Pectus Excavatum
Pectus Carinatum
What is Pectus Excavatum?
Clinical Features
Pectus excavatum (sunken chest) is a dip or concave recession in the chest wall. It is four times as common as pectus carinatum (pigeon chest). It is more frequent in males, and may be symmetrical or asymmetrical. These children are usually thin: muscular and ligamentous laxity is marked. The sternal depression is maximal at the sterno-xiphisternal junction, but may extend up the sternum to any level.
When most of the sternum is affected, the depression is shallow and the deformity may be described as the ‘saucer’ type. A localised depression is compatible with a well-developed upper chest, and the very localised ‘funnel’ may be referred to as the ‘cup’ type.
In asymmetrical lesions, the sternum is rotated, producing prominence of the costal cartilages on one side and recession of those on the other.
The costal margin (bottom of the rib cage) on each side may become generally everted or protrude known as costal flaring. Postural kyphosis (forward bending of the spine) is common, and scoliosis (lateral bending) not infrequent.
The deformity may be of any degree of severity, from being barely detectable to one in which the lower sternum seems to almost touch the front of the vertebral column.
One third may be present at birth, but the most common history is that it becomes much more apparent during the pubertal growth spurt, when rapid growth in height is occurring. In general there is a tendency for the deformity to progress until growth ceases.
Symptoms
In the more severe cases there may be an effect on cardiac function and many of those patients will report shortness of breath with significant exertion. More commonly the symptoms relate more to the psychological effects.
Psychological features may become apparent from an early age. The child resents being ‘different’ and, when undressed for examination, brings the arms together to try to conceal the shape of the chest. Older children may refuse to go swimming for fear of becoming an object of attention. Comments from schoolmates may be unkind. The psychological effects can completely alter the child’s personality and affect social development.
Diminished exercise tolerance is uncommon, and confined to those with the most severe deformities. Pain may be present. The deformity is not responsible for any increased tendency to respiratory infections.
The progression and severity of the condition, and its significance to the patient and parents can best be assessed by serial examinations at yearly intervals, with photographs recording the deformity at each visit.
A CT or MRI of the chest is sometimes taken to demonstrate the position of the heart and to establish the distance of the sternum from the vertebral column.
Treatment
Surgery is often performed as the child approaches puberty. A minimally invasive corrective procedure is usually undertaken named the “Nuss” procedure .This involves placing a curved metal strut behind the sternum, to correct the deformity , using a minimally invasive technique.
Occasionally a classical sternochondroplasty (Open repair) will be more appropriate. The chest cage is exposed through a transverse incision, with elevation of muscle flaps. The sternum is mobilised and the costal cartilages divided to correct the deformity. The corrected position of the sternum is maintained by struts (usually of steel) placed behind the sternum.
Post-operative management
Both operations cause considerable discomfort and require careful pain management. We will usually do this in hospital using an epidural catheter followed by oral pain relief post-operatively. The pain usually takes about 6 weeks to dissipate.
In the first 3 months post operatively there should be strict avoidance of upper chest rotation, pushing back on your arms and upper body exercise. This may be resumed after 3 months. Heavy contact sports are however discouraged while the Nuss bar is in, and for 1 year post sternochondroplasty (open repair)
The Nuss bar is removed between 2-3 years after it was inserted. By this time, the costal cartilages have re-modelled keeping the sternum in its new position. This requires a second procedure. The stay in hospital for this procedure is usually 1-2 days.
What is Pectus Carinatum?
Clinical Features
Pectus Carinatum (PC) is a chest wall deformity where there is overgrowth of the cartilage between the ribs and the sternum, also known as the breastbone, causing the sternum (breastbone) to protrude. Although the shape of the chest wall is distorted, it does not usually affect the internal organs.
PC is most common in adolescent males. Four times as many males as females have PC. It becomes more noticeable during puberty when the body is going through its rapid growing stage. PC continues until skeletal maturity is reached. Skeletal maturity usually occurs around 18 years of age.
PC does not cause any functional problems but could impact on you/your child’s social life or be a cause of concern or anxiety for you/your child ie: exposing the protrusion at beach/pool, etc.
Treatment
The deformity cannot be improved by any form of exercise. Encouragement of participation in physical activity helps some children to become less self-conscious about lesser degrees of depression deformity. In selecting patients for surgical correction of the deformity, it is important that the patient wants to have the correction.
The treatment for PC would depend on how compressible the deformity is. A compressible deformity is more likely to respond to bracing.
In the event you choose to undergo treatment we would undertake a series of baseline investigations including, lung function tests, 3-D photography, and sometimes an MRI.
Bracing
In the event that it is compressible, PC is treated using a chest orthosis, also known as a brace. The orthosis/brace helps to correct the shape of the chest wall by compressing or squeezing the overgrown ribs or breastbone back to a ‘normal’ position. The orthosis is worn until satisfactory results are achieved and the chest wall can maintain the correct position.
There are two main phases of wear: Corrective and Maintenance.
- Corrective Phase: The first 3-6 months during which the orthosis is worn 23hrs a day.
- Maintenance Phase: When the chest overgrowth has flattened sufficiently, the orthosis is worn during the night for eight hours. During this phase patients continue to monitor their chests to make sure there is no relapse. This phase usually lasts another 3-6 months.
- The orthosis should not be worn during high intensity activities such as sport.
- Your Orthotist will show you how to wear the orthosis correctly and adjust it appropriately.
Treatment is monitored by regular reviews and photography. The chest orthosis is reviewed one month after fitting and then every two months after that. We and the orthotists will arrange reviews and photograph the chest wall to monitor the progress of your child’s chest and adjust the orthosis as required.
Surgical
Surgery is reserved for cases where bracing has not been successful either due to compliance or rigidity of the lesion.
Surgery is often performed as the child approaches puberty. In the classical sternochondroplasty (Open repair), the chest cage is exposed through a transverse incision, with elevation of muscle flaps. The sternum is mobilised and the costal cartilages divided to correct the deformity
Post-operative management
The operation can cause considerable discomfort and require careful pain management. We will usually do this in hospital using an epidural catheter followed by oral pain relief post-operatively. The pain usually takes about 6 weeks to dissipate.
In the first 3 months post operatively there should be strict avoidance of upper chest rotation, Pushing back on your arms and upper body exercise. This may be resumed after 3 months. Heavy contact sports are discouraged for 1 year post sternochondroplasty (open repair)