Pulmonary Assessment in Thoracic Insufficiency Syndrome Patients

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.

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)

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.

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.

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

Apparent diffusion coefficient (ADC) maps are extracted from the Xenon-129 MRI from a single breath-hold pulse sequence.

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

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