Pectus Excavatum: Preoperative considerations Kathleen Berfield, MD University of Washington February 17, 2011

Goals Epidemiology Natural History Etiology Diagnosis/Clinical features Presentation Pre operative evaluation Indications for repair

Epidemiology Pectus Excavatum accounts for 90% of all anterior chest wall deformities Up to 5 times more prevalent in males than females Present in between 1 in 400 and 1 in 1,000 live births Up to 45% of patients report a family history of Pectus Excavatum 4% have ho Pectus carinatum Kelly RE, Croitoru DP, et al. A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 1998;33:545-52. Aut dom, auto recess, x linked and multifactorial Creswick HA, Stacey MW, Kelly RE Jr, et al. Family study of the inheritance of pectus excavatum. J Pediatr Surg 2006;41:1699-703.

Natural History 1/3 of cases present in infancy 1/3 of patients >12yo, deformity will worsen Asymmetry can develop with progression of PE 15-21% of patients will also have scoliosis 2% associated with congenital heart disease - Scoliosis and hunched shoulders due to uneven distribution of pressure of the ribs against the spine over time and the asymmetry of the sternum discplacement tends to be to the Right, - shoulders slouch to side of sternal rotation --E.W. Fonkalsrud, Current management of pectus excavatum, World J Surg 27 (2003), pp. 502–508

Etiology Histologically the costochondral cartilage is normal Associated congenital syndromes Poland Marfan’s Ehler’s Danlos Spinal Muscle Atrophy type 1 Mechanical forces on the sternum Acquired sternal depression case reports of Pectus Excavatum after CHD repair Histologically the costochondral cartilage is normal Otters- d/t breaking shells perhaps - animal models D/t impairments of cartilage development/ maturation Sternebrae- lowest two fuse after puberty Upper three fuse after age 25 -bone deposition from 8m -4 years, pattern of deposition depends on shape of sternum and the forces exterted on the vertebra Kelly, Robert E. Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Seminars in Pediatric Surgery

Diagnosis/Clinical features Morphology “Cup” shaped Generally located centrally at xiphoid “Saucer” shaped Tendency to be more shallow “Canal” shaped Grossly asymmetric, rotation of sternum toward defect Mixed Excavatum and Carinatum Cup:- costal cartilages 4-7 Canal- deformity of costal cartilages themselves- causes the sternum to rotate in towar the defect more common in femiales over 2:1 Park, Hyung Joo, Lee, In Sung, Kim, Kwang Taik Extreme eccentric canal type pectus excavatum: morphological study and repair techniques Eur J Cardiothorac Surg 2008 34: 150-154

Presentation Symptoms Additional Physical exam findings Dyspnea with exertion, loss of endurance Chest pain with activity Palpitations, tachycardia Progressive fatigue Psychosocial manifestations Additional Physical exam findings Split S2, systolic murmur, mid systolic click Scoliosis, Thoracic kyphosis Sxs may worsen with time Cause of dyspnea reduced diastolic filling of right heart, reduced SVO2 A negative P wave in lead V1, a negative T wave in V1 to V2 or V4, and incomplete right bundle branch block are the characteristic electrocardiographic (ECG) fi ndings1,4; they are the result of changes in the relation between the heart and the electrodes.compliance may help the improvements. The subjective physical improvements after operation are sometimes vigorous and dramatic and not entirely explainable by changes in objective cardiac and respiratory 8,18ﾐ20

Preoperative evaluation Radiographic CXR CT Physiologic PFTs ECHO Cardiopulmonary exercise testing Some feel that in sever cases a CXR is all that is needed. -- but CT preferred because it allows for evaluation of symmetry of the chest better than CXR CT- allows better eval of anatomy/ compression of the heart - down falls- radiation, static exam- Use of haller index but dimensions vary with inspiration and expiration which can cause wide variation in data. Haller index > 3.25 is considered severe and would be an indication for surgery PFTs:- not indicated as pts usualy do not have any intrinsic lung disease, studies suggest that post operative resolution of sxs of DOE etc are improved d/t the cardiovascular improvements and oxygen delivery d/t better right heart filling rather than improvements in pulmonary function. Malek et al in 2006 meta analysis concluded that pre and post operative PFTs were not significantly improved Pulmonary function following surgical repair of pectus excavatum: a meta-analysis Malek M H, Berger D E, Marelich W D, Coburn J W, Beck T W, Housh T J Malek MH, Berger DE, Housh TJ. Cardiovascular function following surgical repair of pectus excavatum: a metaanalysis. Chest 2006;130: 506-16.

CXR PA Lateral Vertical orientation of anterior ribs Indistinct right heart border Lateral Depression of sternum

Computed Tomography Evaluation of anatomy Haller Index Symmetry Rotation Depth Length Haller Index >3.25 considered “severe” CXR aand CT correlation

PFTs Preoperatively patients noted to have decreased VC, FEV1 and FVC No consensus that PFTs should be included in routine preoperative evaluation FVC noted to decrease after Nuss bar placement- likely d/t restriction and decreased elasticity of chest wall - studies show that post operatively there is no change in PFTs after nuss bar removal

Echo RA/RV decreased diastolic filling noted preoperatively d/t compression of the right heart Higher incidence of MVP in Pectus Excavatum patients than general population No indication for routine use unless in Marfan’s patients (evaluation of Aortic arch) Mitral Valve Prolapse 17% in study at E Virginia MS, Children’s hospital 2008 paper by Kelly Up to 65% depending on study ~50% of MVP resolves post operatively RV filling also noted in improve intraoperatively Kreuger, T, et al. Cardiac Function Assessed by Transesophageal Echocardiography During Pectus Excavatum Repair 2010, Ann Thorac Surg;89:240-244

Cardiopulmonary Exercise Testing Many PE patients report decreased exercise tolerance More sensitive than spirometry in detection of abnormalities in exercise response Abnormal stress PFTs, in setting of normal baseline PFTS VO2 noted to be reduced in patients with severe Pectus excavatum Numerous studies to evaluate this, mixed reviews present study was that average cardiovascular function increased by greater than one half SD (standard deviation) following the surgical repair of pectus excavatum.ﾓ

Indications for repair Chest CT shows cardiac and pulmonary compression or both and a CT index of 3.25 or greater. Cardiology evaluation demonstrations cardiac compression, displacement, mitral valve prolapse, murmurs, or conduction abnormalities. Pulmonary function study shows severe restrictive lung disease Previous repair has failed Significant activity limitation Progression of deformity

References Creswick HA, Stacey MW, Kelly RE Jr, et al. Family study of the inheritance of pectus excavatum. J Pediatr Surg 2006;41:1699-703. Kelly, Robert E. Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Seminars in Pediatric Surgery 2008;17, 181-193 E.W. Fonkalsrud, Current management of pectus excavatum 2003; World J Surg 27, pp. 502–508 Park, Hyung Joo, Lee, In Sung, Kim, Kwang Taik. Extreme eccentric canal type pectus excavatum: morphological study and repair techniques. Eur J Cardiothorac Surg; 2008, 34: 150-154 Hebra, A. Minimall invasive repair of pectus excavatum 2009, Semin Thoracic Cardiovascular Surg 21: 76-84 Malek MH, Berger DE, Housh TJ. Cardiovascular function following surgical repair of pectus excavatum: a metaanalysis. Chest 2006;130: 506-16. Restrepo, CS, Martinez S, et al. Imaging Apperances of the Sternum and Sternoclavicular joints 2009. Radiographics, 29:839-859. Kreuger, T, et al. Cardiac Function Assessed by Transesophageal Echocardiography During Pectus Excavatum Repair 2010, Ann Thorac Surg;89:240-244

Post operative effects Improved exercise tolerance Improved body image Decline in