Reconstructive Repair of Pectus Deformity Form

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Reconstructive Repair of Pectus Deformity - Medical Policy

Updated Revision Effective: September 1, 2025 Policy Number:

UM712POL

Approval Date: 6/24/2025

Line(s) of Business:

Commercial
Medicare Advantage
Medicaid (BeHealthy)

Description

Chest wall deformities are usually congenital but may be acquired due to trauma. There are three 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. Indications for reconstructive repair of Pectus Deformities (Excavatum or Carinatum) 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. While the optimal age for surgical repair is generally between the ages of 11 and 18 years, it may be performed in adults. Each case must be reviewed individually for the presence of impaired cardiopulmonary symptoms. Surgery for Pectus Excavatum (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 Pectus Carinatum (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; or a wedge-shaped osteotomy in the anterior sternal plate.

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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.

 Line of Business

Commercial:

• HNE has adopted InterQual* criteria for the following procedures:

o Abramson Repair, Minimally Invasive Repair of Pectus Carinatum, Minimally Invasive Repair of Pectus Excavatum, Nuss Procedure, Open Repair of Pectus Carinatum, Open Repair of Pectus Excavatum, Ravitch Procedure: CP:Procedures, Pectus Deformity Repair (Pediatric).

• Refer to criteria under the Policy section in this medical policy for the following procedures:

o Reconstructive Treatment of Poland syndrome.

Medicaid – BeHealthy:

• There are no Masshealth guidelines for treatment of Pectus Cavinatum or Pectus Excavatum. HNE has adopted InterQual* criteria for the following procedures:

o Abramson Repair, Minimally Invasive Repair of Pectus Carinatum, Minimally Invasive Repair of Pectus Excavatum, Nuss Procedure, Open Repair of Pectus Carinatum, Open Repair of Pectus Excavatum, Ravitch Procedure: CP:Procedures, Pectus Deformity Repair (Pediatric).

• Refer to criteria under the Policy section in this medical policy for the following procedures:

o Reconstructive Treatment of Poland syndrome.

Medicare:
• Medicare does not have a National Coverage Determination (NCD) or a Local Coverage Determination (LCD) for the treatment of Pectus Cavinatum or Pectus Excavatum. HNE has adopted InterQual* criteria for the following procedures:

o Abramson Repair, Minimally Invasive Repair of Pectus Carinatum, Minimally Invasive Repair of Pectus Excavatum, Nuss Procedure, Open Repair of Pectus Carinatum, Open Repair of Pectus Excavatum, Ravitch Procedure: CP:Procedures, Pectus Deformity Repair (Pediatric).

• Refer to criteria under the Policy section in this medical policy for the following procedures:

o Reconstructive Treatment of Poland syndrome.

*To obtain InterQual® SmartSheets™: If you are a registered Health New England provider click here: https://www.hnedirect.com/login/ to access the Provider website. If you do not have access to the portal call (413) 787-4004 to obtain a copy.

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Policy

I. Abramson Repair, Minimally Invasive Repair of Pectus Carinatum, Minimally Invasive Repair of Pectus Excavatum, Nuss Procedure, Open Repair of Pectus Carinatum, Open Repair of Pectus Excavatum, Ravitch Procedure

A. Health New England has adopted the following InterQual criteria.

o CP:Procedures, Pectus Deformity Repair (Pediatric).

B. For members who are 18 years of age or older, the request will be reviewed on a case-by-case basis.

C. Abramson Repair, Minimally Invasive Repair of Pectus Carinatum, Minimally Invasive Repair of Pectus Excavatum, Nuss Procedure, Open Repair of Pectus Carinatum, Open Repair of Pectus Excavatum, Ravitch Procedure for all other indications other than in criteria above are considered NOT MEDICALLY NECESSARY.

II. Reconstructive Treatment of Poland syndrome

A. Reconstructive Treatment of Poland syndrome is considered MEDICALLY NECESSARY irrespective of member’s age when criteria 1 and 2 are met:

1. When there is functional impairment, documented by one of the following:

   a) Decreased cardiac output and/or abnormal pulmonary function during exercise; or b) Anticipation of future cardiovascular compromise; or c) 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; or d) Arrhythmias or clinical stigmata of decreased cardiac output.

   AND

2. The procedure is expected to correct the functional impairment and rib formation is absent.

   B. Reconstructive repair of Poland Syndrome is considered NOT MEDICALLY NECESSARY when above criteria are not met.

   Policy Guidelines and Definitions

   Definitions:

   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

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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. This condition is not usually diagnosed until the rapid growth that occurs during puberty. PC is characterized by a protrusion of the sternum that occurs as a result of an abnormal and unequal growth of the costal cartilage connecting the ribs to the sternum. Rather than growing flat along the chest wall, the costal cartilages grow outward pushing the sternum forward. There can be asymmetry of the chest with one side more prominent than the other. 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.

defects, developmental abnormalities, trauma, infection, tumors or disease. The purpose of reconstructive surgery is to correct abnormal structures of the body to improve function or create a normal appearance to the extent possible.

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 Reconstructive surgery is covered to correct or repair abnormal structures of the body caused by congenital 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.

Coding Guidance

Code
Description
PA
21740 Reconstructive repair of pectus excavatum or carinatum; open Yes 21742 Minimally invasive approach (Nuss procedure), without thoracoscopy Yes 21743 Minimally invasive approach (Nuss procedure), with thoracoscopy Yes

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Note: CPT/HCPCS codes are included for informational purposes and may not be all inclusive. Inclusion or exclusion of a CPT/HCPCS code(s) does not signify or imply that the service described by the code is a covered or non-covered health service. Benefit coverage for health services is determined by the member’s specific benefit plan document and applicable laws that may require coverage for a specific service. The inclusion of a code does not imply any right to reimbursement or guarantee of payment. Other policies and coverage determination guidelines may apply.

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References

Baban A, Torre M, Bianca S, et al. Poland syndrome with bilateral features: Case description with review of the literature. Am J Med Genet A. 2009;149A(7):1597-1602

Freitas Rda S, Tolazzi AR, Martins VD, et al. Poland's syndrome: Different clinical presentations and surgical reconstructions in 18 cases. Aesthetic Plast Surg. 2007;31(2):140-146.

Martinazzoli A, Cangemi V, Baccarini AE, et al. Poland syndrome. Problems of reconstructive and aesthetic surgery -- a clinical case. G Chir. 1995;16(11-12):497-501.

Moir, Christopher r, et al. Polands’s syndrome. Seminars in Pediatric Surgery. 2008 Aug; 17(3): 161-166

Notrica, D, McMahon, L, Jaroszewski, D. Pectus Disorders: Excavatum, Carinatum and Arcuatum. Adv Pediatr.. 2024 Aug;71(1):181-194

Nuchtern, Jed G, et al. Pectus carinatum and arcuatum, last updated 9/14/2023. UpToDate, Waltham, MA.

Policy Implementation

Approved by the Medical and Pharmacy Policy Committee

Kate McIntosh MD MBA

Chief Medical Officer

Saad Usmani MD MBA

Medical Director

Date Update 5/2020 Initial Policy Date 03/2022 No policy changes 04/2023 No policy changes 1/2024 Added Line of Business Section, updated references 07/2024 Minor Criteria changes. 5/2025 No policy changes

Medical Criteria Disclaimer

Property of Health New England. All rights reserved. The treating physician or primary care provider must submit to Health New England the clinical evidence that the patient meets the criteria for the treatment, testing or surgical procedure. Without this documentation and information, Health New England will not be able to properly review the request for prior authorization. The clinical review criteria reflect how Health New England determines whether certain services or supplies are medically necessary. Health New England established the clinical review criteria based upon a review of currently available clinical information (including clinical outcome studies in the peer-reviewed published medical literature, regulatory status of the technology, evidence-based guidelines of public health and health research agencies, evidence-based guidelines and positions of leading national health professional organizations, views of physicians practicing in relevant clinical areas, and other relevant factors). Health New England expressly reserves the right to revise these conclusions as clinical

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information changes and welcomes further relevant information. Each benefit program defines which services are covered. The conclusion that a particular service or supply is medically necessary does not constitute a representation or warranty that this service or supply is covered and/or paid for by Health New England, as some programs exclude coverage for services or supplies that Health New England considers medically necessary. If there is a discrepancy between this guideline and a member's benefits program, the benefits program will govern. In addition, coverage may be mandated by applicable legal requirements of a state, the Federal Government or the Centers for Medicare & Medicaid Services (CMS) for Medicare and Medicaid members. All coding and web site links are accurate at time of publication. Health New England has adopted the herein policy in providing management, administrative and other services to its Health Plan.