Pectus carinatum
Encyclopedia
Pectus carinatum, 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
.
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.
Pectus carinatum is rarer than pectus excavatum
, another pectus disorder, occurring in only about 20% of people with pectus deformities. About four out of five patients are male
s.
.
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.
Apart from the possible physiologic consequences, pectus deformities can have a significant psychologic impact. Some people, especially those with milder cases, live happily with pectus carinatum. For others, though, the shape of the chest can damage their self-image and confidence, possibly disrupting social connections and causing them to feel uncomfortable throughout adolescence and adulthood. As the child grows older, especially if male, bodybuilding techniques may be useful for balancing visual impact.
Body building exercises (often attempted to cover the defect with pectoral muscles) will not alter the ribs and cartilage of the chest wall, and are generally considered not harmful.
Most insurance companies no longer consider chest wall deformities like pectus carinatum to be purely cosmetic conditions. While the psychologic impact of any deformity is real and must be addressed, the physiological concerns must take precedence. The possibility of lifelong cardiopulmonary difficulties is serious enough to warrant a visit to a thoracic surgeon.
Depending on the manufacturer and / or the patient's preference the brace may be worn on the skin or it may be worn over a body 'sock' or sleeve called a Bracemate that is specifically designed to be worn under braces. Your physician or orthotist or brace manufacturers representative will show you how to check and see if the brace is in correct position on the chest.
Bracing is becoming more popular over surgery for pectus carinatum. Mostly due to eliminating the risks that accompany surgery. The prescribing of bracing as a treatment for pectus carinatum has 'trickled down' from pediatric surgeons and thoracic surgeons down to the family pysician and pediatricians again due to its low risks and very high well documeneted successful outcomes.
Regular supervision during the bracing period is required for optimal results. Adjustments may be needed to the brace as the child grows and the pectus improves.
If bracing should fail for whatever reason then surgery would be the next step. The two most common procedures are the Ravitch technique and the Reverse Nuss procedure.
The Nuss was developed by Donald Nuss at the Children's Hospital of the King's Daughters in Norfolk, Va. The Nuss is primarily used for Pectus Excavatum, but has recently been revised for use in some cases of PC, primarily when the deformity is symmetrical.
Pectus excavatum
Pectus excavatum is the most common congenital deformity of the anterior wall of the chest, in which several ribs and the sternum grow abnormally. This produces a caved-in or sunken appearance of the chest...
.
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'. Pectus Carinatum appears to be more prevalent in males with Jewish heritage.It may occur as a solitary congenital abnormality or in association with other genetic disorders or syndromes : Marfan syndrome
Marfan syndrome
Marfan syndrome is a genetic disorder of the connective tissue. People with Marfan's tend to be unusually tall, with long limbs and long, thin fingers....
, Morquio syndrome
Morquio syndrome
Morquio's syndrome is an autosomal recessive mucopolysaccharide storage disease , usually inherited. It is a rare type of birth defect with serious consequences...
, Noonan syndrome
Noonan syndrome
Noonan Syndrome is a relatively common autosomal dominant congenital disorder considered to be a type of dwarfism, that affects both males and females equally. It used to be referred to as the male version of Turner's syndrome ; however, the genetic causes of Noonan syndrome and Turner syndrome...
, Trisomy 18, Trisomy 21, homocystinuria
Homocystinuria
Homocystinuria, also known as cystathionine beta synthase deficiency or CBS deficiency, is an inherited disorder of the metabolism of the amino acid methionine, often involving cystathionine beta synthase...
, osteogenesis imperfecta
Osteogenesis imperfecta
Osteogenesis imperfecta is a genetic bone disorder. People with OI are born with defective connective tissue, or without the ability to make it, usually because of a deficiency of Type-I collagen...
, multiple lentigines syndrome, Sly syndrome
Sly syndrome
Sly syndrome, also called Mucopolysaccharidosis Type VII or MPS, is an autosomal recessive lysosomal storage disease characterized by a deficiency of the enzyme β-glucuronidase, a lysosomal enzyme. Sly syndrome belongs to a group of disorders known as mucopolysaccharidoses, which are lysosomal...
and Scoliosis
Scoliosis
Scoliosis is a medical condition in which a person's spine is curved from side to side. Although it is a complex three-dimensional deformity, on an X-ray, viewed from the rear, the spine of an individual with scoliosis may look more like an "S" or a "C" than a straight line...
.
In about 25% of cases of pectus carinatum, the patient has a family member with the condition.
Epidemiology
Pectus deformities are common; about 1 in 400 people have a pectus disorder.Pectus carinatum is rarer than pectus excavatum
Pectus excavatum
Pectus excavatum is the most common congenital deformity of the anterior wall of the chest, in which several ribs and the sternum grow abnormally. This produces a caved-in or sunken appearance of the chest...
, another pectus disorder, occurring in only about 20% of people with pectus deformities. About four out of five patients are male
Male
Male refers to the biological sex of an organism, or part of an organism, which produces small mobile gametes, called spermatozoa. Each spermatozoon can fuse with a larger female gamete or ovum, in the process of fertilization...
s.
Symptoms
People with pectus carinatum usually develop normal hearts and lungs, but the deformity may prevent these from functioning optimally. In moderate to severe cases of pectus carinatum, the chest wall is rigidly held in an outward position. Thus, respirations are inefficient and the individual needs to use the diaphragm and accessory muscles for respiration, rather than normal chest muscles, during strenuous exercise. This negatively affects gas exchange and causes a decrease in stamina. Children with pectus deformities often tire sooner than their peers, due to shortness of breath and fatigue. Commonly concurrent is mild to moderate asthmaAsthma
Asthma is the common chronic inflammatory disease of the airways characterized by variable and recurring symptoms, reversible airflow obstruction, and bronchospasm. Symptoms include wheezing, coughing, chest tightness, and shortness of breath...
.
Some children with pectus carinatum also have scoliosis
Scoliosis
Scoliosis is a medical condition in which a person's spine is curved from side to side. Although it is a complex three-dimensional deformity, on an X-ray, viewed from the rear, the spine of an individual with scoliosis may look more like an "S" or a "C" than a straight line...
(curvature of the spine). Some have mitral valve prolapse
Mitral valve prolapse
Mitral valve prolapse is a valvular heart disease characterized by the displacement of an abnormally thickened mitral valve leaflet into the left atrium during systole. There are various types of MVP, broadly classified as classic and nonclassic. In its nonclassic form, MVP carries a low risk of...
, 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.
Apart from the possible physiologic consequences, pectus deformities can have a significant psychologic impact. Some people, especially those with milder cases, live happily with pectus carinatum. For others, though, the shape of the chest can damage their self-image and confidence, possibly disrupting social connections and causing them to feel uncomfortable throughout adolescence and adulthood. As the child grows older, especially if male, bodybuilding techniques may be useful for balancing visual impact.
Prognosis
Pectus deformities usually become more severe during adolescent growth years and may worsen throughout adult life. The secondary effects, such as scoliosis and cardiovascular and pulmonary conditions, may worsen with advancing age.Body building exercises (often attempted to cover the defect with pectoral muscles) will not alter the ribs and cartilage of the chest wall, and are generally considered not harmful.
Most insurance companies no longer consider chest wall deformities like pectus carinatum to be purely cosmetic conditions. While the psychologic impact of any deformity is real and must be addressed, the physiological concerns must take precedence. The possibility of lifelong cardiopulmonary difficulties is serious enough to warrant a visit to a thoracic surgeon.
External bracing technique
In children, teenagers, and young adults who have pectus carinatum and are motivated to avoid surgery, the use of a customized chest-wall brace that applies direct pressure on the protruding area of the chest 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 for your teeth)that work to correct the alignment of teeth. The brace consists of front and back compression plates that are anchored to aluminum bars. These bars are bound together by a tightening mechanism which varies from brace to brace. This device is easily hidden under clothing and must be worn from 14 to 24 hours a day. The wearing time varies with each brace manufacturer and the managing physicians protocol which could be based on the severity of the cariantum deformity (mild moderate severe) and if it is symetric or asymetric.Depending on the manufacturer and / or the patient's preference the brace may be worn on the skin or it may be worn over a body 'sock' or sleeve called a Bracemate that is specifically designed to be worn under braces. Your physician or orthotist or brace manufacturers representative will show you how to check and see if the brace is in correct position on the chest.
Bracing is becoming more popular over surgery for pectus carinatum. Mostly due to eliminating the risks that accompany surgery. The prescribing of bracing as a treatment for pectus carinatum has 'trickled down' from pediatric surgeons and thoracic surgeons down to the family pysician and pediatricians again due to its low risks and very high well documeneted successful outcomes.
Regular supervision during the bracing period is required for optimal results. Adjustments may be needed to the brace as the child grows and the pectus improves.
Surgical
For patients with severe pectus carinatum, surgery may be necessary. However bracing could and may still be the first line of treatment. Some severe cases treated with bracing may result in just enough improvement that patient is happy with the outcome and may not want surgery afterwards.If bracing should fail for whatever reason then surgery would be the next step. The two most common procedures are the Ravitch technique and the Reverse Nuss procedure.
The Nuss was developed by Donald Nuss at the Children's Hospital of the King's Daughters in Norfolk, Va. The Nuss is primarily used for Pectus Excavatum, but has recently been revised for use in some cases of PC, primarily when the deformity is symmetrical.
Other options
After adolescence, some males use bodybuilding as a means to hide their deformity, where women find that their breasts, if large enough, serve the same purpose. Some plastic surgeons perform breast augmentation to disguise mild to moderate cases in women. Bodybuilding is suggested for people with symmetrical pectus carinatum.External links
- http://www.pectusdeformity.com/
- http://www.pectus.org (UK)
- http://www.carinatum.com (US)