Description:
Chest wall deformities are usually congenital but may be acquired due to trauma. There are 3 types of congenital chest wall deformities: pectus excavatum, pectus carinatum and Poland’s syndrome. Chest wall abnormalities can cause cardiopulmonary problems due to physiologic impairment. Symptoms frequently do not appear during childhood because of the pliability of the chest. As the child grows, the chest becomes more rigid, causing the patient to develop symptoms.

Pectus excavatum (PE) is the most common congenital chest wall deformity and is found predominantly in males. Pectus excavatum is also known as cobbler’s chest, sunken chest, hallowed breast or funnel breast. This condition is thought to occur due to an excessive growth of the lower costal cartilages, which causes a concave appearance of the chest. The lower third part of the inwardly displaced sternum is usually most affected. PE is usually diagnosed within the first year of life and tends to worsen as the child grows. It typically worsens during puberty, which is a time of rapid growth. The deformity may be deeper on the right side than the left, possibly due to a rotation of the sternum. This can cause various degrees of sternal depression. Sternal depression may cause dyspnea, chest pain, palpitations and fatigue with mild physical activity. Other symptoms of PE are frequent respiratory infections, asthmatic symptoms and functional impairment due to respiratory impairment. PE is frequently associated with scoliosis, Marfan syndrome and congenital heart disease.

Pectus carinatum (PC) is not as common as pectus excavatum, but like PE, is most frequently seen in males. Pectus carinatum is also known as pigeon breast, chicken breast and keeled chest. PC appears as a flattened chest with a bowed out appearance. This condition is not usually diagnosed until the rapid growth that occurs during puberty. Patients with pectus carinatum may have symptoms such as restrictive air exchange. Depending on the degree of the defect, they may have difficulty with expiration of air out of the lungs. Conditions associated with PC are respiratory infections, rickets, asthma and cardiac abnormalities.

Poland syndrome is a rare birth defect and appears with lateral depression of the ribs, usually on the right side more often than the left side. Poland syndrome is also known as Poland’s anomaly or Poland’s syndactyly. It, like PE and PC, is seen most frequently in males. When the defect occurs on the left side of the sternum, the heart and lungs are more affected because they are covered only by a thin layer of skin and tissue. Poland syndrome appears with absences or incomplete development of the pectoralis minor muscles and of the costal cartilages. Hypoplasia of the breast, subcutaneous tissue, lack of axillary hair and hand and upper extremity defects are also signs. Portions of the second, third and fourth ribs may be partially absent, as well as upper costal cartilage.

Diagnosis and Evaluation:
The severity of the chest wall abnormality is dependent upon the depth, symmetry and width of the deformity. Chest radiographs are commonly used to determine the degree of chest wall deformity. Plain anteroposterior and lateral radiographs are used to determine the Haller index (a measurement of chest diameter). Cross-sectional imaging such as computerized tomography (CT) scans and magnetic resonance imaging (MRI) may be used to evaluate the degree of cardiac compression (ventricular compression), pulmonary compression and cardiac displacement. CT scan ratios that reveal transverse to AP diameter of greater than 3.25 are considered significant for pectus excavatum. A normal chest has an index of 2.5 (Malek et al., 2003; Fonkalsrud, 2004). Another method being investigated to determine the severity of chest wall deformity is the correction index that involves measuring the minimum distance between posterior sternum and anterior spine and the maximum distance between anterior spine most anterior portion of the chest. The difference between the two is then divided by the latter ( × 100) to give the percentage of chest depth representing the defect (St. Peter et al., 2011). The correction index is a newer method proposed to define the pectus deformity; however, in comparison to the Haller Index, the correction index has no clearly established threshold for determining the degree of chest wall deformity.

Echocardiography and/or electrocardiography may also be used to evaluate cardiac status. Respiratory status can be determined with the use of pulmonary function studies. In some cases, pulmonary function studies may reveal a restrictive pattern (incomplete lung expansion) and a subsequent decrease in pulmonary volume and reserve. The forced expiratory volume (in one second) (FEV1), forced vital capacity (FVC) and total lung capacity (TLC) are reduced while the ratio of FEV1/FVC may be normal or increased in the presence of restrictive airway disease. TLC < 80 percent predicted value signifies restrictive pulmonary disease. 

The diagnosis of Poland syndrome is usually obtained by clinical exam. Chest wall abnormalities and determining the presence of latissimus dorsi muscles may require CT scans while chest radiographs may be utilized to evaluate rib formation.

Policy:
Surgical Treatment of Pectus Excavatum, Pectus Carinatum, or Poland syndrome may be Medically Necessary and reconstructive when the following guidelines are met:

1. When there is functional impairment, documented by one of the following: The procedure is expected to correct the functional impairment.
   • Decreased cardiac output and/or abnormal pulmonary function during exercise
   • Anticipation of future cardiovascular compromise
   • Signs or symptoms that impair the patient’s ability to participate in usual activities, such as shortness of breath (dyspnea) at rest or on exertion
   • Arrythmias or clinical stigmata of decreased cardiac output.
2. The procedure is expected to correct the functional impairment.
3. The anatomical criterion for the condition is met:
   • For treatment of Pectus Excavatum, the Haller Index is greater than or equal to 3.2.
   • For treatment of Pectus Carinatum, the Haller Index is less than or equal to 2.0.
   • For treatment of Poland syndrome, when rib formation is absent.

If the above criteria are not met surgical treatment is considered Not Medically Necessary.  

Non-surgical orthotic compressive bracing to treat patients with pectus carinatum is considered MEDICALLY NECESSARY when the following indications are met:

1. Documented cardiac or pulmonary impairment
2. Skeletal growth is incomplete
3. Willingness and ability to adhere to the bracing schedule
4. Brace is custom made

Policy Guidelines:
The Haller index, or pectus severity index, is the most common used scale for determining the severity of chest wall deformities. The Haller index is obtained by dividing the inner width of the chest at its widest point by the distance between the posterior surface of the sternum and the anterior surface of the spine. This measurement uses the deepest level of the inner sternal depression to the anterior aspect of the vertebral body. CT scans are used to define the index. At times the rotation, asymmetry and underlying chest structure may make this measurement difficult to obtain. A normal chest has a Haller index of about 2.5.

Rationale: 
Surgical Treatment
Indications for surgical correction are controversial and vary widely. Surgical repair is offered primarily as a method of improving cosmesis and psychological factors but may be necessary to improve cardiopulmonary function in some patients, as the disfigurement may be accompanied by physiologic impairment.

Pectus Excavatum/Pectus Carinatum: While the optimal age for surgical repair is generally between the ages of 11 and 18 years, and may be performed in adults, each case must be reviewed individually for the presence of impaired cardiopulmonary symptoms. Criteria that may be used to demonstrate severe PE and the need for surgical repair include (Goretsky et al., 2004):

• • A Haller CT index greater than 3.25.
  • Pulmonary function studies that indicate restrictive or obstructive airway disease.
  • A cardiology evaluation, where the compression is causing murmurs, mitral valve prolapse, cardiac displacement, or conduction abnormalities on the echocardiogram or EKG.

Surgery for PE may be performed using any one of several techniques, including a sternal osteotomy (i.e., a modified osteotomy that involves supporting, removing and repositioning the sternum) or implantation of a Silastic mold in the subcutaneous space to fill the defect without altering the thoracic cage. Surgical correction often employs a metal bar behind the sternum; the bar may be removed in one to two years, after remolding has occurred. The standard surgical procedure is the open Ravitch procedure, which involves extensive dissection, cartilage resection and sternal osteotomy. More recently, minimally invasive techniques, such as the Nuss procedure (i.e., a minimally invasive repair of pectus excavatum [MIRPE]), have been utilized that involves the insertion of a convex steel bar beneath the sternum through small thoracic incisions. These recently developed minimally invasive methods do not require cartilage resection or osteotomy.

For correction or improvement of PC, bracing is used to exert pressure on the anteroposterior direction. More specifically, bracing is recommended for skeletally immature children with mild deformities; however, the candidate must be motivated to wear the brace (Goretsky et al., 2004). If bracing is unsuccessful, surgical repair may be considered. The initial surgical repair for PC involves removing the affected cartilages and mobilizing the skin and pectoralis muscle flaps. To straighten the sternum, any one of the following surgeries may be performed:

• • An osteotomy
  • A subperichondrial resection of the involved costal cartilages
  • A wedge-shaped osteotomy in the anterior sternal plate

Poland Syndrome: Patients with Poland syndrome typically present for surgical reconstruction to improve physical appearance and correct breast asymmetry. Surgical procedures involving the breast and muscles to achieve symmetry are considered cosmetic, since there is no significant impairment being corrected. Patients who present with absent ribs are also considered candidates for surgical repair (Townsend, 2004). In such cases, operative reconstruction may eliminate paradoxical motion, improving respiratory impairment. For more severe conditions, reconstructive surgery also provides protection of the underlying heart and lung structures. While there are a variety of surgical techniques to correct the deformity, a common approach is to use the latissimus dorsi muscle with autologous rib grafts to reconstruct the chest wall.

Surgical treatment of Poland Syndrome often consists of reconstruction of the breast and nipple on the affected side by a plastic surgeon, in addition to surgical repair of the chest wall muscles and hypoplastic bone. Surgery is performed early (approximately age 13) in males; however, in females, reconstructive surgery is often deferred until breast development is complete. If there are rib abnormalities and paradoxical motion, the rib grafts or other chest wall stabilization may occur before breast development is complete. Generally, reconstruction of the breast involves tissue expansion, placement of permanent breast implants and may involve myocutaneous or latissimus dorsi flaps if there is an associated anomaly of the pectoral muscle. Nipple-areolar reconstruction is generally performed at a later stage. Consequently, for patients with Poland syndrome, treatment provided before complete breast development may involve the use of tissue expanders in the affected side that can be inflated periodically to match development of the unaffected breast. Expanders allow for tissue expansion and accommodation of a permanent implant and latissimus muscle upon completion of breast development. Once breast development is complete, the expander is removed and a permanent prosthesis is inserted and breast reconstruction is performed.

Surgical repair of the chest wall includes the reconstruction of the pectoral muscles and resection of deformed cartilages. This repair typically involves muscle transfers and/or flaps to match normal development of the unaffected side, reconstruction of the axillary line and correction of infraclavicular flattening. If necessary, reconstruction of the rib cage may be performed at this time with autologous rib grafts.

Pectus Carinatum
External Bracing
In 2018, de Beer et al. performed a systematic review, the authors noted that patients with pectus carinatum have been treated with orthotic braces since the 1970s. (11) By applying external pressure to the anterior chest wall, a normal chest shape can be restored. However, high patient treatment dropout rates were reported because of the subjectively high pressures applied to the patient´s skin. An electronic database search was completed: 14 studies were identified (eight studies between 2008 – 2018 were included). The 8 studies included 1,185 patients. The median age was 14 years (range 2 – 28) and 87% were male. The mean study follow-up period was 16 months; 44% of patients were still under treatment, 29% of patients successfully completed treatment. 6% dropped out and 21% were lost to follow-up. Complication noted included Mild chest discomfort or tightness was reported in 12% and skin lesions occurred in 5.1%. The overall recurrence rate was 2.6%. The reviewers concluded dynamic compression appears to be a safe, non-invasive and efficient treatment to correct pectus carinatum in patients with a non-rigid thorax. Patients experience less discomfort, which in turn results in better compliance. However, accurate selection of patients based on age, pressure of initial correction and motivation is important and an objective scoring system to assess the esthetic and long-term physical and psychological results of the treatment is needed.

In 2017, Emil et al. reported on a 4-year prospective study to establish factors that can prognosticate outcomes of bracing for pectus carinatum. (12) Prospective data were collected on all patients enrolled in a dynamic bracing protocol from July 2011 to July 2015. Pressure of correction (POC) was measured at initiation of treatment, and pressure of treatment (POT) was measured pre- and post-adjustment at every follow-up visit. Results reported included of 114 patients, 64 (56%) succeeded, 33 (29%) were still in active bracing, and 17 (15%) failed or were lost to follow-up. In successful patients, active and maintenance bracing was 5.66 ± 3.81 and 8.80 ± 3.94 months, respectively. Asymmetry and older age were significantly associated with failure. Multivariable Cox proportional hazard analysis of time-to-maintenance showed that asymmetry (p = 0.01) and smaller first drop in POT (p = 0.02) were associated with longer time to reach maintenance. The authors concluded that pressure of correction does not predict failure of bracing, but older age, asymmetry, and smaller first drop in pressure of treatment are associated with failure and longer bracing duration.

In 2020, Fraser et al. evaluated the impact of manipulation and a tailored program for compressive bracing on the quality of life of patients with flexible pectus carinatum. (13) Two hundred forty-nine sequential patients attending a clinic for assessment of pectus carinatum deformities underwent outpatient manipulation and then followed a prescribed schedule of continuous external compressive bracing but without significant progressive tightening. There was successful sustained reduction of the deformity in 244 patients with high reported rates of concordance (98%) and satisfaction (94%). Patients experienced a reduction in symptoms of anxiety and depression (P < .001) and had improved body satisfaction (P < .001). Mild skin irritation occurred in 18% of patients (n = 44), and there were 2 severe cases of skin irritation, 1 of which resulted in abandonment of bracing. The authors concluded that manipulation and non-tightening compressive bracing was associated with complete concordance, high levels of successful bracing, improved confidence and reduced psychological morbidity.

Hunt and Patel noted in a 2020 review that with external compressive orthotics, similar outcomes have been reported compared with surgical intervention for pectus carinatum chest wall deformities. (14) The authors note there have been fewer major complications reported with dynamic compression bracing on the chest wall; however, they note that the Achilles heel of this approach still lies with treatment tolerability and compliance. A literature search was performed to evaluate the evidence concerning the techniques, modifications, and outcomes associated with external compressive bracing in the setting of pectus carinatum. Sixteen articles were integrated after literature review and data were collected on methods of assessing pectus carinatum (degree of severity as well as type), patient selection protocol (i.e., suitability for external compressive bracing), the bracing protocol itself, duration of treatment, metrics used to assess outcome, and success, compliance, and dropout rate, and length of long-term follow-up. The authors noted that compressive external bracing appears to be a safe and well tolerated non-surgical treatment option for young patients with flexible pectus carinatum deformities. However, there is still a need for robust level I randomized data from multiple centers with a clearly standardized bracing protocol, objective measurement of outcomes, and recording of results at the end of the bracing treatment program in sufficiently powered sample sizes over a significant follow-up period.

References:

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2. Bawazir OA, Montgomery M, Harder J, Sigalet DL. Midterm evaluation of cardiopulmonary effects of closed repair of pectus excavatum. J Pediatr Surg. 2005 May;40(5):863-7.
3. Boas S. Pectus Excavatum. Skeletal Diseases Influencing Pulmonary Function. In: Behrman RE, Kliegman RM, Jenson HB, editors; Nelson Textbook of Pediatrics, 19th ed. Copyright© 2011.Ch 411.
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Coding Section 

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.   

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other nonaffiliated technology evaluation centers, reference to federal regulations, other plan medical policies and accredited national guidelines.

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