The Children's Hospital of Philadelphia, Division of Pulmonary and Sleep Medicine, Philadelphia, PA, United States.
Perelman School of Medicine at The University of Pennsylvania, Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104, United States.
Paediatr Respir Rev. 2022 Dec;44:78-84. doi: 10.1016/j.prrv.2022.02.003. Epub 2022 Mar 7.
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.
胸壁发育不全综合征（TIS）于 2003 年被描述为胸廓无法支撑正常呼吸或肺生长。TIS 包括一组广泛而不同的典型退行性胸脊柱疾病。尽管 TIS 是由一组异质性疾病引起的，因此其发病率无法很好地量化，但存在一般的管理和治疗方法。不断发展的影像学技术和健康相关生活质量的测量方法增加了对肺功能的测试，以量化疾病负担，进行纵向和干预前后的评估。干预主要是通过生长保护手术来实现，目前有几种设备可供选择，以便在骨骼成熟时进行确定性脊柱融合之前，保持脊柱的纵向生长。
