Hassani Sara, Tong Yubing, Lomer Nima Broomand, Udupa Jayaram K, Wu Caiyun, McDonough Joseph M, Gogel Samantha, Mayer Oscar H, Biko David M, Cahill Patrick J, Anari Jason B, Torigian Drew A
Medical Image Processing Group, Department of Radiology, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
The Wyss/Campbell Center for Thoracic Insufficiency Syndrome, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
Spine Deform. 2025 Sep;13(5):1507-1515. doi: 10.1007/s43390-025-01095-y. Epub 2025 May 6.
Thoracic insufficiency syndrome (TIS) impairs normal respiration and lung growth. In pediatric patients with or without neuromuscular disease (NMD), the relationship between thoracic structure and regional respiratory function remains understudied. We aimed to evaluate these interrelationships using dynamic magnetic resonance imaging (dMRI) and computed tomography (CT).
This study included 25 pediatric TIS patients: 8 with NMD and 17 without NMD. Thoracic dMRI and CT images were analyzed to quantify intercostal muscle (ICM) volume, skeletal muscle (SM) volume, lung volumes at end-inspiration and end-expiration, chest wall excursion volume, and diaphragm excursion volume. Right-to-left volumetric symmetry ratios were compared using unpaired t-testing, and correlations between structural and functional parameters were assessed using Pearson correlation analysis.
Lung and muscle volumes were generally larger in the TIS-NMD compared to TIS non-NMD, reflecting age-related differences, although lung tidal volumes and diaphragmatic and chest wall excursion volumes were generally similar. The degree of asymmetry reflected by diaphragmatic respiratory function symmetry ratios exceeded that of chest wall symmetry ratios in both groups, with greater disparity in TIS-NMD (3.6 vs 1.8 in TIS-NMD compared to 2.8 vs 2.5 in TIS non-NMD). Correlation between thoracic SM volume and tidal volume was stronger than the correlation between ICM volume and tidal volume in both groups. TIS-NMD patients demonstrated unique compensatory dynamics, including a negative correlation between SM volume and diaphragmatic excursion volume.
Distinct interrelationships exist between thoracic structure and function in TIS patients with and without NMD. These findings suggest that NMD significantly influences respiratory mechanics, emphasizing the role of SM and diaphragm interaction.
胸廓发育不全综合征（TIS）会损害正常呼吸和肺生长。在患有或未患有神经肌肉疾病（NMD）的儿科患者中，胸廓结构与局部呼吸功能之间的关系仍未得到充分研究。我们旨在使用动态磁共振成像（dMRI）和计算机断层扫描（CT）来评估这些相互关系。
本研究纳入了25例儿科TIS患者：8例患有NMD，17例未患有NMD。对胸廓dMRI和CT图像进行分析，以量化肋间肌（ICM）体积、骨骼肌（SM）体积、吸气末和呼气末肺体积、胸壁 excursion 体积和膈肌 excursion 体积。使用不成对t检验比较左右体积对称比，并使用Pearson相关分析评估结构和功能参数之间的相关性。
与非NMD的TIS患者相比，NMD的TIS患者的肺和肌肉体积通常更大，这反映了与年龄相关的差异，尽管肺潮气量、膈肌和胸壁 excursion 体积通常相似。两组中，膈肌呼吸功能对称比所反映的不对称程度超过胸壁对称比，NMD的TIS患者差异更大（NMD的TIS患者为3.6对1.8，而非NMD的TIS患者为2.8对2.5）。两组中，胸廓SM体积与潮气量之间的相关性强于ICM体积与潮气量之间的相关性。NMD的TIS患者表现出独特的代偿动力学，包括SM体积与膈肌 excursion 体积之间呈负相关。
患有和未患有NMD的TIS患者的胸廓结构与功能之间存在明显的相互关系。这些发现表明，NMD显著影响呼吸力学，强调了SM与膈肌相互作用的作用。
