Abstract
Thoracic insufficiency syndrome (TIS) is defined as the inability of the thorax to support respiration and normal pulmonary growth. It includes a variety of musculoskeletal disorders that can impair pulmonary function to a point where treatment may become necessary. The majority of pulmonary development occurs in the initial 5 years of life and can be hampered by progressive chest wall and spinal deformities in patients with early-onset scoliosis (EOS). When untreated, the majority of patients progress to develop restrictive lung disease, with a minority of patients developing obstructive lung disease due to distortion of the bronchi. Although a variety of tests have been described, forced vital capacity (FVC) remains the parameter of choice to serially monitor pulmonary function over some time. Polysomnograms are useful to determine respiratory treatment during the perioperative period. The goal of treatment is deformity correction and preservation of growth capacity. Although various surgical options are available to treat TIS, in the presence of chest wall anomalies, vertical expandable prosthetic titanium rib (VEPTR) is a preferred treatment modality although efficacy remains questionable, as no dramatic change in deformity, pulmonary function or quality of life has been noted over some time. In developing countries, various limitations, such as a lack of universal availability of modern implants and technical support, financial constraints, and loss of follow-up, make the management of TIS a challenging task.
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Gadiya, A., Dhawale, A., Nene, A. (2023). Thoracic Insufficiency Syndrome. In: Zacharia, B., Raja, S.D.C., KV, N. (eds) Paediatric Scoliosis . Springer, Singapore. https://doi.org/10.1007/978-981-99-3017-3_27
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DOI: https://doi.org/10.1007/978-981-99-3017-3_27
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