Pectus excavatum repair, also called "funnel chest repair" or "chest deformity repair," is a type of surgery performed to correct pectus excavatum, a deformity of the front of the chest wall with depression of the breast-bone
(sternum) and rib (costal) cartilages. It is sometimes associaated with Marfan or Poland syndromes.
Purpose
The chest consists of the rib cage and sternum, which protect the upper-abdominal cavity and its contents. Pectus excavatum, also called "funnel chest" or "depressed sternum" is a deformity that is usually diagnosed shortly after birth. In some people, it is not visible until they are older. The exact cause is not known, but it is believed to be due to overgrowth of the rib cartilage connected to the sternum, which results in the sternum being pushed backward toward the spine. Most people have no symptoms, but if the breastbone is pushed back far enough, heart and lung function may be affected. The purpose of pectus excavatum repair surgery is to correct the deformity to improve physical appearance, posture, and breathing.
Demographics
In the United States, pectus excavatum is the most common chest wall deformity observed in children, occurring more commonly in boys than in girls. Pectus excavatum tends to run in families. The funnel chest usually progresses as the child grows, often showing a dramatic deterioration during the puberty growth spurt.
Pectus excavatum repair is technically easiest to perform in preadolescent children, and the recovery is faster. However, almost half of the patients undergoing the operation are teenagers. Repair is rarely performed on children under eight years of age. In recent years, a large number of adults over the age of 21 years have undergone repair with equally good results as those observed with children.
Description
Pectus excavatum repair is always performed with the patient under general anesthesia. An epidural catheter is inserted for the management of pain after the operation. The surgeon makes two incisions over the sternum, on either side of the chest, for insertion of a curved steel bar or strut under the sternum. He or she proceeds to remove the deformed cartilages. The rib lining is left in place to allow renewed cartilage growth. The sternum is then repositioned, and the metal strut is placed behind it and brought out through the muscles and skin for future attachment to a brace, which will stay in place six to 12 weeks. The metal strut is fixed to the ribs on either side, and the incisions are closed and dressed. A small steel grooved plate may be used at the end of the bar to help stabilize and fix the bar to the rib. A blood transfusion is not required during surgery. The surgeon may insert a temporary chest tube to re-expand the lung if the lining of the lung is entered.
A common technique is the Nuss procedure, developed in 1987 by Dr. Donald Nuss, a pediatric surgeon at Children's Hospital of the The King's Daughters and Eastern Virginia Medical School in Norfolk, Virginia. The procedure is minimally invasive, and results in very little blood loss and short recovery times.
Leonard procedure
Another surgical approach that drastically reduces the time required for surgery is the Leonard procedure, developed by Dr. Alfred Leonard, a Minneapolis thoracic and pediatric surgeon. This operation does not violate the chest, and is combined with a bracing technique.
Diagnosis/Preparation
A pediatrician diagnoses pectus excavatum after observing a child when he or she inhales, exhales, and rests. The pediatrician also calculates the depth of the chest from front to back using x rays of the chest to determine whether the diameter is shorter than average, as is the case with funnel chest. The heart is usually larger and displaced to the left. The pediatrician also evaluates lung capacity using exercise tests and lung scans that can reveal mismatched lungs.
Other diagnostic tests may include:
Electrocardiogram (ECG or EKG). This test records the electrical activity of the heart, and shows abnormal rhythms (arrhythmias or dysrhythmias).
Echocardiogram (echo). This test evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that yields a moving picture of the heart and its valves.
Before surgery, a bone density test is performed to ensure that the patient does not have soft bones that would deform again right after the surgery. After a complete health history is taken, a patient whose condition is considered severe enough to warrant surgery is sent for a CT scan and further evaluation of his or her pulmonary function.
Because of the great variablity of pectus excavatum among those who have it, custom-made bars (or braces) must be used. The brace is a light vest to which the deformity-correcting wire will be attached at surgery. Patients are fitted with the brace prior to surgery.
Aftercare
Usual recovery time in the hospital is four to five days. Attention is paid to post-operative pain management. The patient is encouraged to breathe deeply, and receives assistance with movement (to avoid dislodging the bar). After discharge, the patient slowly resumes a normal, but restricted, activity level. Most children are able to return to school in two to three weeks, with exercise restrictions for six weeks (no physical education classes, heavy lifting, or athletics).
The pectus excavatum support bar is removed under general anesthesia two to four years after insertion, usually on an outpatient basis. In most cases, patients are able to leave the hospital within one to two hours after bar removal.
Risks
Risks associated with pectus excavatum repair include those normally associated with the administration of anesthesia (such as adverse reactions to medications and breathing problems), and risks associated with any surgery (such as bleeding and infection). Specific pectus excavatum surgery risks may include lung collapse (pneumothorax) and the recurrence of the funnel chest. Bar displacement may occasionally require repositioning.
Normal results
Pectus excavatum repair, in almost all instances, restores the ability of patients to participate in full activities, even strenuous activities and athletics. Also, there is a marked improvement in the patient's self image.
Morbidity and mortality rates
According to the National Institutes of Health (NIH), excellent results (95–98%) are reported over a lengthy follow-up time of 25 years. Long-term follow-up (over 15 years) shows that the Nuss procedure provides excellent results with less than 5% recurrence of the deformity after the bar is removed.
Alternatives
Mild cases of pectus excavatum may respond to an exercise and posture physiotherapy program. Many patients with rounded shoulders and a slouching posture have benefited from these techniques, with or without additional surgical correction. However, body-building exercises usually result in worsening of cosmetic appearance due to the enhancement of the pectoral muscles.
BOOKS
Pearson, F. G. Thoracic Surgery. Philadelphia: W. B. Saunders Co., 2002.
Ravitch, M. M. Congenital Deformities of the Chest Wall andTheir Operative Correction. Philadelphia: W. B. Saunders Co., 1977.
PERIODICALS
Engum, S., F. Rescorla, K. West, T. Rouse, L.R. Scherer, and J. Grosfeld. "Is the Grass Greener? Early Results of the Nuss Procedure." Journal of Pediatric Surgery 35 (2000): 246-51.
Genc, A., and O. Mutaf. "Polytetrafluoroethylene Bars in Stabilizing the Reconstructed Sternum for Pectus Excavatum Operations in Children." Chest 110 (July 2002): 54-7.
Hebra, A., B. Swoveland, M. Egbert, E.P. Tagge, K. Georgeson, H.B. Othersen, and D. Nuss. "Outcome Analysis of Minimally Invasive Repair of Pectus Excavatum: Review of 251 Cases." Journal of Pediatric Surgery 35 (2000): 252-7.
Jacobs, J. P., J.A. Quintessenza, V.O. Morell, L.M. Botero, H.M. van Gelder, and C. I. Tchervenkov. "Minimally Invasive Endoscopic Repair of Pectus Excavatum." European Journal of Cardiothoracic Surgery 21 (2002): 869-83.
ORGANIZATIONS
American Pediatric Surgical Association (APSA). 60 Revere Drive, Suite 500, Northbrook, IL 60062. (847) 480-9576. <www.eapsa.org>.
Southern Thoracic Surgical Association. 633 N. Saint Clair St., Suite 2320, Chicago, IL, 60611-3658. (800) 685-7872. <www.stsa.org/>.
