Non-ionizing measurement and quantification of bell-shaped chests in
spinal muscular atrophy: A pilot study
Abstract
Background 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. Methods A 3D
depth camera and a chest X-ray (CXR) were used to capture chest images
in 14 SMA patients and 28 controls. Euclidean and geodesic chest and
abdominal distances were measured from the 3D images, and horizontal
distances were measured from the CXR images. The ratio of the chest to
abdominal distances was used to quantify chest shape in both the 3D
depth camera and the CXR, and both were compared between healthy and SMA
patients. Results The mean 3D Euclidian ratio of distances 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 geodesic measurements (0.99 vs. 0.89, respectively,
p = 0.03). Conclusion The herein-described novel
noninvasive 3D method for measuring the chest and abdominal distances
was shown to distinguish the bell-shaped chest configuration in patients
with SMA from the normal chests of controls. This method bears several
advantages over CXR and may be readily applicable in clinical settings
that manage children with SMA.