Pectus deformities: causes and effects

Pectus deformities are the most common congenital chest-wall deformities and are better known as pigeon chest and funnel chest. The conditions affect patients psychologically and socially, and some complain of physical symptoms. The most significant factors with such deformities are age and the prominence of the body area that is affected.

The incidence of pectus deformities is one to eight per 1,000 population and they are more common in boys than in girls. A corrective operation is available to those who are unable to live with the shape of their chest, but only a minority of patients undergo this. Because of the small numbers of specialist centres, many do not receive the correct advice or sufficient information.

In pectus excavatum (funnel chest) the sternum is depressed in a concave shape and in pectus carinatum (pigeon chest) the sternum is protruded in a convex shape (Saxena and Willital, 1999). There may be asymmetry of the chest and the sternum may be rotated. The deformity is often caused by excessive growth of the costal cartilages, although the reason for this is unknown.

The condition is often noticeable at birth and becomes more apparent during rapid skeletal growth in early adolescence. Musculoskeletal abnormalities associated with pectus deformities are scoliosis and Marfan's syndrome.

Pectus deformities have been documented as affecting cardiopulmonary function (Haller and Loughlin, 2000). There are also reports of patients experiencing a decrease in stamina and endurance during strenuous exercise (67%), frequent respiratory infections (32%), chest pain (8%) and asthma (7%) (Fonkalsrud et al, 2000). However, recognition of these symptoms remains highly controversial.

While the physiological effects of pectus deformities remains debatable, the psychological difficulties patients face are evident, yet there is little published information on these. Some patients are able to accept and live happily with the shape of their chest. This is important to note as health care professionals often assume that a person with a physical disfigurement has a negative self-image (Anderson, 1982).

But many patients with pectus deformities do have a negative self-image and little self-confidence. An unwillingness to be seen without a shirt while swimming or to participate in sports or social activities are common comments. It is important to note that having a pectus deformity can affect all areas of life.

In patients aged over 11 with funnel chest, Einsiedel and Clausner (1999) found a high degree of self-observation, motivational problems, anxiety regarding social contact, depression and a poor opinion of their own appearance. As patients grow older, their psychological problems may increase. Among those aged 18-35, Einsiedel and Clausner (1999) found an excessive dependence on the parental home, alcohol and drug abuse, suicidal tendencies and generalised hypochondria. In scoliosis, a deformity with similar cosmetic effects, adolescents are at increased risk of having suicidal thoughts and concern over body development and peer interactions (Payne et al, 1997).

To understand how patients with a pectus deformity see themselves, it is essential to understand the concept of body image, self-concept and coping.

Schilder (1935) described body image as 'the picture of our body which we form in our mind, that is to say, the way in which our body appears to ourselves'. Several factors make the problems patients with pectus deformities face and the way they cope with them unique.

The age factor
Progressive growth spurts lead to a marked deformity by adolescence. Peer acceptance and conformity with the perceived norm are important to adolescents and can cause problems for those with chest deformities. Schilder (1935) said that body image is dynamic - it changes with age. During the teenage years, a patient may be acutely aware of the shape of his or her chest, but once they have a career, family and financial commitments, they may focus on their deformity less.

Body area
Society, and the media in particular, places a high priority on physical appearance and physique, especially in teenagers. Many adolescents with pectus deformities feel unable to wear low-cut or open tops as these would expose their chests, so they often disguise their deformity by wearing loose-fitting clothes.

Schilder (1935) confirmed that short-term solutions such as changes in clothing can alter body image. Acceptance of a disability or disfigurement is easier if the affected part of the body is not central to a person's self-concept: if the person places more value on intelligence, personality or relationships than physical beauty, he or she is more likely to adapt to the disability (Anderson, 1982).

The attitudes of others
Interpersonal relationships and other people's attitudes will affect a person's self-perception. People, particularly adolescents, not only search for their identity from within but also in others (Anderson, 1982). People pick up cues from others to help them define themselves.

The relationship with, and the attitudes of, those closest to the person with the disability have particular significance. Some parents dismiss the pectus deformity as being part of the person and little different from the colour of their hair or eyes, for example. Others seem to draw attention to the deformity, thereby heightening the child's anxiety. Parental attitudes can affect how well children cope with their deformity and may result in them deciding not to have surgery later in life.

Socioeconomic factors
Education, occupation, income, social class and status profiles, as well as the level of resources available to the person with the disability, are all factors that may affect their self-concept. Many young patients are demanding more information on the options of treating pectus deformities.

Surgical correction
Ravitch (1952) was the first surgeon to correct pectus deformity. Surgical correction of such deformities today involves low morbidity, little cost, minimal limitation and a high chance of symptom improvement. Fonkalsrud et al (2000) state that more than 97% of patients have a very good or excellent result.

Wheeler and Foote (2000) state that in comparison with the USA, UK surgeons see few patients with a pectus deformity. The general impression seems to be that patients in the UK are advised to 'put up' with it. Most are told, incorrectly, that it will improve with age; that the malformation produces few symptoms and is merely a cosmetic problem; and that the surgery is dangerous, minimally effective and unnecessary. In fact, less than 15% of patients with a pectus deformity have surgery (Fonkalsrud et al, 2000).

The operation performed at the Royal Brompton and Harefield Hospitals is a modified Ravitch (1952) procedure. The patient receives an epidural infusion for pain relief over 24-48 hours postoperatively and is nursed in the high-dependency unit on the first postoperative night. Most patients go home three to five days later.

Exercise is essential for a successful outcome without side-effects: it will cause breathlessness, which makes the lungs re-expand to their former size and function. It is important for patients to take regular painkillers to control the pain, enabling them to exercise. However, contact sports must be avoided for at least three to four months to ensure all bones heal.

Postoperative complications are uncommon but include pneumothorax, keloid scarring and discomfort from the sternal bar (Fonkalsrud and Bustorff-Silva, 1999).

The Nuss procedure is a minimally invasive technique that places one or two curved steel bars behind the sternum, forcing it into the correct shape. This method has recently been developed and is being continuously evaluated. The results in very young patients are encouraging. Engum et al (2000) suggest it may become the preferred method of pectus excavatum surgery for patients aged five to 15 years.

Pectus correction surgery is usually performed by specialist thoracic surgeons who have an obligation to operate on patients with diseases and disorders that are life-threatening. Because of constraints on finances in the NHS and because pectus correction surgery is often viewed as cosmetic, patients may find they have a struggle to get the operation.

Because of the need for evidence-based decisions on the allocation of resources, it is vital to study the psychosocial outcomes, patient satisfaction and quality of life before and after surgical correction of pectus deformities.

The Royal Brompton and Harefield NHS Trust will be conducting a small-scale study to address these issues. The increasing and justifiable demand from patients for more information has prompted us to write a patient education leaflet and set up a patient database. In March last year, we launched a website that gets several hundred hits a month. We also receive several personal inquires every week from people seeking further information and support.

- Royal Brompton and Harefield NHS Trust: www.pectus.org

- Johns Hopkins University School of Medicine: nabtt.org/pediatric_surgery/diseases/index.html

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