Non-ionizing measurement and quantification of bell-shaped chests in spinal muscular atrophy: a pilot study.

Abstract

Spinal Muscular Atrophy (SMA) is manifested by deformation of the chest wall, including a bell-shaped chest. We determined the ability of a novel non-ionizing, non-volitional method to measure and quantify bell-shaped chests in SMA. A 3D depth camera and a chest x-ray (CXR) were used to capture chest images in 14 SMA patients and 28 controls. Both methods measure the distance between two points, but measurements performed by 3D analysis allow for the consideration of the curve of a surface (geodesic measurements), whereas the CXR allows solely for the determination of the shortest path between two points, with no regard for the surface (Euclidean measurements). The ratio of the upper to lower chest distances was quantified to distinguish chest shape in imaging by both the 3D depth camera and the CXR, and the ratios were compared between healthy and SMA patients. The mean 3D Euclidean ratio of distances measured by 3D imaging was 1.00 in the control group and 0.92 in the SMA group (p = 0.01), the latter indicative of a bell-shaped chest. This result repeated itself in the ratio of geodesic measurements (0.99 vs. 0.89, respectively, p = 0.03). The herein-described novel, noninvasive 3D method for measuring the upper and lower chest distances was shown to distinguish the bell-shaped chest configuration in patients with SMA from the chests of controls. This method bears several advantages over CXR and may be readily applicable in clinical settings that manage children with SMA.