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Thursday, May 14, 2009

Pectus carinatum

Pectus carinatum, also called pigeon chest, is a deformity of the chest characterized by a protrusion of the sternum and ribs.


Causes

Possible causes are, among others, Marfan syndrome, Morquio syndrome, and scoliosis (a curvature of the spine).


Epidemiology

Pectus deformities are common; about 1 in 400 people have a pectus disorder.

Pectus carinatum is more rare than pectus excavatum, another pectus disorder, occurring in only about 20% of people with pectus deformities. About three out of four patients are males.


Symptoms

Pectus carinatum, with the chest wall held in an outward position, may prevent complete expiration of air from the lungs and thus may restrict air exchange considerably. These patients often experience severe shortness of breath, wheezing and occasionally mild to moderate asthma. With exercise, carinatum patients often develop a very rapid respiratory rate.

Some children with pectus carinatum also have scoliosis (curvature of the spine). Some have mitral valve prolapse, a condition in which the heart mitral valve functions abnormally. Connective tissue disorders involving structural abnormalities of the major blood vessels and heart valves are also seen. Although rarely seen, some children have other connective tissue disorders, including arthritis, visual impairment and healing impairment.


Prognosis

Pectus deformities usually become more severe during adolescent growth years and remain the same after age 18 years throughout life. Adults often experience increasing symptoms with advancing age. Body building exercises will not alter the ribs and cartilage of the chest wall, and are generally considered not harmful.


Treatment

External bracing technique

In children up to age 18 who have mild to moderate pectus carinatum and are motivated to avoid surgery, the use of a custom-fitted chest-wall brace pushing directly on the sternum produces excellent outcomes. Willingness to wear the brace as required is essential for the success of this treatment approach. The brace works in much the same way as orthodontics (braces) works to correct the alignment of teeth. It consists of front and back compression plates that are anchored to aluminum bars. These bars are bound together by an adjustable leather strap on each side. This device is easily hidden under clothing and must be worn from 14 to 23 hours a day, (depending on stage of correction and the treatment protocol employed) until treated or full height is reached. Children are taught how to tighten the straps of the brace so to gradually increase the pressure applied to their chest. Parents learn how to check to see if adequate pressure is being applied. Pediatric surgeons monitor progress at office visits.

Research from 2006 supports real-world success with patients in the clinic. "Compressive bracing results in a significant subjective and objective improvement in PC appearance in skeletally immature patients. However, patient compliance and diligent follow up appear to be paramount for the success of this method of treatment. Further studies are required to show the durability of this method of treatment.



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