Chest Wall Malformations

Question 1

Define scoliosis.

Answer 1

An abnormal lateral curvature of the spine.

Question 2

What are the most common causes of congenital scoliosis?

Answer 2

Inherited neuromuscular disesases, and rib/vertebral anomalies.

Question 3

What is the most common form of scoliosis?

Answer 3

Idiopathic scoliosis comprises 80-85% of all scoliosis.

Question 4

What are the three categories of idiopathic scoliosis?

Answer 4

Infantile (0-3 years), juvenile (4-9 years), and adolescent (10-17 years).

Question 5

What degree of thoracic curvature in scoliosis can result in respiratory symptoms?

Answer 5

Mild scoliosis is asymptomatic, but when the thoracic curve is >50°, you may have pulmonary function abnormalities.

Question 6

What degree of thoracic curvature in scoliosis can result in cardiopulmonary compromise?

Answer 6

Severe scoliosis causes respiratory abnormalities, including restrictive lung disease and/or distortion of the airways or large pulmonary vessels. Cardiopulmonary compromise generally occurs if the Cobb angle exceeds 90°.

Question 7

What is the typical management of scoliosis?

Answer 7

If diagnosed early enough, bracing usually corrects or limits progression of the curve.

Question 8

When is surgical intervention used in the treatment of scoliosis?

Answer 8

Surgical intervention is often required when scoliosis is congenital or caused by neuromuscular weakness.

Question 9

Define kyphosis.

Answer 9

Kyphosis is an abnormal anteroposterior convex curvature of the spine.

Question 10

Where does kyphosis most commonly occur in the spine?

Answer 10

Kyphosis typically involves the upper thoracic spine.

Question 11

List some (7) etiologies of kyphosis.

Answer 11

Congenital, trauma, infection, tumor, neuromuscular disease, Scheuermann disease, or other underlying conditions.

Question 12

What is the treatment for kyphosis?

Answer 12

Treatment can include bracing, physical therapy, and NSAIDs. For more severe cases (progressive kyphosis >80°), surgery is often indicated.

Question 13

What is Scheuermann kyphosis?

Answer 13

In Scheuermann kyphosis there is ≥5° anterior wedging of at least 3 adjacent thoracic vertebrae. The cause is unknown, but it appears that damage to the vertebral growth plate is followed by abnormal vertebral growth.

Question 14

In which patient population is Scheuermann kyphosis most likely to be diagnosed?

Answer 14

It is most common in males and typically begins during the prepubertal growth spurt.

Question 15

What findings would you expect on x-ray of a patient with Scheuermann kyphosis?

Answer 15

Anterior wedging of at least 3 adjacent thoracic vertebrae, irregularities of the vertebral endplates, and Schmorl nodes (herniation of the nucleus pulposis through the vertebral body endplate into the adjacent vertebrae).

Question 16

What is the recommended management of Scheuermann kyphosis?

Answer 16

Initial management is conservative, consisting of avoiding pain triggers, treatment with NSAIDs, and exercises to increase strength and flexibility. Curves >60° in the skeletally immature may be improved with bracing, and curves >80° that are uncontrolled by bracing sometimes require surgery.

Question 17

What is pectus excavatum?

Answer 17

Pectus excavatum is a depression of the midsternum.

Question 18

What additional conditions might be seen in patients with pectus excavatum?

Answer 18

These patients will sometimes have connective tissue disorders or other genetic conditions (e.g. Marfan, Ehlers-Danlos). It can also occur in response to some underlying respiratory conditions.

Question 19

What is the association between pectus excavatum and cardiac or respiratory compromise?

Answer 19

Pectus excavatum rarely causes respiratory or cardiac problems, though some complain of exercise intolerance.

Question 20

In what cases should patients with pectus excavatum be considered for surgical referral?

Answer 20

Consider surgical referral in those who have concurrent thoracic scoliosis, significant cardiopulmonary limitation, or electively if the patient desires to improve the aesthetic appearance of their chest.

Question 21

What is pectus carinatum?

Answer 21

Pectus carinatum is the anterior protrusion of the sternum with lateral depression of the costal cartilages.

Question 22

What is the recommended management of pectus carinatum?

Answer 22

Bracing and surgery are recommended only to improve appearance.

Question 23

What is asphyxiating thoracic dystrophy (Jeune syndrome)?

Answer 23

An autosomal recessive disorder that primarily affects the bones. Patients often have short ribs, a small rib cage, and renal disease. Some children also have short-limb dwarfism, pelvic anomalies, polydactyly, and liver disease. The short ribs limit lung growth and expansion, so restrictive lung disease is common and can require long term continuous ventilation or ventilation only with respiratory infections.