Akash Gupta, R1

 16 y/o male seen in clinic with Dr. Meehan  Obvious deformity of R Chest Wall – cosmesis worsening over 2 years  Activities: Soccer (3 months), Marching Band (drums)  Haller Index 3.1 (normal 2.5)  With absence of pulmonary symptoms and Haller Index <3.25, are there any physiologic consequences to look for requiring operative intervention?  How/when to treat is controversial  No consensus as to whether symptoms justify surgery

 Deformity of chest wall  Sternal depression  Midpoint of manubrium to xyphoid  90% of all anterior chest wall abnormalities  1 in live births  3-5 times more common in males

 Disproportionate muscular forces on costal cartilage and sternum  Defective cartilage structure and growth  Associations with connective tissue disorders suggests abnormal cartilage development  Marfan’s, Ehlers-Danlos, Osteogenesis imperfecta  Genetic imbalance of cartilage promotion and inhibition  Noonan’s syndrome, Turner’s syndrome, MEN 2b  Association with scoliosis

 2 or more of the following:  Cardiopulmonary symptoms  Progression of deformity  Paradoxical chest wall motion with deep inspiration  Pectus index of >3.25 (measured on CT scan)  Cardiac compression, displacement, or pulmonary compression on echocardiogram or CT  Abnormal pulmonary function testing showing significant restrictive disease  Abnormal exercise testing  Mitral valve prolapse  Bundle branch block or other cardiac pathology  Failed prior repair  Significant patient concern about appearance

 Previous studies reported decreased lung volumes  Recent studies suggest lung volumes are within range of normal  Type of lung disease differs between age groups

 Body plethysmography for measurement volume loss  Spirometry  Before and after administration of bronchodilator

 Decreased AP diameter  inability to fully expand  Cannot meet increases in metabolic demand  Displacement of heart  Turbulent blood flow  Rotation of great vessels

 EKG  R axis deviation  ST segment depression  Echocardiogram  Evaluate for mitral valve prolapse  Compression of R ventricle  Compression of pulmonary artery  Decreased stroke volume and cardiac output  Cardiopulmonary exercise testing  Helps evaluate limitations  Distinguishes pulmonary versus cardiac dysfunction

 Effects on spine  Asymmetric depression of sternum  abnormal pressure on spine  displacement of vertebral bodies  Effect on respiratory muscles  No direct effect  Mechanical disadvantage of intercostals  Quantify with Maximal inspiratory pressure (MIP) and Maximal expiratory pressure (MEP)

 Haller Index> 3.25 is usual indication for surgery  Lawson et al  Spirometry data in 310 patients and lung volumes in 218 patients age 6-21  Increasing Haller Index leads to abnormal FVC, FEV1 and TLC  Haller index of 7: 4 times more likely to demonstrate restrictive pattern than Haller index of 4  Does not evaluate functional impairment  Of limited diagnostic value  Mainly for pre-operative planning

 Malek et al, 2006 – Meta-analysis identified 8 studies, 169 patients  Evaluated CV parameters: CO, EF, EDV, HR, VO2 Max, SV  Used Effective Size statistic for each dependent variable in each study  Standardized measure of a study finding across studies providing information on magnitude and direction of intervention  Combined all CV parameters into single index for each study then evaluated mean gain from pre-op to post-op  Combined ES for each study represents the improvement (by standard devation) seen by operative intervention

 ES of 0.59 across all studies  Patients improved their CV parameters by 0.59 standard deviations (p = )  ES of 0.5 when severe (outlier) cases removed  Patients who did not have severe pectus excavatum improved their CV parameters by 0.5 standard deviations (p < 0.001)

 Prior studies have been inconclusive or too small to demonstrate CV improvement  Surgery mostly has been thought to be for improvement of cosmesis or psychological /psychosocial factors  Inconsistent findings and high cost of surgery (>$30000)  insurance companies reluctant to authorize procedure  First definitive study showing statistically and clinically significant improvement in CV parameters

 Kim et al. looked at various parameters on CT  Cardiac compression index: tranverse/AP diameter of heart  Cardiac asymmetry index: paramedian/AP diameter of heart  Chest wall compression index: transverse/middle diameter of chest  Chest wall asymmetry index: Left AP/Right AP diameter of chest

 CCI and CAI most sensitive and specific  Improvement in indices after operative correction  No correlation to physiologic improvement yet

 Physiologic consequences to pectus excavatum  Exercise intolerance due to restrictive lung disease, air trapping, and cardiovascular compression  Surgical correction demonstrates improved cardiovascular parameters  Preoperative work up of cardiopulmonary dysfunction important to predict benefit of treatment