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congenital lobar hyperinflation

Wednesday 22 June 2005

Digital cases

- HPC:390 : Five cases of congenital lobar hyperinflation (or congenital lobar emphysema)
- HPC:422 : Congenital lobar hyperinflation (or congenital lobar emphysema)

Definition: Congenital lobar hyperinflation (or congenital lobar emphysema) is the most frequent fetal/neonatal pulmonary anomaly, before congenital pulmonary airway malformations (CPAMs).

Lobar hyperinflation in the neonate is usually congenital, resulting from cartilage deficiency causing bronchomalacia and distal air trapping.

Nota bene: An acquired lobar hyperinflation is usually associated with chronic lung disease or endobronchial obstruction such as mucus plugging or a pedunculated endobronchial polyp. (17206431)

Synopsis

- Resection of a hyperlucent left upper lobe
- roughly triangular overdistended area of lung
- cut surface: few tiny apparent cysts beneath the pleura
- overinflation of a lobe of lung
- usually middle or upper lobe
- Compression of surrounding normal lung
- May demonstrate partial bronchial obstruction
- Frequently no anatomic cause can be found
- Presents with dyspnea in the first weeks of life
- Lucent area on chest x-ray
- Surgically excised lobe is filled with air

  • does not collapse after removal
    - Microscopically shows distended air spaces
  • best appreciated by comparing with age-matched control lung
    - overdistension of alveoli and alveolar ducts (to four to five times the normal diameter).
  • The magnitude of the distension may not be evident until compared with an age-matched control section of lung.
    - possible interstitial emphysema
    - rare bronchial obstruction associated

Differential diagnosis

- unilobar congenital pulmonary lymphangiectasis (15136998)

Associations

- intralobar pulmonary sequestration (15995872)
- pulmonary lobar lymphangiectasis (HP.13341) (4715376, 5936105)
- lobar bronchial atresia (7093137, 17101340, 5775061)
- abnormal bronchial cartilage (7093137)
- polysplenia (20864788)
- great vessel anomaly (left aortic arch, right descending aorta and left ligamentum arteriosum) (18937763)
- laryngotracheal cleft (17635408)
- congenital heart disease (16616669)
- lobus venae azygos (2982179)
- bronchial sling around a normal right main pulmonary artery (875429)

See also

- lungs

  • pulmonary malformations

References

- Ball valve obstruction of a bronchus causing lobar emphysema in a neonate. Clubley E, England RJ, Cullinane C, Crabbe DC. Pediatr Surg Int. 2007 Jul;23(7):699-702. PMID: 17206431

- Mani H, Suarez E, Stocker JT. The morphologic spectrum of infantile lobar emphysema: a study of 33 cases. Paediatr Respir Rev. 2004;5 Suppl A:S313-20. PMID: 14980289

- Congenital lobar emphysema: a case with bronchial atresia and abnormal bronchial cartilages. Warner JO, Rubin S, Heard BE. Br J Dis Chest. 1982 Apr;76(2):177-84. PMID: 7093137

Portfolio

  • Congenital lobar emphysema
  • Congenital lobar emphysema
  • Congenital lobar emphysema
  • Congenital lobar hyperinflation
  • Congenital lobar hyperinflation