Pectus Excavatum

Epidemiology

  • Incidence: 8 per 1000

Etiology

  • Probably due to congenital defect of connective tissue surrounding sternum
  • Associated with : Marfan’s syndrome

Physiology

  • Pulmonary HTN due to chest wall disease (decreased chest volume with normal rib cage mobility)
  • Hypoventilation leads to hypoxia/ acidosis (with resultant pulmonary vasoconstriction)

Diagnosis

  • PFT’s (usually mild restriction):
    -FEV1/FVC ratio: normal (about 80% of predicted)
    -VC: normal-mildly decreased (usually about 90% of predicted)
    -TLC: normal-mildly decreased (usually about 80% of predicted)
    -Lung compliance: normal
    -MVV: normal

Exercise test: controversial as to whether exercise is altered


Clinical

(symptoms occur in up to 30% of cases)

  • Chest pain
  • Exertional dyspnea
  • Sunken anterior chest
  • Pulmonary Hypertension/Cor Pulmonale (see [[Pulmonary Hypertension]])
  • Chronic Hypoventilation (see Chronic Hypoventilation, [[Chronic Hypoventilation]])

Treatment

  • Surgery: useful for patients with transthoracic distance: sternovertebral distance ratio >3.5
  • May improve TLC/ MVV/ VO2max (controversial) but is cosmetically useful
  • Younger patients may benefit more (due to normal growth following surgery) but this is unproven

References

  • xxx