Thoracic Insufficiency Syndrome
Conditions
Keywords
Hyperpolarized MRI, Dynamic Lung MRI
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.
Interventions
DEVICEXenon-129
Patients will undergo a hyperpolarized MRI with administered Xenon gas
Sponsors
Patrick Cahill, MD
Xemed LLC
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Analyze volume of lungs at end-inspiration and end-expiration | up to 2 years | 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 Ventilation Maps | up to 2 years | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| oxygen partial pressure (PAO2) maps | up to 2 years | 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 vital capacity (FVC) | up to 2 years | Forced vital capacity will be extracted from the computer-automated dynamic lung MRI software. |
| Forced expiratory volume (FEV1) | up to 2 years | 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 |
| Xenon-129 MRI apparent diffusion coefficient (ADC) maps | up to 2 years | Apparent diffusion coefficient (ADC) maps are extracted from the Xenon-129 MRI from a single breath-hold pulse sequence. |
| total lung capacity | up to 2 years | Total lung capacity will be extracted from the computer-automated dynamic lung MRI software. |
| functional residual capacity (FRC) | up to 2 years | 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 |
Countries
United States
Contacts
PRINCIPAL_INVESTIGATORPatrick Cahill, MD
Children's Hopsital of Philadelphia
Outcome results
None listed