Pectus deformities: causes and effects
VOL: 97, ISSUE: 28, PAGE NO: 36
Caroline Emery, RGN, DipHealth Studies, is preadmission clinic nurse, Royal Brompton and Harefield NHS TrustPectus 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.
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