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.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
BASIC_SCIENCE
Masking
NONE
Enrollment
15
Patients will undergo a hyperpolarized MRI with administered Xenon gas
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, United States
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)
Time frame: 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.
Time frame: up to 2 years
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.
Time frame: up to 2 years
Forced vital capacity (FVC)
Forced vital capacity will be extracted from the computer-automated dynamic lung MRI software.
Time frame: 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
Time frame: 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.
Time frame: up to 2 years
total lung capacity
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Total lung capacity will be extracted from the computer-automated dynamic lung MRI software.
Time frame: 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
Time frame: up to 2 years