Thoracic Insufficiency Syndrome

Curr Probl Pediatr Adolesc Health Care. 2016 Mar;46(3):72-97. doi: 10.1016/j.cppeds.2015.11.001. Epub 2015 Dec 30.


Thoracic insufficiency syndrome (TIS) is a broad grouping of disorders that have a substantial impact on the chest wall, spine, and in many situations, both. While the conditions are varied, they share a potentially substantial impact on respiratory capacity and development over time and a presentation and need for intervention that is often in early childhood. Addressing these conditions has required a new paradigm that involves both deformity correction and a preservation of growth capacity. While there are now a number of options to treat severe spinal deformity early in life, when the deformity causes or is caused by a chest wall deformity, the Vertical Expandable Prosthetic Titanium Rib(VEPTR) is able to support surgical correction of both. The skeletal correction is often quite dramatic, but the functional measurements of quality of life and pulmonary function often do not show as dramatic and improvement, but consistently show a stabilization indicating control of the progressive thoracospinal disorder that produced TIS.

Publication types

  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Comorbidity
  • Ellis-Van Creveld Syndrome / diagnosis
  • Ellis-Van Creveld Syndrome / therapy
  • Humans
  • Quality of Life
  • Respiratory Tract Diseases / physiopathology
  • Respiratory Tract Diseases / therapy
  • Spinal Diseases* / congenital
  • Spinal Diseases* / diagnosis
  • Spinal Diseases* / physiopathology
  • Spinal Diseases* / therapy
  • Thoracic Diseases* / congenital
  • Thoracic Diseases* / diagnosis
  • Thoracic Diseases* / physiopathology
  • Thoracic Diseases* / therapy

Supplementary concepts

  • Jeune syndrome