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What is pectus excavatum?
Commonly called “funnel chest,” pectus excavatum is a depression or hollow caused when the sternum (breastbone) is abnormally pushed inward. The depression in the chest is due to abnormal growth of the cartilage that attaches the sternum to the ribs.
Because of the deep depression, the abdomen often protrudes and gives the appearance of a potbelly in younger children. If both sides of the breastbone are depressed in an equal fashion, the defect will appear symmetrical. However, in many cases, the chest wall appears asymmetric, with the left side of the chest being wider than the right side. In such cases, the sternum is abnormally rotated.
Pectus excavatum occurs in 1 in 500 to 1,000 children. It may be minimal, with only slight depression of the chest or it may be quite severe, causing compression of the heart and lungs and altering their functioning. The abnormality increases with age and often worsens during the growth spurts that occur during late childhood and adolescence. It stabilizes after skeletal growth is complete.
What causes pectus excavatum?
While the cause of this abnormality is unknown, the fact that it tends to occur in families suggests that genetics may play a role. Excessive growth and structural abnormalities of the cartilage (tough, connective tissue) of the ribs and breastbone are present in pectus excavatum. Also, pectus excavatum is seen in some inherited connective tissue disorders such as Marfan syndrome, homocystinuria, and Ehlers-Danlos syndrome.
What are the symptoms of pectus excavatum?
Although some children are asymptomatic (experience no symptoms), symptoms usually vary with the severity of the abnormality. Younger children are thus less symptomatic than older children.
- Children with less severe defects often have mild respiratory problems or frequent and prolonged colds.
- Children with moderate to severe pectus deformities often experience breathing difficulties upon exertion and decreased exercise tolerance due to impaired lung and/or heart function. Lung capacity is decreased and the filling capacity of the heart chambers is restricted due to compression from the sternum. These symptoms can be quite severe at times, thus limiting the child’s activity level.
- Growing adolescents may also experience chest pain in the area of rib cartilages.
How is pectus excavatum diagnosed?
Pectus excavatum can be diagnosed by physical examination. Chest X-rays are largely unhelpful. Diagnostic imaging tests such as computed tomography scan (CT) are used to quantify the severity of the defect and permit calculation of a pectus index. Children with a pectus index of greater than 3.5 are advised to undergo repair.
An electrocardiogram (ECG or EKG) and an echocardiogram (echo) may be performed. An ECG is a test that records the electrical activity of the heart, showing abnormal rhythms and detecting heart muscle stress. An echo is a procedure that uses sound waves to study the structures and function of the heart.
Additionally, pulmonary function tests (PFTs) may be performed. PFTs can help detect breathing problems that may be related to the pectus excavatum.
How is pectus excavatum treated?
Mild abnormalities occasionally correct themselves early in childhood. Children with moderate to severe abnormalities may require surgery, which is usually not performed in children younger than age 6. Since children have softer cartilage and more flexible bones than adolescents, the operation is easier to perform on them and the cosmetic results are superior. However, good outcomes are now being obtained even in young adults.
Infants and very young children are usually not considered for operation unless very severe abnormalities or other illnesses necessitate earlier surgery.
Both traditional open surgery (Ravitch repair) and newer minimally invasive techniques (Nuss repair) can be used to correct pectus excavatum. We generally recommend the Nuss repair, unless there is marked asymmetry of the sternum and anterior chest wall. Both techniques generally improve chest appearance, improve rib and sternum configuration, as well as cardiopulmonary (heart and lung) functioning. Both techniques result in a return to normal activity and improved exercise tolerance within the first few months following surgery.
The determination of which surgical approach to use is based on a number of factors. These factors include:
- The surgeon’s level of expertise with each of these two techniques
- The severity of the defect and its symmetry (in the middle or more to one side)
- The child’s age
Open surgery in boys is performed through a horizontal incision on the anterior chest wall, usually just below the nipple area. In girls, this incision is placed to coincide with the lower breast margins when possible. The lower 4-5 cartilages that are abnormal are removed, leaving the perichondrium (the lining that envelops the outer portion of rib cartilage). This allows the cartilage to regrow in its new position. The sternum is supported in its correct position by a thin flat metal bar that corrects and stabilizes it during healing. The incision is usually closed with internal sutures that minimize scarring. Surgical drains are placed to prevent fluid accumulation and are generally removed prior to discharge from the hospital.
The length of hospital stay following surgery is typically 4 to 5 days. Children often experience some discomfort for several weeks. Postoperative epidural analgesia catheters or intravenous narcotics may be required for several days following surgery. Milder pain is managed with oral pain medication.
Outpatient surgery to remove the supporting bar is usually performed 6 to 12 months later.
Cosmetic and physical outcomes in patients who have undergone surgery in mid-childhood or early adolescence are good to excellent, with less than 5% requiring another operation for significant pectus excavatum recurrence. An additional5- 10% of patients have some residual (remaining) abnormalities that sometimes worsen with adolescence.
Minimally Invasive Surgery (Nuss Procedure)
Minimally invasive surgical techniques have been used for more than a decade. With this approach, two small lateral incisions are made. A bar that has been shaped to the desired chest contour is inserted into and across the chest and positioned underneath the sternum. This is done using the guidance of an endoscope (instrument used to visualize the inside of the chest). The bar is held in place by sutures and by a small metal plate that prevents rotation of the bar as the chest is reshaped. This procedure is very similar to placing braces on teeth, which facilitates their correct realignment. Sometimes a second bar is placed to maintain the correction. This is generally necessary in teenagers.
Length of hospital stay is usually 3 to 5 days. Children often experience more discomfort initially with the minimally invasive procedure. Postoperative epidural analgesia catheters or intravenous narcotics may be required for several days following surgery. Milder pain is managed with oral pain medication, which may be needed for up to six to eight weeks in teenagers. Activity is slowly resumed, with limitations on exercise until the bar is healed in place, usually four to six weeks.
In contrast to the brief duration (6 to 12 months) in which the bar is left in place with open surgery, with this approach it is left in place for 2 to 3 years, allowing the chest to remold. The bar is then removed during outpatient surgery.
In our experience, short-term outcomes with minimally invasive surgery are at least comparable to outcomes with open surgery. They result in a better cosmetic outcome with less scarring (due to smaller incisions), good restoration of normal chest contour, and good muscle function. Although the minimally invasive approach is comparatively new, long-term outcomes currently appear to be at least as good as those with traditional open surgery.
Source: Cincinnati Children’s Hospital Medical Center