Pectus Excavatum, Pectus Carinatum and Poland's Syndrome

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PECTUS EXCAVATUM, PECTUS CARINATUM AND POLAND’S SYNDROME: SURGICAL CORRECTION BlueReview POSTED DATE: 3/23/2004 EFFECTIVE DATE: 7/1/2004 ______

COVERAGE:

Surgical repair of Pectus Excavatum and Pectus Carinatum is considered reconstructive and medically necessary if the patient meets ALL the following criteria:

1) Well documented evidence of complications arising from the sternal deformity. Complications include, but may not be limited to:

a) Cardiopulmonary impairment documented by respiration and/or cardiac function tests; b) Frequent lower respiratory tract infections; c) Asthma; or d) Exercise limitations; e) Atypical chest pain; AND

2) An EKG or echocardiogram has been done if a heart murmur or known heart disease is present to define the relationship of the cardiac problem to the sternal deformity; AND

3) A CT scan of the chest demonstrates a pectus index greater that 3.25. This is derived from dividing the transverse diameter of the chest by the anterior-posterior diameter.

Surgical repair of Pectus Excavatum and Pectus Carinatum is considered cosmetic if the procedure is primarily to alter appearance.

Surgical repair of the deformities associated with Poland’s Syndrome is considered reconstructive and considered medically necessary.

In order to determine medical necessity for the repair of Pectus Excavatum, Pectus Carinatum or deformities associated with Poland’s Syndrome, all of the following information must be provided:

1) Copies of diagnostic studies with radiologic interpretation;

2) Medical documentation describing indications for the procedure with signs and symptoms of complications arising from the sternal deformity;

3) History and physical; AND

4) Photographs of deformities. ______DESCRIPTION:

Pectus Excavatum (also known as Funnel Chest) is the most common of Blue Cross and Blue Shield of Texas, a Division of Health Care Service Corporation, a Mutual Legal Reserve Company* Southwest Texas HMO, Inc.* d/b/a HMO BlueÒ Texas * Independent Licensees of the Blue Cross and Blue Shield Association

PECTUS EXCAVATUM, PECTUS CARINATUM AND POLAND’S SYNDROME: SURGICAL CORRECTION BlueReview POSTED DATE: 3/23/2004 EFFECTIVE DATE: 7/1/2004 ______the major congenital deformities of the sternum. This occurs when the sternum is displaced posteriorly to produce a funnel shaped depression. The degree of sternal depression is assessed on a CT scan or lateral chest X-Ray by measuring either the sternovertebral distance or a ratio of sternovertebral distance to transthoracic diameter. The etiology is not precisely understood, but overgrowth of the lower costal cartilages and ribs is thought to be responsible. The extent of the depression varies from person to person. Pectus Excavatum can cause medical complications when cardiopulmonary function is impaired.

Both traditional open surgery (Ravitch repair) and newer minimally invasive techniques (Nuss repair) can be used to correct pectus excavatum. Both techniques generally improve chest appearance. They also 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.

Pectus Carinatum, often called "pigeon breast," is caused when the sternum (breastbone) is pushed outward and is also a congenital deformity of the sternum. The forward protrusion of the sternum often causes pain during exercise or during times of increased respiratory effort. It increases in severity with age and generally worsens during growth spurts that occur during late childhood and adolescence. While pectus carinatum occasionally interferes with overall health, it generally does not interfere with cardiopulmonary functioning. The etiology is not precisely understood, but overgrowth of the lower costal cartilages and ribs is thought to be responsible.

Surgery may be necessary to restore normal chest contour. Open surgery in boys is performed through a horizontal incision on the anterior chest well, usually just below the nipple area. In girls, this incision is placed to coincide with the lower breast margins when possible. The lower four to five 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 surgically fractured and placed in the correct position. The incision is usually closed with internal sutures that minimize scarring.

Poland's Syndrome is a congenital absence of the pectoralis major (chest muscle), absence or hypoplasia (underdevelopment) of the pectoralis minor, absence of costal cartilages, or hypoplasia of the breast and subcutaneous tissue. There can also be brachysyndactyly (missing fingers, missing phalanges, webbing between the fingers, one hand shorter than the other, or a combination of these deformities).

Blue Cross and Blue Shield of Texas, a Division of Health Care Service Corporation, a Mutual Legal Reserve Company* Southwest Texas HMO, Inc.* d/b/a HMO BlueÒ Texas * Independent Licensees of the Blue Cross and Blue Shield Association

PECTUS EXCAVATUM, PECTUS CARINATUM AND POLAND’S SYNDROME: SURGICAL CORRECTION BlueReview POSTED DATE: 3/23/2004 EFFECTIVE DATE: 7/1/2004 ______

As children with these deformities grow older, the defects usually become more severe. ______RATIONALE:

Correction of pectus excavatum and pectus carinatum is usually performed to restore normal appearance, although some patients may have associated medical complications, such as:

· Respiratory Infections, · Pneumonia, · Asthma, · Cardiac Arrhythmias, · Atypical chest pain, or · Exercise intolerance.

It is the severity of the medical complications and the objective results of diagnostic studies that will help determine the medical necessity for surgical repair of pectus excavatum and pectus carinatum.

Poland’s syndrome is a congenital defect and patients may require reconstructive surgery to the chest wall musculature, breasts and subcutaneous tissue. ______PRICING:

None ______REFERENCES:

· Morshuis, W., Folgering, H., et al. “Exercise cardiorespiratory function before and one year after operation for pectus excavatum.” Journal of Thoracic Cardiovascular Surgery (1994) 107: 1403-1409. · Morshius, W., Folgering, H., et al. “Pulmonary function before surgery for pectus excavatum and at long term follow-up.” Chest (1994) 105(6): 1646-1652. · Fonkalsrud, W.E., Dunn, J.C., et al. “Repair of pectus excavatum deformities: 30 years of experience with 375 patients.” Annuals of Surgery. (2000) 231(3): 443-448. · Swoveland, B., Medvick, C., et al. “The Nuss procedure for pectus excavatum correction.” AORN Journal (2001) 74(6): 828-841. · Kobayashi, S., Yoza, S., et al. “Correction of pectus excavatum and pectus carinatum assisted by the endoscope.” Plastic Reconstructive Surgery. (1997) 99(4); 1037-1045.

PECTUS EXCAVATUM, PECTUS CARINATUM AND POLAND’S SYNDROME: SURGICAL CORRECTION BlueReview POSTED DATE: 3/23/2004 EFFECTIVE DATE: 7/1/2004 ______

· Fonkalsrud, E.W., Beanes, S. “Surgical management of pectus carinatum: 30 years’ experience.” World Journal of Surgery. (2001) 25(7): 898-903. · Gatti, J.E. “Poland’s deformity reconstructions with a customized extra soft silicone prosthesis.” Annual of Plastic Surgery. (1997) 39(2): 122-130. · Borschel, G.H., Izenberg, P.H., Cederna, P.S. “Endoscopically assisted reconstruction of male and female Poland Syndrome.” Plastic Reconstructive Surgery. (2002) 109(5): 1536-1543. ______DISCLAIMER:

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