Thoracic Insufficiency Syndrome: An Overview

Document Type : Review article

Authors

1 Mayaguez Medical Center, Mayaguez, Hospital de la Concepcion, San German, Puerto Rico

2 School of Medicine, Ponce Health Sciences University, Ponce, Puerto Rico

3 Ponce Research Institute, Ponce, Puerto Rico

4 Department of Physiology, Neuroscience Division, Ponce Health Sciences University, Ponce Research Institute, Ponce, Puerto Rico

10.30483/rijm.2015.118394

Abstract

Context: Thoracic insufficiency syndrome is a condition in which the thorax is unable to support either normal respiration or lung growth. This condition is a progressive one, and it can further develop into restrictive lung disease. Evidence Acquisition: Currently, there is no simple laboratory test that can be used to identify thoracic insufficiency syndrome; it can, however, be identified based on a given patient’s history of respiratory symptoms, the findings of a physical examination, radiographs, computed tomography scans, pulmonary function studies and other laboratory tests of respiratory function. As is the case with other medical conditions, this syndrome should be monitored with clinical and radiological evaluations. However, if a patient undergoes obvious deterioration (as determined by clinical, radiological, and laboratory assessments), surgical intervention is advised. The most commonly proposed surgical intervention involves the use of a device known as the vertical expandable prosthetic titanium rib (VEPTR). Results: A direct enlargement of the thorax that will thus provide sufficient space for lung growth; the correction of both the threedimensional thoracic deformity and the progressive scoliosis. Recent findings have revealed that VEPTR instrumentation can result in a modest correction of the Cobb angle, with a decrease in the percentage of the predicted forced vital capacity value and accompanying chest-wall stiffness and decreased respiratory system compliance. Conclusions: Getting a complete knowledge of the condition and evaluating different treatment modalities to treat Thoracic Insufficiency Syndrome.

Keywords


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