Pectus carinatum
| Pectus carinatum | |
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| ICD-10 | Q67.7 |
| ICD-9 | 754.82 |
| DiseasesDB | 29402 |
| MedlinePlus | 003321 |
| eMedicine | ped/1803 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
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Pectus carinatum, (L carīnātus, equiv. to carīn(a) keel), also called pigeon chest, is a deformity of the chest characterized by a protrusion of the sternum and ribs. It is the opposite of pectus excavatum.
Causes
Pectus carinatum is an overgrowth of cartilage causing the sternum to protrude forward. It occurs in 3 different ways.
The least common way is post surgically after open heart surgery. Sometimes the sternum does not heal flat and there is a protrusion of the sternum.
The second most common is from birth. It is evident in newborns as a rounded chest and as they reach 2 or 3 years old the sternum begins to grow outwardly even more.
The most common occurrence for pectus carinatum seems to be in the 11-14 year old pubertal male undergoing a growth spurt. Some parents report that their child's pectus seemingly popped up 'overnight'.
It may occur as a solitary congenital abnormality or in association with other genetic disorders or syndromes : Marfan syndrome, Morquio syndrome, Noonan syndrome, Trisomy 18, Trisomy 21, homocystinuria, osteogenesis imperfecta, multiple lentigines syndrome, Sly syndrome and Scoliosis.
In about 25% of cases of pectus carinatum, the patient has a family member with the condition.
Incidence/prevalence
Pectus deformities are common (about 1 in 400 people have a pectus disorder).[1]
Pectus carinatum is more rare than pectus excavatum, another pectus disorder, occurring in only about 20% of people with pectus deformities.[1]
It occurs much more commonly in males - about 75% of patients.[2]
Pectus carinatum, with the chest wall held in an outward position, prevents complete expiration of air from the lungs and thus restricts 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, abnormal growth, visual impairment and healing impairment.
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, but are not harmful.
Treatment
External bracing technique
In children up to age 16 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 over a T-shirt for 14 to 16 hours a day for a minimum of two years or until 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 every six months.
Surgery
In children who are not candidates for bracing, surgery may be necessary to restore normal chest contour. Open surgery in boys is performed through a horizontal incision on the anterior chest wall, usually just below the nipple area. In girls, this incision is placed to coincide with the lower breast margins when possible. The lower four to five cartilages that are abnormal are removed, leaving the perichondrium (the lining that envelops the outer portion of rib cartilage). This allows the cartilage to regrow in its new position. The sternum is surgically fractured and placed in the correct position. The incision is usually closed with internal sutures that minimize scarring.
The length of hospital stay following surgery is typically three to four days, but children often experience some discomfort for several weeks. For several days following surgery pain can be well controlled by epidural analgesia catheters or intravenous narcotics. Milder pain is managed with oral medication. Although minor complications sometimes occur, these are quite easy to treat. Cosmetic and physical outcomes in children who have undergone surgery in mid-childhood or early adolescence are generally excellent.
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