Pulmonary Assessment in Thoracic Insufficiency Syndrome Patients

Thoracic Insufficiency Syndrome Clinical Trial

Official title:

New Strategies for Pulmonary Assessment in Spinal and Chest Wall Deformity

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04977830
• Other study ID #: 20-017344
• Status: Recruiting
• Phase: N/A
• Start date: December 2024
• Est. completion date: August 30, 2025

Study information

• Verified date: December 2023
• Source: Children's Hospital of Philadelphia
• Contact: Hamidou Keita
• Phone: 267-426-5433
• Email: keitah[@]chop.edu
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 5
• Est. completion date: August 30, 2025
• Est. primary completion date: August 30, 2025
• Accepts healthy volunteers: No
• Gender: All
• Age group: 6 Years to 18 Years

Eligibility

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

Related Conditions & MeSH terms

• Syndrome
• Thoracic Insufficiency Syndrome

Intervention

Device:
• Xenon-129
Patients will undergo a hyperpolarized MRI with administered Xenon gas

Locations

Country	Name	City	State
United States	Children's Hospital of Philadelphia	Philadelphia	Pennsylvania

Sponsors (1)

Lead Sponsor	Collaborator
Children's Hospital of Philadelphia

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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
Primary	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	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
Secondary	Forced vital capacity (FVC)	Forced vital capacity will be extracted from the computer-automated dynamic lung MRI software.	up to 2 years
Secondary	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
Secondary	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
Secondary	total lung capacity	Total lung capacity will be extracted from the computer-automated dynamic lung MRI software.	up to 2 years
Secondary	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