Chest Wall Deformaties

Question 1

Broad classification of chest wall deformaties

Answer 1

• Pectus excavatum
• Pedtus carinatum
• Poland’s syndrome
• Sternal defects
• Miscellaneous syndromes (Jeune’s disease and Jarcho-Levin syndrome)

Question 2

Most common congenital chest wall deformity

Answer 2

Pectus excavatum

sternal depression and flaring of costal margins

Question 3

Characteristics of Pectus Excavatum

Answer 3

• Association with Marfan’s syndrome
• Excessive growth of lower costal cartilage
  
  • manubrium and first/second ribs usually spared
• Frequently greater depression on Right
• Progressive
  
  • potential for significant worsening with growth

Question 4

Potential physiolgoic consequences of Pectus Excavatum

Answer 4

• Reduced VC
• Reduced exercise tolerance
• Depression of RV
  
  • reduced cardiac performace
  • MV prolapse due to sternal pressure on annulus

Question 5

MC presentation of Pectus Excavatum

Answer 5

• Mostly asymptomatic
• Psychosocial factors and cosmetic conerns most common reason for presentaiton
• Pain at site of deformity

Question 6

__ % of patients with Pectus Excavatum will have scoliosis

Answer 6

~ 25%

Question 7

Diagnostic radiologic features to characterize severity of Pectus Excavatum

Answer 7

Haller Index

Ratio of lateral diameter : AP distance on CXR

Normal index 2.5

Pectus excavatum index > 2.5

Question 8

Caridopulmonary evaluation for Pectus Excavatum

Answer 8

• EKG: abnormalities common
• Pulmonary function: does not correlate well with degree of exercise limitation
• Echocardiography: if MV prolapse suspected

Question 9

Surgical options for Pectus Excavatum

Answer 9

Surgial correction (Standard of Care)

• Ravich repair (open repair)
  
  • subperichondrial resection of costal segments
  • wedge sternal osteotomy (allow sternal elevation)
  • sternal fixation with retrosternal bar
• Nuss repair (minimally invasive repair)
  
  • Thoracoscopic guided convex retrosternal bar placement
    
    • behind sterum through anterior mediastinum
    • rotated to push sternum and deformed cartilage forward
  • Lateral fixation of bar to chest wall
  • Bar placed before growth spurt
  • Greater pain compared to Ravich
  • Bar in place for 3 years
  • Risk of recurrence (~ 7%)
• Sternal eversion (free graft of sternum)
  
  • rarely performed d/t potential for devestating complications

Question 10

Accounts for ~ 10% of chest wall deformities

Answer 10

Pectus carinatum

More common in boys

Presents later in childhood

More likely to present with pain

May be related to history of trauma

Question 11

Haller index for Pectus carinatum

Answer 11

Index < 2

Question 12

Surgical approach to Pectus carinatum

Answer 12

• Subperichondrial excision of affected costal elements
• 1 or more osteotomies

Question 13

Definition of Poland’s syndrome

Answer 13

Unilateral absence of pectoralis major and minor muscles + syndactyly

Question 14

Proposed etiology of Poland’s sydnrome

Answer 14

in urtero vascular events that affect subclavian supply

Question 15

Assocaited defects and characteristics of Poland’s syndrome

Answer 15

• Amastia
• Absence of axillary hair
• Rib aplasia (profound chest wall deformities)

Question 16

Surgical correction of Poland’s syndrome

Answer 16

Rib grafts

Patch materials

Question 17

Types of sternal defects

Answer 17

• Sternal cleft
• Ectopia cordis
• Thoracoabdominal extopia cordis/Cantrell’s pentology

Question 18

Characteristics of sternal cleft

Answer 18

Defect of upper portion of sternum

Protrusion during infant crying

Heart, lungs, overlying skin normal

Repair indicated to protect mediastinal structures

Question 19

Definition and characteristics of ectopia cordis

Answer 19

• Complete fialure of midline covering of the heart (absence of skin, sternum, pericardium)
• Part or all of the heart may be positioned outside of the heart
• Heart usually rotated anteriorly
• Congenital cardiac defects in almost all cases

Question 20

5 components of Cantrell’s pentology

(Thoracoabdominal ectopia cordis)

Answer 20

• Omphalocele
• Anterior diaphragmatic herina
• Sternal cleft
• Ectopia cordis
• Intracardiac defect (VSD or ventricular diverticulum)

Question 21

Distinct finding of heart with thoracoabdominal ectopis cordis (Cantrell’s pentology)

Answer 21

Heart is covered by thin membrane and not rotated

(Different form ectopia cordis where heart is exposed and rotated anteriorly)

Question 22

Intracardiac defects noted with thoracoabdominal ectopia cordis (Cantrell’s pentology)

Answer 22

VSD

LV diverticulum (unclear significance)

Question 23

2 miscellaneous syndromes with chest wall deformities

Answer 23

Jeune’s syndrome

Jarcho-Levin synrome

Question 24

Definition and characteristics of Jeune’s syndrome

Answer 24

(a.k.a. asphyxiating thoracic dystorphy)

• Narrow, bell shaped chest that is severely restrictive
• Ribs oriented horizontally and foreshortened
• Pulmonary development limited
  
  • extremely poor respiratory mechanics
  • frequent pulmonary complications

Question 25

Definition and characteristics of Jarcho-Levin syndrome

Answer 25

(a.k.a Spondylothoracic dysplasia) * presence of alternating hemivertebrae * short in height, resulting in close approxiation of ribs * very poor pulmonary mechanics * frequent pulmonary complications