An atypical case of congenital lobar emphysema in an adult, non-smoker patient presenting with pneumothorax

Asnake, Zekarias T.; Salabei, Joshua K.; Pierce, Jordan; Fernández, Angela Pascual; Ahmad, Ramin; Ismail, Zeeshan; Mathew, Calestino

doi:10.1016/j.rmcr.2021.101435

Cited by 1 publication

(2 citation statements)

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“…Rarely, asymptomatic adults can be diagnosed incidentally [ 3 , 4 ]. A case of CLE in an adult presenting with pneumothorax has also been reported [ 5 ]. Left upper lobe (43%) involvement is more prevalent, followed by right middle lobe and right upper lobe [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…Bronchial cartilage anaplasia, hypoplasia, or dysplasia causes bronchial collapse and air trapping due to the ball valve mechanism in one-fourth of cases. Other infrequent causes are polyalveolar lobes and internal and external bronchial obstruction [ 5 , 6 ]. Emerging data suggest that minor transcription errors in the fibroblast growth factor-10 pathways, thyroid transcription factor-1 and Homeobox protein Nkx 2.1 may result in localized bronchial cartilage anomalies, leading to CLE, because these are essential for pulmonary branching morphogenesis [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

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Congenital Lobar Emphysema Presenting as Respiratory Distress in a Newborn

Choudhury¹,

Dubey²,

Kumar³

2023

Cureus

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Congenital lobar emphysema (CLE) is a rare developmental malformation that presents as neonatal respiratory distress and can be successfully managed with prompt intervention. Hyperinflation of the affected lobe with mediastinal shift is the characteristic radiological finding. However, the etiology mostly remains unknown. Here, we report a case of CLE that microscopically revealed bronchial cartilaginous hypoplasia as an underlying cause of this malformation.

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