[Thoracic insufficiency syndrome]

The compendium of disorders that affect the normal growth or function of the thorax will cause Thoracic Insufficiency Syndrome (TIS). TIS is defined as the inability of the chest to sustain normal breathing and/or lung growth. The etiology of the syndrome may be secondary to spinal deformities, global deformities of the chest, neuromuscular dysfunction or the combination of any these. Its manifestation is based on a history that highlights respiratory symptoms, a physical examination that demonstrates chest deformity, abnormal radiographic findings and/or computed tomography of the chest, accompanied by alterations in lung function or other studies of respiratory function. This syndrome must be treated with haste since it progressively worsens with the aggravation of the underlying condition(s) which is unfavorable to the irreversible physiological changes of the lung that occur during development, and are directly related to the respiratory insufficiencies. The vertical expandable prosthetic titanium rib (VEPTR) was developed as a treatment procedure that aims to restore the volume and function of the thorax with the purpose of enabling thoracic growth during the development of the child or adolescent. The treatment targets the components of the rib cage as a unit, in order to prevent or treat respiratory insufficiencies. Its indications include children with early development scoliosis who are prone to develop SIT. The proposed procedure entails a high incidence of complications and conflicting results that limit its efficacy as a treatment, which is why it is a subject of great controversy in the medical literature.