Pectus carinatum

Last updated
Pectus carinatum
Other namesPigeon chest, pectus cavernatum, bird chest, convex chest
Ben Fraser pectus carinatum.jpg
Severe case of pectus carinatum
Pronunciation
Specialty Orthopedics

Pectus carinatum, also called pigeon chest, is a malformation of the chest characterized by a protrusion of the sternum and ribs. It is distinct from the related malformation pectus excavatum.

Contents

Signs and symptoms

People with pectus carinatum usually develop normal hearts and lungs, but the malformation 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 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 malformations often tire sooner than their peers due to shortness of breath and fatigue. Commonly concurrent is mild to moderate asthma.

Some children with pectus carinatum also have scoliosis (i.e., 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 malformations 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, bodybuilding techniques may be useful for balancing visual impact.[ citation needed ]

A less common variant of pectus carinatum is pectus arcuatum (also called type 2 pectus excavatum, chondromanubrial malformation or Currarino–Silverman syndrome or pouter pigeon malformation), which produces a manubrial and upper sternal protrusion, [2] particularly also at the sternal angle. [3] Pectus arcuatum is often confused with a combination of pectus carinatum and pectus excavatum, but in pectus arcuatum the visual appearance is characterized by a protrusion of the costal cartilages and there is no depression of the sternum. [4]

Causes

Pectus carinatum is an overgrowth of costal cartilage causing the sternum to protrude forward. It primarily occurs among four different patient groups, and males are more frequently affected than females. Most commonly, pectus carinatum develops in 11-to-14-year-old pubertal males undergoing a growth spurt. Some parents report that their child's pectus carinatum seemingly popped up overnight. Second most common is the presence of pectus carinatum at or shortly after birth. The condition may be evident in newborns as a rounded anterior chest wall. As the child reaches age 2 or 3 years of age, the outward sternal protrusion becomes more pronounced. Pectus carinatum can also be caused by vitamin D deficiency in children (Rickets) due to deposition of unmineralized osteoid. Least common is a pectus carinatum malformation following open-heart surgery or in children with poorly controlled bronchial asthma.

Pectus carinatum is generally a solitary, non-syndromic abnormality. However, the condition may be present in association with other syndromes: Turner syndrome, Noonan syndrome, Loeys–Dietz syndrome, Marfan syndrome, Ehlers–Danlos syndrome, Morquio syndrome, trisomy 18, trisomy 21, homocystinuria, osteogenesis imperfecta, multiple lentigines syndrome (LEOPARD syndrome), Sly syndrome (mucopolysaccharidosis type VII), and scoliosis.

In about 25% of cases of pectus carinatum, the patient has a family member with the condition.

Diagnosis

The pectus carinatum can be easily diagnosed by certain tests like a CT scan (2D and 3D). It may then be found out that the rib cage is in normal structure. If there is more than average growth of sternum than pectus carinatum protrudes.[ clarification needed ] Also it is of two types, as pectus carinatum is symmetrical or unsymmetrical. On the basis of that further treatment is given to the patient.[ citation needed ]

Treatment

External bracing technique

The use of orthotic bracing, pioneered by Sydney Haje as of 1977, is finding increasing acceptance as an alternative to surgery in select cases of pectus carinatum. [5] 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 that 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 carinatum malformation (mild moderate severe) and if it is symmetric or asymmetric.[ citation needed ]

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, specifically designed to be worn under braces. A physician or orthotist or brace manufacturer's representative can show how to check to see if the brace is in correct position on the chest.[ citation needed ]

Bracing is becoming more popular over surgery for pectus carinatum, mostly because it eliminates the risks that accompany surgery. The prescribing of bracing as a treatment for pectus carinatum has 'trickled down' from both paediatric and thoracic surgeons to the family physician and pediatricians again due to its lower risks and well-documented very high success results. The pectus carinatum guideline of 2012 of the American Pediatric Surgical Association has stated: "As reconstructive therapy for the compliant pectus [carinatum] malformation, nonoperative compressive orthotic bracing is usually an appropriate first line of therapy as it does not preclude the operative option. For appropriate candidates, orthotic bracing of chest wall malformations can reasonably be expected to prevent worsening of the malformation and often results in a lasting correction of the malformation. Orthotic bracing is often successful in prepubertal children whose chest wall is compliant. Expert opinion suggests that the noncompliant chest wall malformation or significant asymmetry of the pectus carinatum malformation caused by a concomitant excavatum-type malformation may not respond to orthotic bracing." [6]

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.[ citation needed ]

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.[ citation needed ]

A modified Ravitch technique uses bioabsorbable material and postoperative bracing, and in some cases a diced rib cartilage graft technique. [7]

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 malformation is symmetrical.[ citation needed ]

Other options

After adolescence, some men and women use bodybuilding as a means to hide their malformation. Some 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. [8] Some try to hide the deformity under a layer of muscle by doing bodybuilding. But the deformity can reappear when training is stopped. [9]

Prognosis

Pectus malformations 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.[ citation needed ]

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.[ citation needed ]

Most insurance companies no longer consider chest wall malformations like pectus carinatum to be purely cosmetic conditions. While the psychologic impact of any malformation 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.[ citation needed ]

Epidemiology

Pectus malformations are rare; about 1 in 400 people have a pectus disorder. [10]

Pectus carinatum is rarer than pectus excavatum, another pectus disorder, occurring in only about 20% of people with pectus malformations. [10] About four out of five patients are males. [11]

See also

Related Research Articles

<span class="mw-page-title-main">Marfan syndrome</span> Genetic disorder involving connective tissue

Marfan syndrome (MFS) is a multi-systemic genetic disorder that affects the connective tissue. Those with the condition tend to be tall and thin, with long arms, legs, fingers, and toes. They also typically have exceptionally flexible joints and abnormally curved spines. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. The lungs, eyes, bones, and the covering of the spinal cord are also commonly affected. The severity of the symptoms is variable.

<span class="mw-page-title-main">Prune belly syndrome</span> Medical condition

Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 40,000 births. About 97% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, characterized by a triad of symptoms. The syndrome is named for the mass of wrinkled skin that is often present on the abdomen of those with the disorder.

<span class="mw-page-title-main">Scoliosis</span> Medical condition of the spine

Scoliosis is a condition in which a person's spine has a sideways curve. The curve is usually "S"- or "C"-shaped over three dimensions. In some, the degree of curve is stable, while in others, it increases over time. Mild scoliosis does not typically cause problems, but more severe cases can affect breathing and movement. Pain is usually present in adults, and can worsen with age. As the condition progresses, it may impact a person's life and hence, can also be considered a disability.

<span class="mw-page-title-main">Thorax</span> Frontal part of an animals body, between its head and abdomen

The thorax or chest is a part of the anatomy of humans, mammals, and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main divisions of the creature's body, each of which is in turn composed of multiple segments.

<span class="mw-page-title-main">Pectus excavatum</span> Congenital deformity of the chest

Pectus excavatum is a structural deformity of the anterior thoracic wall in which the sternum and rib cage are shaped abnormally. This produces a caved-in or sunken appearance of the chest. It can either be present at birth or develop after puberty.

<span class="mw-page-title-main">Pectoralis major</span> Main human chest muscle

The pectoralis major is a thick, fan-shaped or triangular convergent muscle of the human chest. It makes up the bulk of the chest muscles and lies under the breast. Beneath the pectoralis major is the pectoralis minor muscle.

<span class="mw-page-title-main">Morquio syndrome</span> Medical condition

Morquio syndrome, also known as mucopolysaccharidosis type IV (MPS IV), is a rare metabolic disorder in which the body cannot process certain types of sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides). In Morquio syndrome, the specific GAG which builds up in the body is called keratan sulfate. This birth defect, which is autosomal recessive, is a type of lysosomal storage disorder. The buildup of GAGs in different parts of the body causes symptoms in many different organ systems. In the US, the incidence rate for Morquio syndrome is estimated at between 1 in 200,000 and 1 in 300,000 live births.

<span class="mw-page-title-main">Back brace</span> Corrective medical device worn around a patients back

A back brace is a device designed to limit the motion of the spine in cases of bone fracture or in post-operative spinal fusiona, as well as a preventative measure against some progressive conditions or to correct patient posture.

<span class="mw-page-title-main">Respiratory examination</span> Inspection conducted as part of a physical

A respiratory examination, or lung examination, is performed as part of a physical examination, in response to respiratory symptoms such as shortness of breath, cough, or chest pain, and is often carried out with a cardiac examination.

<span class="mw-page-title-main">Nuss procedure</span> Procedure for treating pectus excavatum

The Nuss procedure is a minimally invasive procedure, invented in 1987 by Dr. Donald Nuss for treating pectus excavatum. He developed it at Children's Hospital of The King's Daughters, in Norfolk, Virginia. The operation typically takes approximately two hours.

<span class="mw-page-title-main">Poland syndrome</span> Medical condition

Poland syndrome is a birth defect characterized by an underdeveloped chest muscle and short webbed fingers on one side of the body. There may also be short ribs, less fat, and breast and nipple abnormalities on the same side of the body. Typically, the right side is involved. Those affected generally have normal movement and health.

<span class="mw-page-title-main">Fetal warfarin syndrome</span> Congenital disorder caused by maternal warfarin administration

Fetal warfarin syndrome is a disorder of the embryo which occurs in a child whose mother took the medication warfarin during pregnancy. Resulting abnormalities include low birth weight, slower growth, intellectual disability, deafness, small head size, and malformed bones, cartilage, and joints.

<span class="mw-page-title-main">Sternocostal joints</span>

The sternocostal joints, also known as sternochondral joints or costosternal articulations, are synovial plane joints of the costal cartilages of the true ribs with the sternum. The only exception is the first rib, which has a synchondrosis joint since the cartilage is directly united with the sternum. The sternocostal joints are important for thoracic wall mobility.

<span class="mw-page-title-main">Kyphoscoliosis</span> Medical condition

Kyphoscoliosis describes an abnormal curvature of the spine in both the coronal and sagittal planes. It is a combination of kyphosis and scoliosis. This musculoskeletal disorder often leads to other issues in patients, such as under-ventilation of lungs, pulmonary hypertension, difficulty in performing day-to-day activities, psychological issues emanating from anxiety about acceptance among peers, especially in young patients. It can also be seen in syringomyelia, Friedreich's ataxia, spina bifida, kyphoscoliotic Ehlers–Danlos syndrome (kEDS), and Duchenne muscular dystrophy due to asymmetric weakening of the paraspinal muscles.

<span class="mw-page-title-main">Loeys–Dietz syndrome</span> Medical condition

Loeys–Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder. It has features similar to Marfan syndrome and Ehlers–Danlos syndrome. The disorder is marked by aneurysms in the aorta, often in children, and the aorta may also undergo sudden dissection in the weakened layers of the wall of the aorta. Aneurysms and dissections also can occur in arteries other than the aorta. Because aneurysms in children tend to rupture early, children are at greater risk for dying if the syndrome is not identified. Surgery to repair aortic aneurysms is essential for treatment.

<span class="mw-page-title-main">Haller index</span>

The Haller index, created in 1987 by J. Alex Haller, S. S. Kramer, and S. A. Lietman, is a mathematical relationship that exists in a human chest section observed with a CT scan. It is defined as the ratio of the transverse diameter and the anteroposterior diameter.

<span class="mw-page-title-main">Sternum</span> Flat bone in the middle front part of the rib cage

The sternum or breastbone is a long flat bone located in the central part of the chest. It connects to the ribs via cartilage and forms the front of the rib cage, thus helping to protect the heart, lungs, and major blood vessels from injury. Shaped roughly like a necktie, it is one of the largest and longest flat bones of the body. Its three regions are the manubrium, the body, and the xiphoid process. The word sternum originates from Ancient Greek στέρνον (stérnon) 'chest'.

Marcelo Martinez-Ferro was born in Buenos Aires and graduated from the Buenos Aires University School of Medicine in 1983. He completed his residency in pediatric surgery at the Ricardo Gutierrez Children´s Hospital and in 1988, joined the staff of Garrahan national Children`s Hospital. In 1992 he completed a fellowship at the Fetal Treatment Center of the UCSF where he confirmed his interest in fetal treatment and video surgery.

Sydney A. Haje was a Brazilian orthopedist, known internationally for his pioneering work on chest wall deformities including the creation of a conservative treatment protocol for the pectus carinatum and pectus excavatum conditions.

The management of scoliosis is complex and is determined primarily by the type of scoliosis encountered: syndromic, congenital, neuromuscular, or idiopathic. Treatment options for idiopathic scoliosis are determined in part by the severity of the curvature and skeletal maturity, which together help predict the likelihood of progression. Non-surgical treatment should be pro-active with intervention performed early as "Best results were obtained in 10-25 degrees scoliosis which is a good indication to start therapy before more structural changes within the spine establish." Treatment options have historically been categorized under the following types:

  1. Observation
  2. Bracing
  3. Specialized physical therapy
  4. Surgery

References

  1. "pectus carinatum". Lexico UK English Dictionary. Oxford University Press. Archived from the original on 2022-08-28.
  2. Restrepo CS, Martinez S, Lemos DF, Washington L, McAdams HP, Vargas D, Lemos JA, Carrillo JA, Diethelm L (2009). "Imaging appearances of the sternum and sternoclavicular joints". Radiographics. 29 (3): 839–59. doi:10.1148/rg.293055136. PMID   19448119.
  3. Anton H. Schwabegger (15 September 2011). Congenital Thoracic Wall Deformities: Diagnosis, Therapy and Current Developments. Springer Science & Business Media. pp. 38–. ISBN   978-3-211-99138-1.
  4. Kuzmichev, Vladimir; Ershova, Ksenia; Adamyan, Ruben (16 March 2016). "Surgical correction of pectus arcuatum". Journal of Visualized Surgery. 2: 55. doi:10.21037/jovs.2016.02.28. PMC   5638405 . PMID   29078483.
  5. Desmarais TJ, Keller MS (2013). "Pectus carinatum". Current Opinion in Pediatrics (Review). 25 (3): 375–81. doi:10.1097/MOP.0b013e3283604088. PMID   23657247. S2CID   46604820.
  6. "Pectus Carinatum Guideline – Approved by the APSA Board of Governors" (PDF). American Pediatric Surgical Association. 8 August 2012. Archived from the original (PDF) on 9 October 2016. Retrieved 17 April 2016.
  7. Del Frari B, Sigl S, Schwabegger AH (2016). "Complications Related to Pectus Carinatum Correction: Lessons Learned from 15 Years' Experience. Management and Literature Review". Plastic and Reconstructive Surgery (Review). 138 (2): 317e–29e. doi:10.1097/PRS.0000000000002414. PMID   27465193. S2CID   5385408.
  8. carinatum.com, Pectus Carinatum Exercise.
  9. , How to fix Pectus Carinatum with exercises.
  10. 1 2 "Pediatric Surgery | Mattel Children's Hospital UCLA - Los Angeles, CA". Surgery.ucla.edu. Archived from the original on 2007-09-01. Retrieved 2011-08-31.
  11. "Pectus Carinatum, Cincinnati Children's Hospital Medical Center". Cincinnatichildrens.org. 2007-09-26. Retrieved 2011-08-31.