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

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

3. 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:
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:

4. 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

5. 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

6. 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 :

7. 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

8. 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:

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

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

11. 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

12. 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:

13. 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

14. 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

15. References
Creswick HA, Stacey MW, Kelly RE Jr, et al. Family study of the inheritance of pectus excavatum. J Pediatr Surg 2006;41:
Kelly, Robert E. Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Seminars in Pediatric Surgery 2008;17,
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:
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:
Restrepo, CS, Martinez S, et al. Imaging Apperances of the Sternum and Sternoclavicular joints Radiographics, 29:
Kreuger, T, et al. Cardiac Function Assessed by Transesophageal Echocardiography During Pectus Excavatum Repair 2010, Ann Thorac Surg;89:

19. Post operative effects
Improved exercise tolerance
Improved body image
Decline in