Thoracic Insufficiency Syndrome

Also known as: TIS.

What is thoracic insufficiency syndrome?

The thorax is the portion of the body between the neck and abdomen that includes the spine, and ribs, sternum (breastbone), the chest wall (rib cage), and lungs. TIS is a rare congenital (before birth) complex condition that involves chest wall abnormalities which prevent normal lung growth or breathing.

What causes thoracic insufficiency syndrome? 

Thoracic insufficiency syndrome commonly occurs with progressive curvature of the spine (scoliosis) but also with congenital absence of ribs, from spinal muscular atrophy, spina bifida and many other conditions.

What are the signs/symptoms of thoracic insufficiency syndrome? 

Common symptoms may include a narrow trunk and rib cage, scoliosis (curved spine), absence of ribs or rib fusion, breathing problems and other symptoms.

What are thoracic insufficiency syndrome care options? 

There may be options, however a common treatment is the surgical insertion of a device called a vertical expandable prosthetic titanium rib (VEPTR) that expands the chest and stabilizes the spine.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 5/22/2018 1:18:34 PM


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