Surgery remains an important option to consider when dealing with pectus deformities but should always be considered carefully having discussed the pectus problem with a surgeon experienced in performing all types of pectus surgery and ideally is familiar with other forms of treatment.
Surgery may be offered for both pectus excavatum and carinatum, though increasingly non-surgical external compression bracing is becoming the first line treatment for pectus carinatum in younger patients. Prosthetic implants are a type of minor surgery that may be offered in patients with pectus excavatum who are symptom free or wish to avoid a more invasive corrective surgical option. The Vacuum bell, a non-surgical option to ‘suck’ the breast bone or sternum forward is a device worn over time and may improve the pectus excavatum particularly in young patients but it’s long term results remain unclear. Occasionally, patients may be candidates for ‘hybrid’ procedures, where several options are offered to get the desired result.
The choice of which operation depends on type and severity of pectus deformity, and the operation that is likely to offer the best functional and cosmetic result as well as considering the risks involved.
The decision to have surgical correction is largely based on the severity and type of pectus deformity, patient choice including the psychosocial impact and physical or functional issues. In patients with symptoms, further investigations should be considered to identify patients with pectus associated functional impairment including musculoskeletal issues. In addition, relative contraindications to surgical correction include complex congenital heart and lung problems and neurodevelopmental disorders.
In the UK across the country about 300 pectus operations are performed each year. Though the trend is toward NUSS procedures, still more open operations are being performed, mainly for pectus excavatum, and this is likely to reflect local surgical experience.
Patients will often seek an expert opinion to understand more about the underlying pectus deformity and its effect on the body including symptoms that they may have, as well as how it appears. Unfortunately, they may have received little or no information, advice or appreciation of what a pectus deformity is previously. In young patients, it is important to elucidate the patient’s wishes as opposed to those of their parents. It is also essential to consider whether patients require psychological or psychiatric support prior to consideration of correction where there are worrying signs of anxiety, depression or body dysmorphia (a condition where a person spends a lot of time worrying about flaws in their appearance).
Assessment before surgery
All patients need to be carefully assessed for symptoms and severity of the pectus deformity. Their psychological concerns should be addressed. If symptoms are significant they should be investigated appropriately. Detailed radiology may help the surgeon assess further particularly if severe, asymmetric or a more complex deformity.
Tests (see table below) that may be required before corrective surgery for pectus excavatum. In general, radiology investigations may be all that is required prior to corrective surgery for pectus carinatum unless patient has specific symptoms or concerns.
|Indication||Investigation before corrective surgery|
|All||Chest wall measurements, Medical photography and/or 3D body scan|
|Chest CT or MRI to assess severity and associated features such as sternal rotation as well as calculation of HI and CI|
|In Presence of Symptoms:|
|Breathlessness||Lung function tests. Consider CPET|
|Palpitations, syncope or pre-syncope||ECG, Transthoracic echocardiogram. Consider Stress Echocardiogram|
|Significant Psychological features||Psychological assessment|
|Features of Marfan syndrome||Chest CT, Transthoracic echocardiogram, genetic referral for Fibrillin-1 mutation and ophthalmology review|
|Those undergoing Bar insertion including Nuss procedure||Metal allergy testing (titanium bars can be considered if there is a metal allergy)|
Surgery for Pectus Excavatum
Surgical correction of the underlying chest wall deformity can be performed through internally bracing the sternum forward, called the Nuss procedure or Minimally Invasive Repair of Pectus Excavatum (MIRPE). Alternatively, the sternum can be brought forward through a ‘break and re-set’ of the chest wall, known as a Modified Ravitch procedure. Both remain important options in treating pectus excavatum particularly in the context of physical or functional problems.
Whilst many patients may be offered surgery on the basis of a clinical assessment of severity of the deformity, the severity can also be quantified by calculating the Haller index (HI). This is a comparison of the width of the thoracic cavity to the depth between the sternum and spine on computer tomography (CT). Alternatives to a CT now include a rapid MRI (magnetic resonance imaging) protocol and 3D topographic body scanning which minimise the radiation exposure in this young patient population.
Typically, a Haller index of >3.25 is considered an indication for surgery, however, this may be helped by a correction index (CI) which accounts for abnormal chest shapes. The CI unlike the HI which relies on the width of the chest wall, appears more accurate in non-standard chest shapes such as an asymmetric chest. A CI >28% correlates with a Haller Index of >3.25.
Compression of structures in the chest secondary to the deformity can cause a decrease in internal chest volume and can lead to heart and lung impingement. It is suggested that symptoms may worsen with age, but the significance of the functional or physical effects of pectus excavatum is controversial and remains an important topic of debate.
Lung Function tests
Lung or pulmonary function tests (PFTs) are regularly performed to investigate exertional breathlessness, ‘wheeziness’ or associated respiratory problems in patients with pectus deformity. However, they are often difficult to interpret, and can appear normal or ‘near normal’. With very severe pectus excavatum, the lungs may be restricted by its compressive effect. In addition, recent research has examined how pectus affects chest wall movement and whether it affects breathing and importantly the sensation of breathlessness particularly during exercise.
The effect of sternal displacement pushing backwards particularly in severe pectus excavatum is obvious physically, on X-ray and on the echocardiogram (heart ultrasound scan). Patients often complain of a ‘constrictive’ feeling and palpations. In severe cases, it causes cardiac compression, and especially affects the right heart. However, like lung function, data from studies remains contradictory and with no consensus on whether the heart is significantly affected despite patient symptoms.
Choice of operation
The two most common types of corrective surgical repair of pectus excavatum are the MIRPE (Minimally Invasive Repair of Pectus Excavatum) or Nuss procedure and the ‘open’ Modified Ravitch operation. The choice of procedure depends on factors including age of patient, severity of deformity, associated significant asymmetry and sternal rotation, risk of complications and the experience of the surgical team.
Surgery for Pectus Carinatum
For surgical correction of pectus carinatum the options include a ‘keyhole’ approach or an ’open’ approach. Experienced Pectus surgeons recognise they can be more challenging in terms of achieving a good cosmetic result. In certain selected cases, a ‘hybrid’ option combining surgery and external compressive bracing may offer an less invasive option.
In addition, unlike Pectus excavatum there is much less evidence of pectus carinatum causing physical symptoms leading to a functional or physiological problem though the psychological impact should not be underestimated.
Unlike pectus excavatum, the severity of the pectus carinatum is often a clinical one and is based on the surgeon’s experience. There are few established radiological assessment tools, though the Pectus Clinic has developed one such tool using topographical measurements. Called, the ’Pectus Index’, a reverse Haller Index, it allows an objective measurement to assess severity of the pigeon chest.
There is no compression of structures in the chest caused by pectus carinatum. Though symptoms, particularly pain and breathlessness on exercise may be noted, the cause of these symptoms is not clear. Like pectus excavatum, such symptoms may be related to how the chest wall movements are affected particularly when breathing heavily. In general, pectus carinatum is not associated with a function problem, though musculoskeletal issues such as ‘stooped or rounded’ shoulders are often seen and can be severe.
Choice of operation
The types of corrective surgical repair of pectus carinatum are the ‘open’ Modified Ravitch operation and less commonly a ‘keyhole’ or Minimally Invasive surgery (MIS) operation, such as the Abrahamson’s technique. In addition, several alternative ’keyhole’ procedures are described including MIS with limited cutting of the cartilage combined with a external compressive brace (a hybrid operation). The choice of procedure depends on factors including age of patient, severity of deformity, associated significant asymmetry and sternal rotation, flexibility of chest wall, risk of complications and the experience of the surgical team.