Pectus excavatum

pectus excavatumPectus excavatum (sunken or funnel chest) is a congenital deformity of the chest wall characterized by the caved-in appearance of the anterior chest wall.


Description

Pectus excavatum is a term originally from Latin and means hollowed chest.

Pectus excavatum is a sunken appearance of the chest. Pectus excavatum may be formed during gestation or infancy until the age of puberty. The deformity of the chest leads to the impairment of the respiratory and circulatory systems and causes pain in the back and the chest.

90% of the chest deformities are pectus excavatum, in 5-7% pectus carinatum is present. Pectus excavatum occurs in 1 child per 300-400 births, more likely affecting males.

 

Causes

It was estimated that in 37% of cases the genetic predisposition to the development of the pectus excavatum. These people have a first degree family member with a sunken chest. Probably increased pressure in uterus, rickets and abnormalities of the diaphragm are involved in the process of the chest’s irregular growth.

It is suggested that the heart function is impaired in individuals with pectus excavatum, although the extent of the dysfunction may differ.

In pectus excavatum the cartilage and bones of the chest are abnormal and usually- ribs on each side of the sternum are affected. The extent of the deformity varies from person to person and the asymmetry between the both sides of the chest is present.

 

Risk factors


Pectus excavatum is associated with other congenital diseases such as Noonan syndrome, Marfan syndrome, Turner syndrome, Loeys-Dietz syndrome, fetal alcohol syndrome, homocystinuria, Poland syndrome,  Ehlers-Danlos syndrome, congenital scoliosis, Poland syndrome  and osteogenesis imperfecta. Almost every of these conditions involves the abnormality of the connective tissue leading to the chest deformity.

Individuals with spinal muscular atrophy develop pectus excavatum as the result of diaphragmal breathing in those cases.
Sometimes the condition may affect those who have celiac disease.

 

Symptoms

Pectus excavatum is commonly present at birth, in 90% of cases the diagnosis is determined during the first year of life. In teenage years when the skeleton grows rapidly and leads to the worsening of the appearance of the chest. Sometimes the abnormality is noticed for the first time at this age.  Commonly only the lower third of the sternum is affected, and the upper third may appear normal. The lower ribs may protrude (“flared ribs”). Affected persons may experience the pain of the chest and the back.

Usually the condition is asymptomatic.

In severe cases the sternum compresses the lungs and the heart causing their dysfunction:

Caved-in breastbone pushes the heart to the left side and reduces the ability of the heart to pump blood. Sometimes mitral valve prolapse may be present. The deformity of the chest leads to the self-image problems.As the result of the psychological problems children develop hunched-forward posture, the decreased self-esteem makes them avoid social activities, causes anxiety, intolerance, frustration and depression.

Diagnosis

To evaluate the diagnosis X-ray examination of the chest and the CT is performed. The Haller index (dividing the transverse chest diameter by the anteroposterior diameter) is measured to decide whether the surgery is needed or not. An index above 3,2 is an indication for the surgery. CT is necessary to reveal the displacement of the internal organs and estimate the body’s condition.
ECG, echocardiography, pulmonary function tests are performed to evaluate the function of the circulatory and pulmonary functions.

 

Treatment


Nonsurgical methods weren’t effective. In mild cases the treatment isn’t needed. Physical exercise may be helpful to correct the posture and strengthen the muscles. Aerobic exercises may improve cardiopulmonary function.
In  15% of cases the funnel chest is being operated. There are several techniques that may be performed. The most popular methods is MIRPE (minimally invasive repair of pectus excavatum) or Nuss technique. Other methods include Ravitch technique, Robicsek technique.

Plastic surgery may also include the application of custom-made implants that help to repair the morphological structure of the chest wall.

Conservative treatment include:

  • The magnetic mini-mover procedure (3MP) – a technique that is performed by using two magnets to realign the sternum with the rest of the chest and ribcage;
  • Vacuum bell is a treatment by cup suction. The created vacuum lifts the sternum and lessens the extent of the deformity;
  • Corset-like orthopedic support vests;
  • Compressive orthosis;