Pulmonary Assessment in Thoracic Insufficiency Syndrome Patients

New Strategies for Pulmonary Assessment in Spinal and Chest Wall Deformity

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 Overview

• Status: Withdrawn
• Conditions: Thoracic Insufficiency Syndrome
• Intervention / Treatment: Device: Xenon-129

• Study Type: Interventional
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • Pennsylvania
    • Philadelphia, Pennsylvania, United States, 19104
      • Children's Hospital of Philadelphia

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No

Description

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 Plan

How is the study designed?

Design Details

• Primary Purpose: Basic Science
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Other: Thoracic Insufficiency Group; Thoracic insufficiency syndrome patients undergoing surgery	Device: Xenon-129; Patients will undergo a hyperpolarized MRI with administered Xenon gas

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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
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 Outcome Measures

Outcome Measure	Measure Description	Time Frame
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
Forced vital capacity (FVC)	Forced vital capacity will be extracted from the computer-automated dynamic lung MRI software.	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	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.	up to 2 years
total lung capacity	Total lung capacity will be extracted from the computer-automated dynamic lung MRI software.	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	up to 2 years

Collaborators and Investigators

• Sponsor: Patrick Cahill, MD
• Collaborators: Xemed LLC
• Investigators: Principal Investigator: Patrick Cahill, MD, Children's Hopsital of Philadelphia

Study record dates

Study Major Dates

• Study Start (Estimated): January 1, 2026
• Primary Completion (Estimated): August 30, 2026
• Study Completion (Estimated): August 30, 2026

Study Registration Dates

• First Submitted: July 14, 2021
• First Submitted That Met QC Criteria: July 23, 2021
• First Posted (Actual): July 27, 2021

Study Record Updates

• Last Update Posted (Actual): March 5, 2026
• Last Update Submitted That Met QC Criteria: March 3, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Keywords: Hyperpolarized MRI; Dynamic Lung MRI
• Additional Relevant MeSH Terms: Pathologic Processes; Disease; Syndrome; Physiological Effects of Drugs; Anesthetics; Central Nervous System Depressants; Anesthetics, General; Anesthetics, Inhalation; Xenon
• Other Study ID Numbers: 20-017344

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes
• product manufactured in and exported from the U.S.: No