD患者中,胸段脊柱侧凸的凸侧主要在右侧,而在全内脏转位患者中则在左侧。