congenital lobar hyperinflation
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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#