Assessment of Regional Functional Effects of Surgical Treatment in Thoracic Insufficiency Syndrome via Dynamic Magnetic Resonance Imaging

Background: Quantitative regional assessment of thoracic function would enable clinicians to better understand the regional effects of therapy and the degree of deviation from normality in patients with thoracic insufficiency syndrome (TIS). The purpose of this study was to determine the regional functional effects of surgical treatment in TIS via quantitative dynamic magnetic resonance imaging (MRI) in comparison with healthy children. Methods: Volumetric parameters were derived via 129 dynamic MRI scans from 51 normal children (November 2017 to March 2019) and 39 patients with TIS (preoperatively and postoperatively, July 2009 to May 2018) for the left and right lungs, the left and right hemi-diaphragms, and the left and right hemi-chest walls during tidal breathing. Paired t testing was performed to compare the parameters from patients with TIS preoperatively and postoperatively. Mahalanobis distances between parameters of patients with TIS and age-matched normal children were assessed to evaluate the closeness of patient lung function to normality. Linear regression functions were utilized to estimate volume deviations of patients with TIS from normality, taking into account the growth of the subjects. Results: The mean Mahalanobis distances for the right hemi-diaphragm tidal volume (RDtv) were −1.32 ± 1.04 preoperatively and −0.05 ± 1.11 postoperatively (p = 0.001). Similarly, the mean Mahalanobis distances for the right lung tidal volume (RLtv) were −1.12 ± 1.04 preoperatively and −0.10 ± 1.26 postoperatively (p = 0.01). The mean Mahalanobis distances for the ratio of bilateral hemi-diaphragm tidal volume to bilateral lung tidal volume (BDtv/BLtv) were −1.68 ± 1.21 preoperatively and −0.04 ± 1.10 postoperatively (p = 0.003). Mahalanobis distances decreased after treatment, suggesting reduced deviations from normality. Regression results showed that all volumes and tidal volumes significantly increased after treatment (p < 0.001), and the tidal volume increases were significantly greater than those expected from normal growth for RDtv, RLtv, BDtv, and BLtv (p < 0.05). Conclusions: Postoperative tidal volumes of bilateral lungs and bilateral hemi-diaphragms of patients with TIS came closer to those of normal children, indicating positive treatment effects from the surgical procedure. Quantitative dynamic MRI facilitates the assessment of regional effects of a surgical procedure to treat TIS. Level of Evidence: Diagnostic Level II. See Instructions for Authors for a complete description of levels of evidence.

[1]  D. Torigian,et al.  Automatic lung segmentation in dynamic thoracic MRI using two-stage deep convolutional neural networks , 2022, Medical Imaging.

[2]  D. Torigian,et al.  Thoracic quantitative dynamic MRI to understand developmental changes in normal ventilatory dynamics. , 2020, Chest.

[3]  A. Samdani,et al.  VEPTR Treatment of Early Onset Scoliosis in Children Without Rib Abnormalities: Long-term Results of a Prospective, Multicenter Study. , 2019, Journal of pediatric orthopedics.

[4]  Yubing Tong,et al.  4D image construction from free-breathing MRI slice acquisitions of the thorax based on a concept of flux. , 2020, Proceedings of SPIE--the International Society for Optical Engineering.

[5]  D. Torigian,et al.  Quantitative Dynamic Thoracic MRI: Application to Thoracic Insufficiency Syndrome in Pediatric Patients. , 2019, Radiology.

[6]  D. Torigian,et al.  Understanding Respiratory Restrictions as a Function of the Scoliotic Spinal Curve in Thoracic Insufficiency Syndrome: A 4D Dynamic MR Imaging Study. , 2018, Journal of pediatric orthopedics.

[7]  Aaron Fenster,et al.  A framework for Fourier‐decomposition free‐breathing pulmonary 1H MRI ventilation measurements , 2018, Magnetic resonance in medicine.

[8]  Li Feng,et al.  Simultaneous Evaluation of Lung Anatomy and Ventilation Using 4D Respiratory‐Motion‐Resolved Ultrashort Echo Time Sparse MRI , 2018, Journal of magnetic resonance imaging : JMRI.

[9]  F. Wacker,et al.  Feasibility of quantitative regional ventilation and perfusion mapping with phase‐resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients , 2018, Magnetic resonance in medicine.

[10]  A. Hell,et al.  Spinal deformity changes in children with long-term vertical expandable prosthetic titanium rib treatment. , 2017, The spine journal : official journal of the North American Spine Society.

[11]  G. Redding,et al.  Thoracic Insufficiency Syndrome. , 2016, Current problems in pediatric and adolescent health care.

[12]  L. Schad,et al.  Non-invasive quantitative pulmonary V/Q imaging using Fourier decomposition MRI at 1.5T. , 2015, Zeitschrift für Medizinische Physik.

[13]  B. Nickerson,et al.  Within-subject variability and per cent change for significance of spirometry in normal subjects and in patients with cystic fibrosis. , 2015, The American review of respiratory disease.

[14]  K. Reinker,et al.  Management of Thoracic Insufficiency Syndrome in Patients With Jeune Syndrome Using the 70 mm Radius Vertical Expandable Prosthetic Titanium Rib , 2015, Journal of pediatric orthopedics.

[15]  R. Campbell,et al.  Management of thoracic insufficiency syndrome in patients with Jarcho-Levin syndrome using VEPTRs (vertical expandable prosthetic titanium ribs). , 2014, The Journal of bone and joint surgery. American volume.

[16]  R. Betz,et al.  VEPTR to Treat Nonsyndromic Congenital Scoliosis: A Multicenter, Mid-term Follow-up Study , 2013, Journal of pediatric orthopedics.

[17]  M. Puder,et al.  Scoliosis after chest wall resection , 2013, Journal of children's orthopaedics.

[18]  R. Campbell VEPTR: past experience and the future of VEPTR principles , 2013, European Spine Journal.

[19]  A. Alanay,et al.  Vertebral Body Growth During Growing Rod Instrumentation: Growth Preservation or Stimulation? , 2012, Journal of pediatric orthopedics.

[20]  L. Lenke,et al.  Correlation of Preoperative Deformity Magnitude and Pulmonary Function Tests in Adolescent Idiopathic Scoliosis , 2011, Spine.

[21]  Shlomo Wientroub,et al.  Intra- and interobserver reliability analysis of digital radiographic measurements for pediatric orthopedic parameters using a novel PACS integrated computer software program , 2010, Journal of children's orthopaedics.

[22]  R. Betz,et al.  The Usefulness of VEPTR in the Older Child With Complex Spine and Chest Deformity , 2010, Clinical orthopaedics and related research.

[23]  Michael Deimling,et al.  Non‐contrast‐enhanced perfusion and ventilation assessment of the human lung by means of fourier decomposition in proton MRI , 2009, Magnetic resonance in medicine.

[24]  V. Deeney,et al.  Thoracic malformation with early-onset scoliosis: effect of serial VEPTR expansion thoracoplasty on lung growth and function in children. , 2009, Paediatric respiratory reviews.

[25]  D. Sanders,et al.  Reproducibility of spirometry during cystic fibrosis pulmonary exacerbations , 2008, Pediatric pulmonology.

[26]  Robert Campbell,et al.  Lung function asymmetry in children with congenital and infantile scoliosis. , 2008, The spine journal : official journal of the North American Spine Society.

[27]  D. Skaggs,et al.  Scoliosis in-brace curve correction and patient preference of CAD/CAM versus plaster molded TLSOs , 2007, Journal of children's orthopaedics.

[28]  Winnie CW Chu,et al.  Dynamic magnetic resonance imaging in assessing lung function in adolescent idiopathic scoliosis: a pilot study of comparison before and after posterior spinal fusion , 2007, Journal of orthopaedic surgery and research.

[29]  L. Lenke,et al.  Prospective Pulmonary Function Comparison Following Posterior Segmental Spinal Instrumentation and Fusion of Adolescent Idiopathic Scoliosis: Is There a Relationship Between Major Thoracic Curve Correction and Pulmonary Function Test Improvement? , 2007, Spine.

[30]  R. Campbell,et al.  The Effect of Mid-Thoracic VEPTR Opening Wedge Thoracostomy on Cervical Tilt Associated With Congenital Thoracic Scoliosis in Patients With Thoracic Insufficiency Syndrome , 2007, Spine.

[31]  C. Bach,et al.  Inter- and intraobserver reliability assessment of the Cobb angle: manual versus digital measurement tools , 2007, European Spine Journal.

[32]  R. Campbell,et al.  Thoracic insufficiency syndrome and exotic scoliosis. , 2007, The Journal of bone and joint surgery. American volume.

[33]  V. Deeney,et al.  Effects on Lung Function of Multiple Expansion Thoracoplasty in Children With Thoracic Insufficiency Syndrome: A Longitudinal Study , 2006, Spine.

[34]  E. Fogarty,et al.  Respiratory Function and Cosmesis at Maturity in Infantile-onset Scoliosis , 2003, Spine.

[35]  G. Manenti,et al.  MRI lung perfusion 2D dynamic breath-hold technique in patients with severe emphysema. , 2003, In vivo.

[36]  Robert M. Campbell,et al.  The Characteristics of Thoracic Insufficiency Syndrome Associated with Fused Ribs and Congenital Scoliosis , 2003, The Journal of bone and joint surgery. American volume.

[37]  L G Nyúl,et al.  On standardizing the MR image intensity scale , 1999, Magnetic resonance in medicine.

[38]  J. Birch,et al.  Measurement of scoliosis and kyphosis radiographs. Intraobserver and interobserver variation. , 1990, The Journal of bone and joint surgery. American volume.

[39]  L. R. Dice Measures of the Amount of Ecologic Association Between Species , 1945 .

[40]  Jayaram K. Udupa,et al.  Disease quantification on PET/CT images without explicit object delineation , 2019, Medical Image Anal..

[41]  Jayaram K. Udupa,et al.  Retrospective 4D MR image construction from free‐breathing slice Acquisitions: A novel graph‐based approach , 2017, Medical Image Anal..

[42]  Maja O’Connor LONGITUDINAL STUDY , 2013 .

[43]  R. Drongowski,et al.  Vertical expandable prosthetic titanium rib device insertion: does it improve pulmonary function? , 2011, Journal of pediatric surgery.

[44]  G. Redding,et al.  Early changes in pulmonary function after vertical expandable prosthetic titanium rib insertion in children with thoracic insufficiency syndrome. , 2009, Journal of pediatric orthopedics.

[45]  Jayaram K. Udupa,et al.  Image background inhomogeneity correction in MRI via intensity standardization , 2009, Comput. Medical Imaging Graph..

[46]  Measurement of Scoliosis and Kyphosis Radiographs , 2006 .