Congenital Chest Wall Conditions
Why do chest wall problems occur?
Usually the cause is unknown, but sometimes chest wall problems occur as part of a syndrome, such Poland’s syndrome. Occasionally injury to the chest in childhood such as burns or radiotherapy can result in developmental chest wall problems. The spectrum of problems is best described according to the structure(s) affected.
The breastbone or sternum and the associated ribs can develop abnormally resulting in undue hollowing or unsual prominence. Pectus excavatum or ‘funnel chest’, is a congenital condition where the breastbone takes on a concave, hollow appearance resulting in an unusual chest contour. It is thought to be an inherited condition. Pectus excavatum can sometimes be associated with Marfan and Ehlers-Danlos syndromes.
Pectus carinatum is often referred to as ‘pigeon chest’ is where the breastbone bows forwards.
Flattening or absence of ribs can also be a cause of chest wall asymmetry, it can be a component of Poland’s syndrome.
Unusual bowing of the ribs at the back of the chest can occur in scoliosis.
In most instances the aim of surgery is purely to improve appearance, but some of the major abnormalities of the sternum and ribs can affect breathing and require correction to help this.
What surgery is available, and what techniques are involved?
More minor degrees of chest wall abnormality are often best left alone provided the patient accepts this.
The contour dip in pectus excavatum can be disguised either by lipofilling or by using a custom-made implant. If a custom-made implant is to be used a mould is taken of the chest and a specially fabricated silicone implant created to fill the dip. Once the implant is ready an operation is carried out to insert the implant via a small incision. For more major degrees of pectus excavatum, an operation to reshape and reposition the breastbone and its associated rib cartilages may be recommended. This is a more major operation and may be done by a thoracic surgeon and/or a plastic surgeon. A much bigger incision is needed, and a longer hospital stay and recovery is required.
Pectus carinatum can only really be corrected by an operation to reshape or reposition the breastbone.
Problems with the ribs in scoliosis are sometimes corrected by the orthopaedic surgeon as part of the management of the spinal curvature.
Rib flattening and muscle absence is disguised by lipofilling, custom-made implants and muscle transfer operations as described for Poland’s syndrome.
Is this surgery available on the NHS?
Surgery for the purposes of improving appearance is rationed on the NHS. In some parts of England, consultations and operations for patients with developmental chest wall problems will be allowed without any restriction, but in other areas treatments are only available in certain circumstances. These exceptional circumstances vary from region to region.
In general, patients with more marked physical problems might be considered exceptional. Your GP will be able to tell you about the local rules that apply for where you live. BAPRAS is unhappy that this type of postcode rationing occurs and has worked with the Department of Health in drawing up guidelines for commissioning cosmetic procedures on the NHS. Different rules also apply in Wales, Scotland and Northern Ireland.
If NHS treatment is not available to you, you will have to consult a plastic surgeon as a private patient and pay for the operation yourself.
Who will I see as a patient?
You may be referred to:
• A plastic surgeon
• A thoracic surgeon
• An orthopaedic surgeon
• A clinical nurse specialist
• A paediatrician
What should I expect in terms of treatment, procedure and outcomes?
When considering corrective surgery of this sort the patient and team treating them must fully discuss the likely benefit and balance this against what is involved in the surgery and any possible risks and complications. The correction of chest wall abnormalities can involve a series of operations. Patients may have to be be prepared for a progressive series of interventions depending on how their problem develops and how it responds to treatment. At each stage your treating team will inform you of what is involved and discuss the aims and likely outcome of the operation.
Many procedures are relatively simple day case operations, but sometimes a brief hospital stay is needed. Operations to reshape and reposition the breastbone are major operations requiring several days in hospital and a recovery period lasting several weeks. All operations result in scarring, but operations are planned to minimise the scars and put them in inconspicuous places.
Every attempt will be made to restore perfect shape and symmetry, but it must be recognised that some imperfection is likely and will have to be accepted. If correction can be made using the patient’s own tissues the result is likely to be static and permanent. If implants are needed it is likely that further operations will be needed as the years go by in order to maintain the result.
patient.info - chest deformity