Pectus Excavatum and Poland's Syndrome: Surgical Correction

Number: 0272

Table Of Contents

Policy
Applicable CPT / HCPCS / ICD-10 Codes
Background
References

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Policy

Scope of Policy

This Clinical Policy Bulletin addresses pectus excavatum and poland's syndrome: surgical correction.

1. Medical Necessity

   Aetna considers the following surgical procedures medically necessary when criteria met:

   1. Surgical repair of severe pectus excavatum deformities that cause functional deficit 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:
         1. Cardiac compression, displacement results in decreased cardiac output demonstrated by echocardiography; or
         2. 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 
         3. 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;
   2. 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 (also see CPB 0185 - Breast Reconstructive Surgery).

2. Experimental and Investigational

   The following interventions for treatment of pectus excavatum are considered experimental and investigational because their effectiveness has not been established:

   1. The magnetic mini-mover procedure
   2. The vacuum bell
   3. Dynamic Compression System.

3. Cosmetic 

   The following procedures are considered cosmetic:

   1. Surgical repair of pectus excavatum cosmetic when criteria are not met in Section I;
   2. Bracing and surgical procedures to correct pectus carinatum because this deformity does not cause physiologic disturbances from compression of the heart or lungs.

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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]

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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.

Combined Bentall and Modified Ravitch Surgery for Marfan Syndrome Persons with Concomitant Pectus Excavatum and Ascending Aortic Aneurysm

Raffa et al (2017) noted that the optimal surgical management of the aortic root phenotype Marfan patients with severe PE is a subject of debate.  All the available literature were reviewed according to preferred reporting items for systematic reviews and meta-analyses (PRISMA) statement to examine the early outcomes of both PE and aortic repair techniques.  These investigators carried out searches in PubMed and Medline electronic databases dating from July 1953 to December 2015.  A total of 97 peer-reviewed publications were retrieved, and 27 relevant publications were identified with a total of 39 Marfan patients with PE who underwent ascending aorta and aortic root surgery.  Emergency acute type-A aortic dissection repair was reported in 5 cases.  Concomitant PE and aortic root repair and composite graft implantation were the most commonly performed procedures.  Complications after a staged or a combined approach were uncommon; and no deaths occurred.  The authors concluded that aortic surgery in Marfan patients with PE was performed according to a variety of strategies, surgical techniques and accesses with low complications rate and no mortality.  Many of these were well-tolerated with minimal complications and no mortality.

Waghefi et al (2022) stated that Marfan syndrome (MS) is a genetic disorder with autosomal dominant inheritance that affects the connective tissue and consequently many organ systems.  The cardiovascular manifestations of MS are notorious and include aortic root dilatation or acute aortic dissection, which could cause morbidity and early mortality.  However, surgical treatment of aortic pathology may be complicated by musculoskeletal deformity of the chest wall, as in PE.  In this regard, 1-stage combined Bentall and Ravitch surgery is an extreme rarity that has also been scarcely reported in the literature.  These investigators presented the medical history and 1-stage Bentall and modified Ravitch surgical treatment of an 18-year-old male MS patient with symptomatic and severe PE in conjunction with a pear-shaped aortic root aneurysm.  To discuss this case in the context of a synopsis of similar published cases, these researchers presented a systematic review of combined Bentall surgical aortic aneurysm repair and Ravitch correction of PE.  A total of 4 studies (1 case series and 3 case reports) and 1 case from the authors’ institution describing a 1-stage combined Bentall and Ravitch operation were included.  Patients were 22 ± 5.9 years of age (median of 22.5 years) and predominantly male (60 %).  All cases reported a mid-line vertical skin incision over the sternum.  The most common surgical approach was mid-sternotomy (80 %).  In all cases, metal struts were used to reinforce the corrected chest wall; and post-operative mortality was 0.  The authors concluded that 1-stage combined Bentall and Ravitch surgery was an under-utilized surgical approach.  Its use in MS patients with concomitant PE and ascending aortic aneurysm that require surgical treatment warrants further investigation.  Mid-sternotomy appeared to be a viable access route that provided sufficient exposure in the single-stage surgical setting.  Although operative time is long, the intra-operative and post-operative risks appeared to be low and manageable.

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.

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References