Thoracic insufficiency syndrome: Approaches to assessment and management

Autor: Katharine Tsukahara, Oscar Henry Mayer
Rok vydání: 2022
Předmět:
Zdroj: Paediatric Respiratory Reviews. 44:78-84
ISSN: 1526-0542
DOI: 10.1016/j.prrv.2022.02.003
Popis: Thoracic insufficiency syndrome (TIS) was described in 2003 as the inability of the thorax to support normal respiration or lung growth. TIS includes a broad and disparate group of typically degenerative thoracospinal conditions. Although TIS arises due to a heterogeneous group of disorders and thus its incidence is not well quantified, general approaches to management and treatment exist. Evolving imaging techniques and measurements of health-related quality of life augment tests of pulmonary function to quantify disease burden, longitudinally and pre- and post-intervention. Intervention is primarily via growth-sparing surgery, for which several device options exist, to preserve vertical growth prior to a definitive spinal fusion at skeletal maturity.
Databáze: OpenAIRE