Chest Wall Deformaties Flashcards

1
Q

Broad classification of chest wall deformaties

A
  • Pectus excavatum
  • Pedtus carinatum
  • Poland’s syndrome
  • Sternal defects
  • Miscellaneous syndromes (Jeune’s disease and Jarcho-Levin syndrome)
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2
Q

Most common congenital chest wall deformity

A

Pectus excavatum

sternal depression and flaring of costal margins

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3
Q

Characteristics of Pectus Excavatum

A
  • 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
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4
Q

Potential physiolgoic consequences of Pectus Excavatum

A
  • Reduced VC
  • Reduced exercise tolerance
  • Depression of RV
    • reduced cardiac performace
    • MV prolapse due to sternal pressure on annulus
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5
Q

MC presentation of Pectus Excavatum

A
  • Mostly asymptomatic
  • Psychosocial factors and cosmetic conerns most common reason for presentaiton
  • Pain at site of deformity
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6
Q

__ % of patients with Pectus Excavatum will have scoliosis

A

~ 25%

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7
Q

Diagnostic radiologic features to characterize severity of Pectus Excavatum

A

Haller Index

Ratio of lateral diameter : AP distance on CXR

Normal index 2.5

Pectus excavatum index > 2.5

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8
Q

Caridopulmonary evaluation for Pectus Excavatum

A
  • EKG: abnormalities common
  • Pulmonary function: does not correlate well with degree of exercise limitation
  • Echocardiography: if MV prolapse suspected
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9
Q

Surgical options for Pectus Excavatum

A

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
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10
Q

Accounts for ~ 10% of chest wall deformities

A

Pectus carinatum

More common in boys

Presents later in childhood

More likely to present with pain

May be related to history of trauma

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11
Q

Haller index for Pectus carinatum

A

Index < 2

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12
Q

Surgical approach to Pectus carinatum

A
  • Subperichondrial excision of affected costal elements
  • 1 or more osteotomies
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13
Q

Definition of Poland’s syndrome

A

Unilateral absence of pectoralis major and minor muscles + syndactyly

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14
Q

Proposed etiology of Poland’s sydnrome

A

in urtero vascular events that affect subclavian supply

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15
Q

Assocaited defects and characteristics of Poland’s syndrome

A
  • Amastia
  • Absence of axillary hair
  • Rib aplasia (profound chest wall deformities)
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16
Q

Surgical correction of Poland’s syndrome

A

Rib grafts

Patch materials

17
Q

Types of sternal defects

A
  • Sternal cleft
  • Ectopia cordis
  • Thoracoabdominal extopia cordis/Cantrell’s pentology
18
Q

Characteristics of sternal cleft

A

Defect of upper portion of sternum

Protrusion during infant crying

Heart, lungs, overlying skin normal

Repair indicated to protect mediastinal structures

19
Q

Definition and characteristics of ectopia cordis

A
  • 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
20
Q

5 components of Cantrell’s pentology

(Thoracoabdominal ectopia cordis)

A
  • Omphalocele
  • Anterior diaphragmatic herina
  • Sternal cleft
  • Ectopia cordis
  • Intracardiac defect (VSD or ventricular diverticulum)
21
Q

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

A

Heart is covered by thin membrane and not rotated

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

22
Q

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

A

VSD

LV diverticulum (unclear significance)

23
Q

2 miscellaneous syndromes with chest wall deformities

A

Jeune’s syndrome

Jarcho-Levin synrome

24
Q

Definition and characteristics of Jeune’s syndrome

A

(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
25
Q

Definition and characteristics of Jarcho-Levin syndrome

A

(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