A 17-year-old patient with a deep pectus excavatum, before surgery (top) and two weeks after the operation (bottom). A minimally invasive (Nuss) repair was performed and two bars inserted. These were removed after three years and very good cosmetic results were achieved (Photographs: Author images)
Anterior chest wall abnormalities occur in 0.3% of neonates. Most of these deformities are either pectus excavatum or pectus carinatum.
Pectus excavatum is the most common type, with a male-to-female ratio of 3:1. It is characterised by a depression of the sternum and anterior ribs, consequently decreasing the distance between the anterior chest wall and thoracic vertebrae.
The severity of the inward caving varies from a mild dip to an extensive funnel with heart and lungs being compressed and displaced. It usually becomes more pronounced in early adolescence as a result of the growth spurt.1
Pectus carinatum is less frequently seen. The sternum protrudes anteriorly, resulting in synonyms such as 'chicken' or 'pigeon breast' deformity, sternal kyphosis and pyramidal chest.
In 37% of cases there is a family history, which makes an underlying genetic disorder likely. The condition often occurs in combination with Marfan syndrome and Ehlers-Danlos syndrome, congenital kyphoscoliosis or cardiac anomalies. Despite this, no genetic marker has been identified.2
Signs and symptoms
The main cardiopulmonary complaints related to severe excavatum deformities are palpitation, chest tightness and pain.
Arrhythmias, conduction disorders and murmur as a result of mitral valve prolapse may also be revealed.
This and limited exercise tolerance appear to be caused by compression of the heart. Spirometry is usually physiological.
Psychological problems usually associated with the moderate and severe forms of chest deformity are commonly acknowledged, mainly in adolescents. Increasing awareness during growth may affect confidence and self-esteem.
A typical feature of excavatum patients is their posture, with slumped forward shoulders, slouching and lumbar spine lordosis. This manoeuvre may be a subconscious attempt to hide the deformity.
Girls with a severe excavatum may in addition be affected by the medial displacement of the breasts.
The carinatum deformity carries a similar risk of social withdrawal and poor self-esteem, because it is difficult to conceal the protrusion with clothing or posture.
Pain from local trauma to the protruding chest wall and inability to sleep prone can add to the patient's psychological difficulties.3
Open surgical repair (the modified Ravitch procedure) is the accepted standard of treatment for severe excavatum and carinatum deformity.
This method is based on the original Ravitch procedure, initially reported in 1949. It is fairly extensive and invasive in nature and leaves the patient with a scar on the anterior chest wall; complete postoperative recovery may take four to six weeks.
Minimally invasive repair of pectus excavatum (MIRPE or Nuss procedure) was introduced in 1998.4 One or more metal bars are inserted behind the sternum through small lateral skin incisions, using video-thoracoscopic guidance. The bars reposition the inward-pointing sternum into a physiological position and support it until the chest wall undergoes remodelling over a suggested three-year period (a mechanism similar to dental bracing).
The length of the operation and the extent of surgical trauma are significantly less compared with open repair and patients seem less restricted by postoperative pain. However, patients are meant to follow a physiotherapy protocol with a gradual increase in physical exercise, leading to a timeframe for complete postoperative recovery of six to eight weeks. Moreover, the implants have to be removed at a later stage.
The popularity of this technique has grown, especially among adolescents. NICE also considers the procedure safe.5 Minimally invasive techniques for repair of carinatum have recently been introduced.
In terms of non-surgical repair, external compression braces or vacuum bells and targeted physiotherapy have been suggested, but evidence for their effectiveness is lacking. External compression bracing might represent an alternative to open repair in adolescents or children with carinatum deformity.6 Long-term compliance is required from patients throughout these treatments.
Referral and therapy
Early referral of adolescent/adult patients with anterior chest wall deformities to a thoracic surgeon, who will advise on management, is recommended. In most patients, the primary indication for correction is cosmetic, so the referring physician should provide all of the information available regarding the psychological aspects of the deformity.
No imaging is required before referral. An in-depth individual surgical assessment and evaluation will follow the referral. The aim is to decide on the best type of management and its timing.7
Once indication for surgery has been confirmed, a preoperative chest CT/MRI scan will guide surgical technique and provide better understanding of the aspects of surgery to patients and relatives.
Where Marfan syndrome is suspected, an echocardiogram is performed.
In agreement with most non-paediatric thoracic surgeons with an interest in anterior chest wall deformities, we recommend surgical repair for the excavatum deformity for patients aged 12-18 years, even more so if the deformity is suitable for minimally invasive repair. Not only is the chest wall still malleable at this age, but these patients also tend to have a better understanding of the operation compared with younger individuals with a view to the benefits, risks and potential complications.
Adults over the age of 30 are being considered for MIRPE, but due to the rigid chest wall in this age group, most surgeons would recommend open repair, as they would for younger patients with fairly asymmetrical defects.
For surgical repair of carinatum, the recommended age is 16-18 years, but adulthood does not represent a contraindication. External compression bracing is also available, especially for mild and moderate carinatum deformities.
- Mr Egri and Mr Froeschle are consultants at the Royal Devon and Exeter NHS Foundation Trust.
1. Shamberger RC. Curr Probl Surg 1996; 33: 469-542.
2. Kotzot D, Schwabegger AH. J Pediatr Surg 2009; 44(10): 2004-11.
3. Kelly RE, Cash TF, Shamberger RC et al. Pediatrics 2008; 122(6): 1218-22.
4. Nuss D, Kelly RE, Croitoru DP et al. J Pediatr Surg 1998; 33(4): 545-52.
5. NICE. Placement of pectus bar for pectus excavatum (also known as MIRPE or the Nuss procedure). IPG310; August 2009.
6. Lee SY, Lee SJ, Jeon CW et al. Eur J Cardiothorac Surg 2008; 34(1): 146-9.
7. Robicsek F, Hebra A. Semin Thorac Cardiovasc Surg 2009; 21(1): 85-8.