Pectus Excavatum and Poland's Syndrome: Surgical Correction

Number: 0272

Policy

  1. Aetna considers surgical repair of severe pectus excavatum deformities that cause functional deficit medically necessary when done for medical reasons in members who meet all of the following criteria:

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

      • Cardiac compression, displacement results in decreased cardiac output demonstrated by echocardiography; or
      • Reduced lung capacity as demonstrated by a total lung capacity (TLC) less than or equal to 80% of predictive value per pulmonary function testing; or 
      • There is objective evidence of exercise intolerance due to reduced lung capacity as documented by exercise pulmonary function tests that are below the predicted values; and
    2. An electrocardiogram 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, derived from dividing the transverse diameter of the chest by the anterior-posterior diameter, greater than 3.25.

    Aetna considers surgical repair of pectus excavatum cosmetic when criteria are not met.

  2. Aetna considers surgical reconstruction of musculoskeletal chest wall deformities (congenital absence or hypoplasia of pectoralis major and minor muscles; congenital partial absence of the upper costal cartilage) associated with Poland's syndrome that cause functional impairment medically necessary (also see CPB 0185 - Breast Reconstructive Surgery).

  3. Aetna considers bracing and surgical procedures to correct pectus carinatum cosmetic because this deformity does not cause physiologic disturbances from compression of the heart or lungs.

  4. Aetna considers the following interventions for the treatment of pectus excavatum experimental and investigational because their effectiveness has not been established:

    • The magnetic mini-mover procedure
    • The vacuum bell
    • Dynamic Compression System.

Background

Chest wall deformities result from abnormal growth of the rib cartilages which pushes the sternum either inward or outward, away from the plane of the chest. The deformities can range from mild, symmetric indentions or protrusions, to severe asymmetric deformities. The appearance of the deformity often changes dramatically around the time of adolescent growth. Chest wall deformities may be corrected using various techniques; most require surgical intervention.

Pectus excavatum (PE) is often a cosmetic defect, but it may have varied anatomic and symptomatic presentations.  There is no conclusive evidence supporting the existence of a functional component whose physiological basis can be consistently defined. 

Pectus excavatum (PE) surgery techniques include, but may not be limited to:

  • Nuss procedure: Minimally invasive procedure in which an incision is made on each side of the chest wall. A concave bar is then inserted through one side of the chest under the sternum (breastbone) using a surgical clamp. Once the bar is pulled through, it is rotated, allowing the sternum to bend outward. Sutures are placed to temporarily attach the bar which eventually becomes held secure by muscle tissues growth occurring during recovery. The bar is left in place for several months or years.
  • Ravitch procedure: Named for the surgeon that developed it, this technique involves removing the ends of the ribs in the area that is depressed at the sternum. The sternum is then straightened out at the point it turns downward by breaking it horizontally. Stitches and a metal bar are used to hold the sternum in place under the skin. After two to three years, when remolding has taken place, the bar may be removed.

Until recently, the indications for surgery in patients with PE were based solely on clinical judgment because the extensive literature on PE demonstrates that there is a discordance between patients' subjective assessment of shortness of breath and objective measures of cardiorespiratory function.  In more recent years, the judgment of when to proceed with surgery has been made more objective by following the pectus index criteria advocated by Haller for surgical intervention.  Computed tomography (CT) scans used in patients being evaluated for surgery document more clearly the severity of the fore-shortening of the antero-posterior diameter of the chest, the degree of cardiac compression and displacement, the degree of lung compression and other unexpected problems.  It clarifies the need for operation by showing the dramatic internal morbidity of what is often portrayed as a "cosmetic" deformity.

The Haller index, also called the pectus index (PI) or pectus severity index (PSI), is the most commonly used scale for determining the severity of chest wall deformities. The index is defined as the width of the chest divided by the distance between the sternum and spine at the point of maximal depression. The normal value is 2.54. In individuals with pectus carinatum, a lower PSI indicates a more severe deformity in contrast to individuals with excavatum, in which a higher PSI indicates a more severe deformity. An index greater than 3.25 is considered severe for pectus excavatum. Computerized tomography (CT) or magnetic resonance imaging (MRI) may be used to determine the index.

As originally described by Sir Alfred Poland, Poland's syndrome consists of absence or hypoplasia of the pectoralis major and minor muscles, hypoplasia or absence of nipple and breast, hypoplasia of subcutaneous fat, absence of axillary hair, and partial absence of the upper costal cartilages and portions of ribs, usually the 2nd, 3rd, and 4th.  The absence of the sternal head of the pectoralis major muscle is considered the minimal expression of this syndrome (Wilhelmi and Cornette, 2002).  Brachysyndactyly, ectrodactyly, and ectromelia are frequently described associations.

Poland syndrome surgery techniques include, but may not be limited to: augmentation with tissue from the opposite breast, musculocutaneous flap to fill hollow space on the exterior of the chest, prosthetic augmentation, and surgical repair of the chest wall

In children with very severe deformity, staged procedures involving split rib grafts from the contralateral side combined with Teflon felt or Marlex mesh have been advocated.  This results in a stable chest wall, abolition of paradoxical movement, and protection of the subjacent viscera.  In the absence of the pectoralis major and with deficient breast and subcutaneous tissue, the chest is still visibly asymmetric.  As soon as the asymmetry becomes a problem for the adolescent female patient, a round tissue expander can be placed beneath the pectoralis muscle and hypoplastic breast through a transaxillary incision, to avoid scars on the breast itself.  The prosthesis is then inflated at appropriate intervals to maintain symmetry until development of the opposite breast stabilizes, at which time the expander can be replaced with a prosthetic mammary implant or an autologous soft-tissue transfer using pedicled myocutaneous flaps.

Schier et al (2005) described their experience in using a vacuum to pull the abnormal chest wall outward in patients with PE.  A suction cup was used to create a vacuum at the chest wall.  A patient-activated hand pump was used to reduce pressure up to 15 % below atmospheric pressure (atm).  The device was used by 60 patients (56 males and 4 females), aged 6.1 to 34.9 years (median of 14.8 years), for a minimum of 30 mins, twice-daily, up to 5 hours per day (median of 90 mins).  Patient progress was documented using photography, radiography, and plaster casts of the defect.  In 14 children this method was used during the Nuss procedure to enlarge the retrosternal space for safer passage of the introducer.  Follow-up occurred between 2 and 18 months (median of 10 months).  Computed tomographic scans showed that the device lifted the sternum and ribs within 1 to 2 mins; this was confirmed thoracoscopically during the Nuss procedure.  The suction cup enlarged the retrosternal space for safer passage of the introducer.  Initially, the sternum sank back after few minutes.  After 1 month, an elevation of 1 cm was noted in 85 % of the patients.  After 5 months, the sternum was lifted to a normal level in 12 patients (20 %) when evaluated immediately after using the suction cup.  All patients exhibited moderate subcutaneous hematoma, although the skin was not injured.  One patient suffered from transient paresthesis in the right arm and leg; 2 patients experienced orthostatic disturbances during the first application of the suction cup.  There were no other complications.  In patients with PE, application of a vacuum effectively pulled the depressed anterior chest wall forward.  The initial results proved dramatic, although it is not yet known how much time is required for long-term correction.  The authors concluded that this vacuum method holds promise as a valuable adjunct treatment in both surgical and non-surgical correction of PE.

Haecker and Mayr (2006) examined the benefits of conservative treatment of patients with PE by means of the vacuum bell.  A suction cup is used to create a vacuum at the anterior chest wall.  A patient-activated hand pump is used to reduce the pressure up to 15 % below atm.  Three different sizes of vacuum bell exist that were selected according to the individual patient’s age.  When creating the vacuum, the lift of the sternum was obvious and remained for a different time period.  The device should be used for a minimum of 30 mins (twice-daily), and may be used up to a maximum of several hours daily.  Presently, a 12- to 15-month course of treatment is recommended.  In addition, the device was used intra-operatively during the minimally invasive repair (MIRPE) procedure to enlarge the retrosternal space to ensure safer passage of the introducer in a few patients.  A total of 34 patients (31 males and 3 females), aged 6 to 52 years (median of 17.8 years) used the vacuum bell for 1 to maximum 18 months (median of 10.4 months).  Follow-up included photography and clinical examination every 3 months.  Computed tomographic scans showed that the device lifted the sternum and ribs immediately.  In addition, this was confirmed thoracoscopically during the MIRPE procedure.  After 3 months, an elevation of more than 1.5 cm was documented in 27 patients (79 %).  After 12 months, the sternum was lifted to a normal level in 5 patients (14.7 %).  Relevant side effects were not noted.  The authors concluded that the vacuum bell has proved to be an alternative therapeutic option in selected patients with PE.  Moreover, they stated that while the initial results proved to be dramatic, long-term results are so far lacking, and further evaluation and follow-up studies are necessary.

Haecker (2011) provided additional data on the 2006 trial by Haecker and Mayr; but the conclusion remained unchanged.  A total of 133 patients (110 males and 23 females) aged from 3 to 61 years (median of 16.21 years) used the vacuum bell for 1 to a maximum of 36 months.  Computed tomographic scans showed that the device lifted the sternum and ribs immediately.  In addition, this was confirmed thoracoscopically during the MIRPE procedure.  A total of 105 patients showed a permanent lift of the sternum for more than 1 cm after 3 months of daily application; 13 patients stopped the application and underwent MIRPE.  Relevant side effects were not noted.  The authors concluded that the vacuum bell has proved to be an alternative therapeutic option in selected patients suffering from PE.  The initial results proved to be dramatic, but long-term results are so far lacking, and further evaluation and follow-up studies are necessary.

Harrison et al (2007) noted that correction of PE results in measurable improvement in lung capacity and cardiac performance as well as improved appearance and self-image.  The Nuss and modified Ravitch approaches attempt to correct the chest wall deformity by forcing the sternum forward in 1-step and holding it in place using a metal strut.  The initial operation requires extensive manipulation under general anesthesia and results in post-operative pain, requiring hospitalization and regional anesthesia.  Pain and disability may last for weeks.  Both procedures are expensive.  A better principle would be a gradual bit-by-bit repair via small increments of pressure applied over many months.  These researchers developed the magnetic mini-mover procedure (3MP) and applied this strategy to correct PE.  The procedure uses magnetic force to pull the sternum forward.  An internal magnet implanted on the sternum and an external magnet in a non-obtrusive custom-fitted anterior chest wall orthosis produce an adjustable outward force on the sternum.  Outward force is maintained until the abnormal costal cartilages are remodeled and the pectus deformity is corrected.  These investigators implanted a magnet in human skeletons and measured the force applied to the sternum when the distance between the internal and external magnets was varied in increments.  With the 2 magnets 1 cm apart, the outward force was adequate to move the sternum at least 1 cm.  They also mapped the magnetic field in the 2-magnet configuration and found that maximum field strengths at the surface of the heart and at the outer surface of the orthosis were at safe levels.  The authors concluded that the 3MP allows correction of PE by applying magnetic force over a period of months.  Crucial questions raised during the design, re-design, and simulation testing have been satisfactorily answered, and the authors have received a Food and Drug Administration (FDA) Investigation Device Exemption (G050196/A002) to proceed with a phase I to II clinical trial.

Harrison et al (2012) performed a pilot study of safety, probable efficacy, and cost-effectiveness of 3MP.  A total of 10 otherwise healthy patients, aged 8 to 14 years, with severe pectus excavatum (pectus severity index [PSI] greater than 3.5) underwent 3MP treatment (mean of 18.8 +/- 2.5 months).  Safety was assessed by post-implant and post-explant electrocardiograms and monthly chest x-rays.  Efficacy was assessed by change in pectus severity index as measured using pre-treatment and post-treatment computed tomographic scan.  Cost of 3MP was compared with that of standard procedures.  The 3MP device had no detectable ill effect.  Device weld failure or mal-positioning required revision in 5 patients.  Average wear time was 16 hrs/day.  Pectus severity index improved in patients in the early or mid-puberty but not in patients with non-compliant chest walls.  Average cost for 3MP was $46,859, compared with $81,206 and $81,022 for Nuss and Ravitch, respectively.  The authors concluded that the 3MP is a safe, cost-effective, outpatient alternative treatment for pectus excavatum that achieves good results for patients in early and mid-puberty stages.

Ji and Luan (2012) reviewed the current development in therapy of congenital funnel chest.  The main therapies for congenital funnel chest are thoracoplasty (Ravitch sternum elevation procedure and minimal invasive Nuss procedure) and prosthesis implantation.  The magnetic mini-mover procedure and the vacuum bell are still in the research phase. 

An UpToDate review on “Pectus excavatum: Treatment” (Mayer, 2013) states that “Currently, surgical correction for PE is done with either the modified Ravitch procedure (open resection of the subperichondrial cartilage and sternal osteotomy, with placement of an internal stabilizing device), or the Nuss procedure (minimally invasive technique in which a curved bar is inserted to lift the sternum; the bar is removed about two years later)”.

Johnson et al (2014) compared outcome measures of current PE treatments, namely the Nuss and Ravitch procedures, in pediatric and adult patients.  Original investigations that stratified PE patients based on current treatment and age (pediatric = 0 to 21 years; adult 17 to 99 years) were considered for inclusion.  Outcome measures were: operation duration, analgesia duration, blood loss, length of stay (LOS), outcome ratings, complications, and percentage requiring reoperations.  Adult implant patients (18.8 %) had higher re-operation rates than adult Nuss or Ravitch patients (5.3 % and 3.3 %, respectively).  Adult Nuss patients had longer LOS (7.3 days), more strut/bar displacement (6.1 %), and more epidural analgesia (3 days) than adult Ravitch patients (2.9 days, 0 %, 0 days).  Excluding pectus bar and strut displacements, pediatric and adult Nuss patients tended to have higher complication rates (pediatric – 38 %; adult – 21 %) compared to pediatric and adult Ravitch patients (12.5 %; 8 %).  Pediatric Ravitch patients clearly had more strut displacements than adult Ravitch patients (0 % and 6.4 %, respectively).  These results suggested significantly better results in common PE surgical repair techniques (i.e., Nuss and Ravitch) than uncommon techniques (i.e., Implants and Robicsek).  The authors concluded that these results suggested slightly better outcomes in pediatric Nuss procedure patients as compared with all other groups.  They recommended that symptomatic pediatric patients with uncomplicated PE receive the Nuss procedure.  They suggested that adult patients receive the Nuss or Ravitch procedure, even though the long-term complication rates of the adult Nuss procedure require more investigation.

In a Cochrane review, de Oliveira Carvalho (2014) evaluated the safety and effectiveness of the conventional surgery compared with minimally invasive surgery for treating people with PE.  With the aim of increasing the sensitivity of the search strategy, these researchers used only terms related to the individual's condition (pectus excavatum); terms related to the interventions, outcomes and types of studies were not included.  They searched the Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, Embase, LILACS, and ICTPR.  Additionally they searched yet reference lists of articles and conference proceedings.  All searches were done without language restriction.  Date of the most recent searches was January 14, 2014.  These investigators considered randomized or quasi-randomized controlled trials that compared traditional surgery with minimally invasive surgery for treating PE.  Two review authors independently assessed the eligibility of the trials identified and agreed trial eligibility after a consensus meeting.  The authors also assessed the risk of bias of the eligible trials.  Initially the authors located 4,111 trials from the electronic searches and 2 further trials from other resources.  All trials were added into reference management software and the duplicates were excluded, leaving 2,517 studies.  The titles and abstracts of these 2,517 studies were independently analyzed by 2 authors and finally 8 trials were selected for full text analysis, after which they were all excluded, as they did not fulfil the inclusion criteria.  The authors concluded that there is no evidence from randomized controlled trials to conclude what is the best surgical option to treat people with PE.

Correction Index

St. Peter et al (2011) noted that the Haller Index (HI), the standard metric for the severity of PE, is dependent on width and does not assess the depth of the defect.  Thus, these researchers performed a diagnostic analysis to assess the ability of HI to separate patients with PE from healthy controls compared to a novel index.  After institutional review board (IRB) approval, CT scans were evaluated from patients who have undergone PE repair and controls.  The correction index (CI) used 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 2 was divided by the latter (×100) to give the percentage of chest depth the defect represents.  There were 220 controls and 252 patients with PE.  Mean HI was 2.35, and the mean CI was 0.92 for the controls.  The mean HI was 4.06, and the mean CI was 31.75 in the patients with PEs.  In the patients with PE, HI demonstrated a 47.8 % overlap with the controls, while there was no overlap for CI.  The authors concluded that the Haller index demonstrated 48 % overlap between normal patients and those with PE. However, the proposed correction index perfectly separated the normal and diseased populations.

Poston et al (2014) noted that the Haller index correlates well with the correction index in pectus patients with standard chest wall dimensions; but is quite discrepant in the non-standard chest.  These researchers recommended operative repair for pectus excavatum with a correction index of 28 % or more, because this value correlated with the long-accepted standard (Haller index greater than or equal to 3.25) and this index remains accurate even in non-standard chest morphologies.

van der Merwe and colleagues (2016) presented the 1st report on in-hospital and long-term outcomes of endoscopic port access atrio-ventricular valve surgery (EPAAVVS) in adult patients with uncorrected congenital chest wall deformities (CCWDs).  The surgical team performed EPAAVVS in 7 consecutive adult patients (mean age of 51.3 ± 16.4 years, 14.3 % women, 50 % older than 60 years, mean EuroSCORE II of 0.8 ± 0.1 %) with uncorrected CCWDs between November 1, 2009 and November 30,  2015.  The mean left ventricular ejection fraction (LVEF) was 66.0 ± 8.5 %.  Surgical indications included isolated or combined symptomatic mitral valve (MV) regurgitation (n = 7, 100 %), left ventricular outflow tract (LVOT) obstruction (n = 1, 14.3 %) and patent foramen ovale (PFO; n = 3, 42.9% ).  Fibro-elastic deficiency accounted for 57.1 % of MV pathology and 5 patients (74.1 %) presented with New York Heart Association (NYHA) Class III symptoms.  CCWDs included isolated pectus excavatum (n = 5, 71.4 %) and mixed pectus excavatum and carinatum (n = 2, 28.6 %). The mean Haller-index and correction index scores were 2.7 ± 0.5 and 21.4 ± 10.2 %, respectively.  Procedures performed included MV repair (n = 7, 100 %), tricuspid valve (TV) repair (n = 1, 14.3 %) and left ventricular septal myomectomy (n = 1, 14.3 %).  There were no sternotomy conversions or complications with chest wall entry or AV valve exposure.  The mean cardiopulmonary bypass and cross-clamp times were 162.1 ± 48.1 and 113.7 ± 33.5 mins, respectively.  No patient required mechanical ventilation or intensive care treatment longer than 24 hours.  There were no surgical revisions, in-hospital respiratory or chest wall morbidities.  The mean length of hospital stay was 7.4 ± 1.0 days.  A total of 208 patient-months (mean of 29.7 ± 26.5) were available for long-term clinical and echocardiographic (EKG) analysis.  There were no 30-day or long-term mortalities and no patient required re-intervention for residual AV valve pathology.  All patients were classified as NYHA I during recent consultations, and EKG follow-up confirmed no residual MV regurgitation greater than Grade 1 in any patient.  The authors concluded that EPAAVVS in adults with uncorrected CCWD was safe, feasible and durable and could successfully be performed by experienced teams to achieve Haller index and correction index scores of up to 3.3 and 38.3 %, respectively, with favorable long-term clinical and EKG outcomes.  The mere presence of uncorrected CCWDs should not deter surgeons from offering these patients the full benefits of minimally invasive cardiac surgery.

Dore et al (2018) stated that minimally invasive repair for pectus excavatum (MIRPE) is controversial in extremely severe cases of pectus excavatum (PE) and an open repair is usually favored.  These researchers described the case of a patient with an extremely severe PE who underwent a minimally invasive approach.  An 8-year old girl with severe sternum depression was assessed.  She had a history of exercise intolerance, nocturnal dyspnea, fatigue, and shortness of breath.  Chest computed tomography (CT) showed that sternum depression was posterior to the anterior vertebral column; thus, Haller and correction index could not be measured.  Spirometry indicated an obstructive ventilation pattern (forced expiratory volume in 1 second [FEV1] = 74.4 %), and EKG revealed a dilated inferior vena cava, MV prolapse with normal ventricular function.  After multi-disciplinary committee evaluation, a MIRPE approach was performed.  All symptoms had disappeared at the 3-month post-operative follow-up; the desired sternum shape was achieved, and normalization of cardio-pulmonary function was observed.  The Nuss bars were removed after a 2-year period.  After 18-month follow-up, the patient could carry out normal exercise and was satisfied with the cosmetic result.  The authors concluded that the Nuss procedure was feasible in this 8-year old patient.  In this case, both the Haller and correction index were not useful to assess the severity of PE.  Thus, under these circumstances, other radiologic parameters have to be taken into consideration for patient evaluation.

Gomez et al (2019) stated that the Haller index (HI) is widely used to indicate surgical intervention in patients with PE.  However, in patients with an atypical thoracic morphology, the severity of the defect can be incorrectly estimated.  These researchers proposed comparing this index with the correction index (CI).  These researchers analyzed clinical data and CT scans of 50 patients who consulted for PE in the authors’ center between 2010 and 2017.  Haller index (HI), Correction index (CI) and ideal thoracic index (ITI) were calculated for each patient.  The ITI allowed dividing the sample into 2 groups based on the thoracic morphology by excluding the PE component, thus, separating those with thorax too wide or too narrow from the standard patients.  A standard group (36 patients) and a non-standard group (14 patients) were generated, among which the HI and the CI were correlated.  The mean HI and CI of all patients were 3.99 and 27 %, respectively; 31 of the 50 patients (62 %) underwent intervention, 8 of them with an HI below 3.25.  When comparing both groups, there was a moderate correlation between HI and CI in the standard group (Spearman r 0.799, p < 0.01) and a greater correlation in the non-standard group (Spearman r 0.858, p < 0.01) between the scale and the presence of foreign body, except for SCORE 1, which was 57 % what these investigators attributed to an information bias.  If the foreign body were not nuts, inorganic or bone, its aspiration was very unlikely, that’s why these researchers included it in the SCORE with -1.  The authors concluded that in this cohort, correlation of HI and CI was not different between both groups of patients.  The CI did not prove its superiority when compared to HI in the surgical indication of patients with PE.

Intercostal Nerve Cryoablation for Post-Operative Analgesia following Pectus Excavatum Repair

Graves and colleagues (2019) noted that minimally-invasive repair of PE by the Nuss procedure is associated with significant post-operative pain, prolonged hospital stay, and high opiate requirement.  In a randomized clinical study, these researchers hypothesized that intercostal nerve cryoablation during the Nuss procedure reduces hospital length of stay (LOS) compared to thoracic epidural analgesia.  This trial examined 20 consecutive patients undergoing the Nuss procedure for PE between May 2016 and March 2018.  Patients were randomized evenly via closed-envelope method to receive either cryoanalgesia or thoracic epidural analgesia.  Patients and physicians were blinded to study arm until immediately pre-operatively.  A total of 20 consecutive patients were recruited from those scheduled for the Nuss procedure.  Exclusion criteria were age of less than 13 years, chest wall anomaly other than PE, previous repair or other thoracic surgery, and chronic use of pain medications.  Primary outcome was post-operative LOS; secondary outcomes included total operative time, total/daily opioid requirement, inpatient/outpatient pain score, and complications.  Primary outcome data were analyzed by the Mann-Whitney U-test for non-parametric continuous variables.  Other continuous variables were analyzed by 2-tail t-test, while categorical data were compared via Chi-squared test, with alpha = 0.05 for significance.  A total of 20 patients were randomized to receive either cryoablation (n = 10) or thoracic epidural (n = 10).  Mean operating room time was 46.5 mins longer in the cryoanalgesia group (p = 0.0001).  Median LOS decreased by 2 days in patients undergoing cryoablation, to 3 days from 5 days (Mann-Whitney U, p = 0.0001).  Cryoablation patients needed significantly less inpatient opioid analgesia with a mean decrease of 416 mg oral morphine equivalent per patient (p = 0.0001), requiring 52 % to 82 % fewer milligrams on post-operative days 1 to 3 (p < 0.01 each day).  There was no difference in mean pain score between the groups at any point post-operatively, up to 1 year, and no increased incidence of neuropathic pain in the cryoablation group.  No complications were noted in the cryoablation group; among patients with epidurals, 1 patient experienced a symptomatic pneumothorax, and another had urinary retention.  The authors concluded that intercostal nerve cryoablation during the Nuss procedure decreased hospital LOS and opiate requirement versus thoracic epidural analgesia, while offering equivalent pain control.

The authors stated that he main drawback of this study was the small sample size (n = 10 for each group).  Although these investigators were able to show significant differences in LOS and opioid requirement between the 2 groups, the groups were not exactly matched as evidenced by a statistically significant difference in age between the groups.  Of note, older patients were generally thought to have more rigid chest walls, which intuitively might contribute to greater post-operative pain and longer LOS; yet the cryoanalgesia group, with a mean older age, experienced neither, suggesting differences in age did not affect these findings.  Although the numbers in this study were too small for subgroup analysis, as these researchers expand their cohort, they hope to further define patient factors such as age, habitus, and Haller index that may predict increased benefit from cryoanalgesia.

Daemen and associates (2020) stated that intercostal nerve cryoablation has recently been proposed as an alternative method with long-acting pain control and shortened hospitalization.  These investigators examined the outcomes of intercostal nerve cryoablation in comparison to thoracic epidural following the Nuss procedure.  A total of 6 scientific databases were searched.  Data concerning the hospital LOS, operative time and post-operative opioid usage were extracted.  If possible, data were submitted to meta-analysis using the mean of differences, random-effects model with inverse variance method and I2 test for heterogeneity.  A total of 4 observational and 1 randomized study were included, enrolling a total of 196 patients.  Meta-analyses demonstrated a significantly shortened hospital LOS [mean difference -2.91 days; 95 % confidence interval (CI) -3.68 to -2.15; P < 0.001] and increased operative time (mean difference [MD] 40.91 min; 95 % confidence interval [CI]: 14.42 to 67.40; p < 0.001) for cryoablation.  Both analyses demonstrated significant heterogeneity (both I2 = 91 %; p < 0.001).  Qualitative analysis demonstrated the amount of post-operative opioid usage to be significantly lower for cryoablation in 3 out of 4 reporting studies.  The authors concluded that intercostal nerve cryoablation during the Nuss procedure may be an attractive alternative to thoracic epidural analgesia, resulting in shortened hospital LOS; however, given the low quality and heterogeneity of studies, more well-designed randomized controlled trials (RCTs) are needed.

The authors stated that the main drawbacks of this review included the low number of included studies and participants, the fact that only 1 randomized trial was included, the overall methodological quality that ranged from some concerns to serious risk of bias, the use of data conversion methods and the heterogeneity among included studies.  Furthermore, despite the funnel plot asymmetry and statistical analyses were not indicative for publication bias, its presence cannot be completely excluded while the tests used are known to be under-powered if less than 10 studies are included.

Archer and co-workers (2020) noted that surgery for PE is associated with significant post-operative pain.  These researchers examined the available evidence regarding post-operative pain control for pediatric patients undergoing minimally invasive repair of PE (MIRPE).  They carried out a systematic search of Medline, Embase, PubMed, CINAHL, Web of Science, and the Cochrane Library for RCTs comparing methods of pain control in pediatric patients undergoing MIRPE; studies were restricted to the English language.  After screening 1,304 references, 9 RCTs enrolling 485 patients were included.  The average age was 11.9 years (± 3.1).  Pain scores were decreased with ropivacaine compared to bupivacaine-based epidurals.  In studies comparing ketamine to opioid based patient-controlled anesthesia (PCA) pumps, the results were variable.  Intercostal and paravertebral nerve blocks had decreased pain scores in 75 % of the studies compared to opioid-based PCA.  Opioid consumption was decreased in 50 % of the trials evaluating ketamine-based infusions and 75 % of the studies comparing intercostal and paravertebral nerve blocks.  Nausea was decreased in several of the ketamine-based infusion and intercostal and paravertebral nerve block studies.  The authors concluded that ketamine-including infusions or paravertebral and intercostal nerve blocks may represent superior methods of post-operative pain control for MIRPE; however, further work is needed to confirm these findings.  Level of evidence = 2A.

Furthermore, an UpToDate review on “Pectus excavatum: Treatment” (Mayer, 2021) states that “A novel approach to pain management is cryoablation of the intercostal nerves in the region of the repair (termed cryoanalgesia), which prevents afferent conduction and therefore the perception of pain.  The nerves eventually heal, and sensation and efferent function is restored.  The most prominent advantage is a decrease in the postoperative length of stay and need for postoperative narcotics”.  However, cryoablation of the intercostal nerves is not listed in the “Summary and Recommendations” section of the review.

Pectus Carinatum

Pectus carinatum is a developmental deformity of the chest characterized by a protrusion of the sternum and ribs. It is extremely uncommon that pectus carinatum will cause a functional/physiological deficit. Pectus carinatum (PC) orthotic compression bracing uses a customized chest wall brace which applies direct, constant pressure to the protruding area of the chest with the goal of reshaping the chest and sternum. The brace has front and back compression pads that are attached to aluminum bars which are bound together by a tightening mechanism. Regular monitoring and adjustment is generally required. PC surgery includes removing the affected cartilages to mobilize both the pectoralis (chest muscles) flaps and mobilizing the skin to straighten the sternum. These surgical techniques include, but may not be limited to: costal cartilage subperichondral resection, osteotomy, and wedge shaped osteotomy in the anterior sternal plate.

An UpToDate review on “Pectus carinatum” (Nuchtern and Mayer, 2014) states that “In more than 90 percent of patients, pectus carinatum deformity is first noted during early adolescence, and it often worsens dramatically during the adolescent growth spurt.  The defect does not resolve spontaneously.  The vast majority of patients have no physiologic symptoms, and cosmetic appearance is the primary concern …. The decision of whether to treat depends on the severity of the defect, and the patient and family's level of concern”.

Ozkaya and Bilgin (2018) over the last 10 years, minimal invasive surgery for correction of pectus carinatum has gained worldwide acceptance.  These investigators reviewed their clinical experience with minimally invasive repair of pectus carinatum (MIRPC) since 2008.  Between 2008 and 2018, a total of 101 patients (77 male, 24 female) underwent correction of pectus carinatum with the MIRPC technique.  The mean age of the patients was 14.7 ± 4.8 (3 to 38) years.  Over their 8-year experience, these researchers slightly modified the original Abramson technique.  All patients presented with cosmetic complaints and all had a flexible chest wall on "compression test".  Early follow-up was on post-operative day 15 and 30.  The mean operative time was 42.1 ± 16.9 mins; and the mean hospital stay was 4.2 ± 0.9 days.  Post-operative complications included pneumothorax (n = 2, 1.9 %), wound infection (n = 2, 1.9 %), skin perforation (n = 2, 1.9 %), intolerable pain (n = 1, 0.9 %), skin hyper-pigmentation (n = 1, 0.9 %), and over-correction (n = 1, 0.9 %).  Initial post-operative results were excellent in all patients.  The bars were removed at a median of 24.8 ± 4.5 months in 44 of 101 patients; and 43 of 44 (97.7 %) patients whose bar were removed reported excellent results.  The authors concluded that MIRPC was a feasible procedure with low morbidity and excellent cosmetic results in the treatment of pectus carinatum deformities in selected patients.

Orrick and colleagues (2019) noted that osteogenesis imperfecta (OI) is a genetic disorder of collagen resulting in a "fragile" skeleton with increased fracture risk and other complications, dependent on the specific variant.  Pectus deformities of the chest wall, while not common, can be associated with OI.  The use of a pectus carinatum brace in a patient with OI poses unknown risks for fractures and adverse treatment outcomes.  These investigators successfully applied external compression bracing using the dynamic compression system to 1 such patient.  The authors concluded that this case illustrated the ability to treat an OI patient with pectus carinatum using a non-surgical brace, without complications, resulting in an excellent cosmetic result.

Table: CPT Codes / HCPCS Codes / ICD-10 Codes
Code Code Description

Information in the [brackets] below has been added for clarification purposes.   Codes requiring a 7th character are represented by "+":

Pectus excavatum:

CPT codes covered if selection criteria are met:
21740 Reconstructive repair of pectus excavatum or carinatum; open
21742      minimally invasive approach (Nuss procedure), without thoracoscopy
21743      minimally invasive approach (Nuss procedure), with thoracoscopy

Other experimental and investigational interventions:

Dynamic Compression System, Vacuum bell:
No specific code

ICD-10 codes covered if selection criteria are met:
J98.4 Other disorders of lung [Covered for compression of lung as demonstrated by a total lung capacity (TLC) less than or equal to 80% of predictive value per pulmonary function testing]
Q67.6 Pectus excavatum [that causes functional deficit]
R94.2 Abnormal results of pulmonary function studies [covered for exercise pulmonary function tests that are below the predicted values and show restrictive lung disease]

ICD-10 codes not covered for indications listed in the CPB:
Q67.7 Pectus carinatum

Poland's syndrome:

CPT codes covered if selection criteria are met:
11960 Insertion of tissue expander(s) for other than breast, including subsequent expansion
11970 Replacement of tissue expander with permanent prosthesis
11971 Removal of tissue expander(s) without insertion of prosthesis
19340 Immediate insertion of breast prosthesis following mastopexy, mastectomy or in reconstruction
19342 Delayed insertion of breast prosthesis following mastopexy, mastectomy or in reconstruction
19357 Breast reconstruction, immediate or delayed, with tissue expander, including subsequent expansion
19361 Breast reconstruction with latissimus dorsi flap, without prosthetic implant
19364 Breast reconstruction with free flap
19367 Breast reconstruction with transverse rectus abdominis myocutaneous flap (TRAM), single pedicle, including closure of donor site
19368      with microvascular anastomosis (supercharging)
19369 Breast reconstruction with transverse rectus abdominis myocutaneous flap (TRAM), double pedicle, including closure of donor site
20900 Bone graft, any donor area; minor or small (e.g., dowel or button)
20902      major or large

ICD-10 codes covered if selection criteria are met:
Q79.8 Other congenital malformations of musculoskeletal system [Poland's syndrome]

The above policy is based on the following references:

Pectus Excavatum

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  3. Cho DG, Kim JJ, Park JK, Moon SW. Recurrence of pectus excavatum following the Nuss procedure. J Thorac Dis. 2018;10(11):6201-6210.
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  8. de Oliveira Carvalho PE, da Silva MV, Rodrigues OR, Cataneo AJ. Surgical interventions for treating pectus excavatum. Cochrane Database Syst Rev. 2014;10:CD008889.
  9. Dore M, Junco PT, De La Torre C, et al. Nuss procedure for a patient with negative Haller index. European J Pediatr Surg Rep. 2018;6(1):e18-e22.
  10. Ellis DG, Snyder CL, Mann CM. The ‘re-do’ chest wall deformity correction. J Pediatr Surg. 1997;32(9):1267-1271. 
  11. Erdogan A, Ayten A, Oz N, Demircan A. Early and long-term results of surgical repair of pectus excavatum. Asian Cardiovasc Thorac Ann. 2002;10(1):39-42.
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  15. Fonkalsrud EW, Salman T, Guo W, et al. Repair of pectus deformities with sternal support. J Thorac Cardiovasc Surg. 1994;107:37-42. 
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  21. Haecker FM, Mayr J. The vacuum bell for treatment of pectus excavatum: An alternative to surgical correction? Eur J Cardiothorac Surg. 2006;29(4):557-561.
  22. Haecker FM. The vacuum bell for conservative treatment of pectus excavatum: The Basle experience. Pediatr Surg Int. 2011;27(6):623-627.
  23. Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: A preliminary report. J Pediatr Surg. 1987;22(10):904-906. 
  24. Haller JA Jr, Scherer LR, Turner CS, et al. Evolving management of pectus excavatum based on a single institutional experience of 664 patients. Ann Surg. 1989;209(5):578-582. 
  25. Harrison MR, Estefan-Ventura D, Fechter R, et al. Magnetic Mini-Mover Procedure for pectus excavatum: I. Development, design, and simulations for feasibility and safety. J Pediatr Surg. 2007;42(1):81-85; discussion 85-86.
  26. Harrison MR, Gonzales KD, Bratton BJ, et al. Magnetic mini-mover procedure for pectus excavatum III: Safety and efficacy in a Food and Drug Administration-sponsored clinical trial. J Pediatr Surg. 2012;47(1):154-159.
  27. Hebra A, Kelly RE, Ferro MM, et al. Life-threatening complications and mortality of minimally invasive pectus surgery. J Pediatr Surg. 2018;53(4):728-732.
  28. Ji K, Luan J. Current development in therapy of congenital funnel chest. Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2012;26(12):1516-1518.
  29. Johnson WR, Fedor D, Singhal S. Systematic review of surgical treatment techniques for adult and pediatric patients with pectus excavatum. J Cardiothorac Surg. 2014;9:25.
  30. Kaguraoka H, Ohnuki T, Itaoka T, et al. Degree of severity of pectus excavatum and pulmonary function in preoperative and postoperative periods. J Thorac Cardiovasc Surg. 1992;104:1483-1488. 
  31. Kelly RE Jr, Cash TF, Shamberger RC, et al. Surgical repair of pectus excavatum markedly improves body image and perceived ability for physical activity: Multicenter study. Pediatrics. 2008;122(6):1218-1222.
  32. Kelly RE Jr, Shamberger RC, Mellins RB, et al. Prospective multicenter study of surgical correction of pectus excavatum: Design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection. J Am Coll Surg. 2007;205(2):205-216.
  33. Kobayashi S, Yoza S, Komuro Y, et al. Correction of pectus excavatum and pectus carinatum assisted by the endoscope. Plast Reconstr Surg. 1997;99 (4):1037-1045. 
  34. Linton SC, Ghomrawi HMK, Tian Y, et al. Association of operative volume and odds of surgical complication for patients undergoing repair of pectus excavatum at children's hospitals. J Pediatr. 2021 Dec 27 [Online ahead of print].
  35. Malek MH, Berger DE, Housh TJ, et al. Cardiovascular function following surgical repair of pectus excavatum: A metaanalysis. Chest. 2006;130(2):506-516.
  36. Malek MH, Berger DE, Marelich WD, et al. Pulmonary function following surgical repair of pectus excavatum: A meta-analysis. Eur J Cardiothorac Surg. 2006;30(4):637-643.
  37. Mayer OH. Pectus excavatum: Treatment. UpToDate [online serial]. Waltham, MA: UpToDate; reviewed February 2013; January 2021. 
  38. Miller KA, Ostlie DJ, Wade K, et al. Minimally invasive bar repair for 'redo' correction of pectus excavatum. J Pediatr Surg. 2002;37(7):1090-1092. 
  39. Morshuis W, Folgering H, Barentsz J, et al. Pulmonary function before surgery for pectus excavatum and at long-term follow-up. Chest. 1994;105(6):1646-1652. 
  40. Morshuis WJ, Folgering HT, Barentsz JO, et al. Exercise cardiorespiratory function before and one year after operation for pectus excavatum. J Thorac Cardiovasc Surg, 1994;107:1403-1409. 
  41. Morshuis WJ, Mulder H, Wapperom G, et al. Pectus excavatum: A clinical study with long term postoperative follow up. Eur J Cardiothorac Surg. 1992;6(6):318-328; discussion 328-329. 
  42. Muhly WT, Beltran RJ, Bielsky A, et al. Perioperative management and in-hospital outcomes after minimally invasive repair of pectus excavatum: A multicenter registry report from the Society for Pediatric Anesthesia Improvement Network. Anesth Analg. 2019;128(2):315-327. 
  43. Nasr A, Fecteau A, Wales PW. Comparison of the Nuss and the Ravitch procedure for pectus excavatum repair: A meta-analysis. J Pediatr Surg. 2010;45(5):880-886.
  44. National Institute for Clinical Excellence (NICE). Minimally invasive placement of pectus bar. Interventional Procedure Guidance 3. London, UK: NICE; July 2003.
  45. Nuss D, Kelly RE Jr, Croitoru DP, et al. A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg. 1998;33(4):545-552. 
  46. Nuss D. Recent experiences with minimally invasive pectus excavatum repair 'Nuss procedure'. Jpn J Thorac Cardiovasc Surg. 2005;53(7):338-344.
  47. Oswald N, Jalal Z, Kadiri S, Naidu B. Changes in chest wall motion with removal of Nuss bar in repaired pectus excavatum - a cohort study. J Cardiothorac Surg. 2019;14(1):4.
  48. Poston PM, Patel SS, Rajput M, et al. The correction index: Setting the standard for recommending operative repair of pectus excavatum. Annal Thoracic Surg. 2014;97(4):1176-1180.
  49. Protopapas AD, Athanasiou T. Peri-operative data on the Nuss procedure in children with pectus excavatum: Independent survey of the first 20 years' data. J Cardiothorac Surg. 2008;3:40.
  50. Quigley PM, Haller JA Jr, Jelus KL, et al. Cardiorespiratory function before and after corrective surgery in pectus excavatum. J Pediatr. 1996;128(5 Pt 1):638-643. 
  51. Schalamon J, Pokall S, Windhaber J, Hoellwarth ME. Minimally invasive correction of pectus excavatum in adult patients. J Thorac Cardiovasc Surg. 2006;132(3):524-529.
  52. Schier F, Bahr M, Klobe E. The vacuum chest wall lifter: An innovative, nonsurgical addition to the management of pectus excavatum. J Pediatr Surg. 2005;40(3):496-500.
  53. Shamberger RC, Welch KJ. Cardiopulmonary function in pectus excavatum. Surg Gynecol Obstet. 1988;166:383-391. 
  54. Shamberger RC. Congenital chest wall deformities. Current problems in surgery. 1996;23:471-542. 
  55. St Peter SD, Juang D, Garey CL, et al. A novel measure for pectus excavatum: The correction index. J Pediatr Surg. 2011;46(12):2270-2273.
  56. Stavrev PV, Stavrev VP, Beshkov KN. Surgical correction of funnel chest. Folia Med (Plovdiv). 2000;42(2):57-60. 
  57. Swoveland B, Medvick C, Kirsh M, et al. The Nuss procedure for pectus excavatum correction. AORN J.  2001;74(6):828-841; quiz 842-845, 848-580. 
  58. Thaker S, McKenna E, Rader C, Misra MV. Pain management in pectus excavatum surgery: A comparison of subcutaneous catheters versus epidurals in a pediatric population. J Laparoendosc Adv Surg Tech A. 2019;29(2):261-266.
  59. van der Merwe J, Casselman F, Stockman B, et al. Endoscopic atrioventricular valve surgery in adults with difficult-to-access uncorrected congenital chest wall deformities. Interact Cardiovasc Thorac Surg. 2016;23(6):851-855.

Poland's Syndrome

  1. 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
  2. Borschel GH, Izenberg PH, Cederna PS. Endoscopically assisted reconstruction of male and female poland syndrome. Plast Reconstr Surg. 2002;109(5):1536-1543. 
  3. Fekih M, Mansouri-Hattab N, Bergaoui D, et al. Correction of breast Poland's anomalies. About eight cases and literature review. Ann Chir Plast Esthet. 2010;55(3):211-218.
  4. Fitjakowska M, Antoszewski B. Surgical treatment of patients with Poland's syndrome - Own experience. Pol Przegl Chir. 2011;83(12):662-667.
  5. 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.
  6. Gatti JE. Poland's deformity reconstructions with a customized, extrasoft silicone prosthesis. Ann Plast Surg. 1997;39(2):122-130. 
  7. Hamdi M, Blondeel P, Van Landuyt K, et al. Bilateral autogenous breast reconstruction using perforator free flaps: A single center's experience. Plast Reconstr Surg. 2004;114(1):83-89; discussion 90-92.
  8. Hodgkinson DJ. Re: Poland's deformity reconstruction with a customized extrasoft silicone prosthesis. Ann Plast Surg. 1998;40(2):194-195. 
  9. Hodgkinson DJ. The management of anterior chest wall deformity in patients presenting for breast augmentation. Plast Reconstr Surg. 2002;109(5):1714-1723. 
  10. Jasonni V, Lelli-Chiesa PL, Repetto P, et al. Congenital deformities of the chest wall. Surgical treatment. Minerva Pediatr. 1997;49(9):407-413. 
  11. Karnak I, Tanyel FC, Tuncbilek E, et al. Bilateral Poland anomaly. Am J Med Genet. 1998;75(5):505-507. 
  12. Longaker MT, Glat PM, Colen LB, et al. Reconstruction of breast asymmetry in Poland's chest-wall deformity using microvascular free flaps. Plast Reconstr Surg. 1997;99(2):429-436. 
  13. Lord MJ, Laurenzano KR, Hartmann RW Jr. Poland's syndrome. Clin Pediatr (Phila). 1990;29(10):606-609. 
  14. Marks MW, Iacobucci J. Reconstruction of congenital chest wall deformities using solid silicone onlay prostheses. Chest Surg Clin N Am. 2000;10(2):341-355. 
  15. 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. 
  16. Mestak J, Zadorozna M, Cakrtova M. Breast reconstruction in women with Poland's syndrome. Acta Chir Plast. 1991;33(3):137-144. 
  17. Pileggi AJ. Poland's syndrome. Clin Pediatr (Phila). 1991;30(2):125. 
  18. Wilhelmi BJ, Cornette PB. Breast, Poland syndrome. eMedicine Plastic Surgery Topic 132. Omaha, NE: eMedicine.com; updated August 5, 2002. 

Pectus Carinatum

  1. Banever GT, Konefal SH, Gettens K, Moriarty KP. Nonoperative correction of pectus carinatum with orthotic bracing. J Laparoendosc Adv Surg Tech A. 2006;16(2):164-167.
  2. Coelho Mde S, Guimarães Pde S. Pectus carinatum. J Bras Pneumol. 2007 Aug;33(4):463-74.
  3. Coskun ZK, Turgut HB, Demirsoy S, Cansu A. The prevalence and effects of pectus excavatum and pectus carinatum on the respiratory function in children between 7-14 years old. Indian J Pediatr. 2010;77(9):1017-1019.
  4. Egan JC, DuBois JJ, Morphy M, et al. Compressive orthotics in the treatment of asymmetric pectus carinatum: A preliminary report with an objective radiographic marker. J Pediatr Surg. 2000;35(8):1183-1186.
  5. Ellis DG. Chest wall deformities. Pediatr Rev. 1989;11(5):147-151. 
  6. Fonkalsrud EW, Beanes S. Surgical management of pectus carinatum: 30 years' experience. World J Surg. 2001;25(7):898-903.
  7. Goretsky M, Kelly R, Croitoru D, Nuss D. Chest wall anomalies: Pectus excavatum and pectus carinatum. Adolescent Med Clinic. 2004;15(3):455-471.
  8. Haje SA. Pectus carinatum successfully treated with bracing -- a case report. Int Orthop. 1995;19(5):332-333. 
  9. Knudsen MV, Pilegaard HK, Grosen K. Pain and sensory disturbances following surgical repair of pectus carinatum. J Pediatr Surg. 2018;53(4):733-739.
  10. Kobayashi S, Yoza S, Komuro Y, et al. Correction of pectus excavatum and pectus carinatum assisted by the endoscope. Plast Reconstr Surg. 1997;99(4):1037-1045. 
  11. Kravarusic D, Dicken BJ, Dewar R, et al. The Calgary protocol for bracing of pectus carinatum: A preliminary report. J Pediatr Surg. 2006;41(5):923-926.
  12. Lee SY, Lee SJ, Jeon CW, Lee CS, Lee KR.Effect of the compressive brace in pectus carinatum. Eur J Cardiothorac Surg. 2008;34(1):146-149.
  13. Mavanur A, Hight DW. Pectus excavatum and carinatum: New concepts in the correction of congenital chest wall deformities in the pediatric age group. Conn Med. 2008;72(1):5-11.
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  16. Orrick BA, Pierce AL, Snyder CL, Alon US. Successful brace treatment of pectus carinatum in osteogenesis imperfecta using the dynamic compression system. European J Pediatr Surg Rep. 2019;7(1):e117-e120.
  17. Ozkaya M, Bilgin M. Minimally invasive repair of pectus carinatum: A retrospective analysis based on a single surgeon's 10 years of experience. Gen Thorac Cardiovasc Surg. 2018;66(11):653-657. 
  18. Robicsek F, Watts LT, Fokin AA. Surgical repair of pectus excavatum and carinatum. Semin Thorac Cardiovasc Surg. 2009;21(1):64-75.
  19. Shamberger RC, Welch KJ. Surgical correction of pectus carinatum. J Pediatr Surg. 1987;22(1):48-53. 
  20. Sigl S, Del Frari B, Harasser C, Schwabegger AH. The effect on cardiopulmonary function after thoracoplasty in pectus carinatum: A systematic literature review. Interact Cardiovasc Thorac Surg. 2018;26(3):474-479.
  21. Snajdauf J, Sintakova B, Fryc R, et al. Surgical treatment of pectus excavatum and pectus carinatum. Cesk Pediatr. 1993;48(10):581-585. 
  22. Stephenson JT, Du Bois J. Compressive orthotic bracing in the treatment of pectus carinatum: The use of radiographic markers to predict success. J Pediatr Surg. 2008;43(10):1776-1780.