deformities and tumors of the thoracic wall - pectus excavatum

Question 1

Pectus excavatum (“funnel chest”)

Answer 1

abnormal development of the rib cage where the sternum caves in, resulting in a sunken chest wall deformity.

usually congenital and may be mild or severe

more often in men

Question 2

etiology is

Answer 2

unknown

Question 3

usually not associated with other conditions but some disorders may include it

Answer 3

Marfan syndrome: A connective tissue disorder, which causes skeletal defects typically recognized by long limbs & ‘spider-like’ fingers, chest abnormalities, curvature of the spine & certain facial features.

Rickets: A deficiency disease occurring primarily in children & results from a lack of vit D / calcium & insufficient exposure to sunlight, which disturbs normal bone growth.

Scoliosis: Abnormal curvature of the spine.

Question 4

clinical presentation

Answer 4

Usually asymptomatic.
•	Fatigue.
•	Shortness of breath.
•	Chest pain.
•	Tachycardia.

Question 5

diagnosis

Answer 5

By clinical presentation.
•	Physical (stress test).
•	Pulmonary function test.
•	Laboratory studies (blood work), such as chromosome studies / enzyme assays.
•	Metabolic studies.
•	Chest x-ray.
•	CT of chest.
•	ECG.
•	Echocardiogram.

Question 6

Treatment:

Answer 6

Dependent on development of symptoms.
Physical therapy in young patients (< 18) - usually no change after this age.
May play a role in slowing the development / possibly reverse some chest wall deformity.
When heart / lung function are compromised - surgery may be recommended.

Question 7

surgery

Answer 7

To correct & improve the patient’s breathing, posture / cardiac function.
Typically by removing a portion of the deformed cartilage & repositioning the sternum.

a variety of surgical procedures are available

Question 8

The highly modified Ravitch technique

Answer 8

completed with a vertical incision in the mid-chest area to remove anterior cartilage. Two stainless-steel struts are placed across the anterior chest to support the sternum & wired to the appropriate ribs on each side, allowing the breast bone to be elevated. The struts are not visible from the outside & removed after 2 years by surgical procedure.

Question 9

The Nuss Procedure

Answer 9

Usually for adolescent patients.
Video-assisted thoracoscopic surgery (VATS) technique.
Through two small incisions on either side of the chest, a curved steel bar (Lorenz Pectus Bar) is inserted under the sternum. Individually curved for each patient, the steel bar is used to ‘pop out’ the depression & then fixed to the ribs on either side. A small steel, grooved plate may be used at the end of the bar to help stabilize & attach the bar to the rib.
The bar is not visible from the outside & stays in place for > 2 years. When it is time, the bar is removed as an outpatient procedure.
A separate, small incision is made to insert a tube with a camera to allow the surgeon to visualize the inside of the chest & insert tools in the remaining small incisions to complete the procedure.