Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI

• Published in: PloS one Vol. 11; no. 7; p. e0158912
• Main Authors: Mogalle, Katja, Perez-Rovira, Adria, Ciet, Pierluigi, Wens, Stephan C. A., van Doorn, Pieter A., Tiddens, Harm A. W. M., van der Ploeg, Ans T., de Bruijne, Marleen
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 08.07.2016; Public Library of Science (PLoS)
• Subjects: Adult; Aged; Biology and Life Sciences; Breathing; Care and treatment; Complications and side effects; Damage prevention; Data processing; Departments; Development and progression; Diaphragm; Diaphragm (anatomy); Diaphragm - diagnostic imaging; Diaphragm - physiopathology; Disease; Feature extraction; Female; Geometry; Glycogen Storage Disease Type II - diagnostic imaging; Glycogen Storage Disease Type II - physiopathology; Glycogen storage diseases; Health informatics; Humans; Image analysis; Image detection; Image processing; Image registration; Imaging, Three-Dimensional - methods; Kinematics; Lungs; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Male; Mechanics (physics); Medical imaging; Medical treatment; Medicine; Medicine and Health Sciences; Metabolic disorders; Middle Aged; Motion perception; Movement; Muscles; Myopathy; NMR; Nuclear magnetic resonance; Patients; Pediatrics; Pulmonary functions; Radiology; Research and Analysis Methods; Respiration; Respiratory function; Respiratory Mechanics; Respiratory system; Thorax; Three dimensional imaging; Netherlands
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0158912

Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness. The proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle. Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls. Dynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response.