Pulmonary Assessment in Thoracic Insufficiency Syndrome Patients

Not Applicable

Not yet recruiting

• Conditions: Thoracic Insufficiency Syndrome
• Interventions: Device: Xenon-129

• Registration Number: NCT04977830
• Lead Sponsor: Patrick Cahill, MD

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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2021-07-14
• Study First Submitted QC: 2021-07-23
• Study First Posted: 2021-07-27
• Status Verified: 2025-04
• Last Update Submitted: 2025-04-29
• Last Update Posted: 2025-05-02
• Study Start: 2025-12
• Primary Completion: 2026-08-30
• Study Completion: 2026-08-30

Recruitment & Eligibility

• Status: NOT_YET_RECRUITING
• Sex: All
• Target Recruitment: 15

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Thoracic Insufficiency Group	Xenon-129	Thoracic insufficiency syndrome patients undergoing surgery

Primary Outcome Measures

Name	Time	Method
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 Outcome Measures

Name	Time	Method
total lung capacity	up to 2 years	Total lung capacity will be extracted from the computer-automated dynamic lung MRI software.
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.
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.
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
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

Trial Locations

Locations (1)

Children's Hospital of Philadelphia; 🇺🇸 Philadelphia, Pennsylvania, United States