Pulmonary Assessment in Thoracic Insufficiency Syndrome Patients

Study Overview
=================
Brief Summary
-----------------
Thoracic insufficiency syndrome (TIS) is a complex condition that involves chest wall deformities that can affect normal breathing and lung growth. In most cases, children with TIS are also born with spine disorders such as scoliosis. The inability of the thorax to support normal respiration or lung growth can cause respiratory distress and even mortality. Investigators aim to validate MRI imaging sequences to use as an assessment tool for pulmonary function.

Official Title
-----------------
New Strategies for Pulmonary Assessment in Spinal and Chest Wall Deformity

Conditions
-----------------
Thoracic Insufficiency Syndrome

Intervention / Treatment
-----------------
* Device: Xenon-129


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Males or females 6 to 18 years old diagnosed with thoracic insufficiency syndrome who are undergoing surgery Exclusion Criteria: patients not diagnosed with thoracic insufficiency syndrome

Ages Eligible for Study
-----------------
Minimum Age: 6 Years
Maximum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Basic Science
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Thoracic Insufficiency Group<br>Thoracic insufficiency syndrome patients undergoing surgery | Device: Xenon-129<br>* Patients will undergo a hyperpolarized MRI with administered Xenon gas<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Analyze volume of lungs at end-inspiration and end-expiration | Investigators will analyze lung volume at end-inspiration and end-expiration will be assessed by performing a thoracic dynamic magnetic resonance imaging scan; measured in total volume units (mL) | up to 2 years | 
| Xenon-129 MRI Ventilation Maps | Xenon-129 MRI will be completed to reveal unventilated regions of the lungs where the gas cannot reach after being inhaled due to restrictions of the airways. | up to 2 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| oxygen partial pressure (PAO2) maps | Oxygen partial pressure (PAO2) maps will be extracted from 129Xe MRI maps in a single breath-hold. Regions of the lungs that show abnormal PAO2 values are susceptible of improper ventilation or gas exchange. | up to 2 years | 
| Forced vital capacity (FVC) | Forced vital capacity will be extracted from the computer-automated dynamic lung MRI software. | up to 2 years | 
| Forced expiratory volume (FEV1) | Forced expiratory volume data will be extracted from the computer-automated dynamic lung MRI software to assess the volume of air forced from the lungs | up to 2 years | 
| Xenon-129 MRI apparent diffusion coefficient (ADC) maps | Apparent diffusion coefficient (ADC) maps are extracted from the Xenon-129 MRI from a single breath-hold pulse sequence. | up to 2 years | 
| total lung capacity | Total lung capacity will be extracted from the computer-automated dynamic lung MRI software. | up to 2 years | 
| functional residual capacity (FRC) | functional residual capacity data will be extracted from the computer-automated dynamic lung MRI software to measure the volume in the lungs at the end of passive expiration | up to 2 years | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Hyperpolarized MRI, Dynamic Lung MRI