Near Complete Obliteration of the Left Hemithorax by Congenital Lobar Emphysema in an Adult

Abstract

Congenital lobar emphysema is a rare pulmonary malformation typically diagnosed during infancy and is characterized by bullous disease. A 28-year-old woman, who presented with 1 week of progressive dyspnea and chest pain, was found to have left hemithoracic lung hyperinflation with perfusion deficit upon radiographic evaluation. Bullous disease was found intraoperatively to originate from 1 lower lobe segment. Normal parenchymal lung expansion occurred following resection of the affected segment. Therefore, we present a rare case of congenital lobar emphysema resulting in near-complete occupancy of the left hemithorax and diagnosed in a previously asymptomatic adult without history of predisposing factors.

<p>Congenital lobar emphysema: diagnosis and treatment options</p>

“…Approximately half of patients show symptoms in the first month of life. Although there are few asymptomatic patients described in the literature, most of them are diagnosed in the first 6 months of life 9,20,68. If the symptoms are mild or moderate, conservative treatment is recommended.…”

Fatal congenital lobar emphysema in a puerpera: a case report and literature review

“…CLE is a developmental abnormality usually diagnosed in the neonatal period and is rarely diagnosed in adulthood. Few cases of adults with CLE have been reported before, and we have summarized the characteristics of them in Table 1 [ 5 , 7 – 11 ]. The gold standard for the diagnosis of CLE relies on imaging examinations [ 1 ].…”

“…Nearly all infants with CLE will develop symptoms and are diagnosed within the first 6 months of life [ 2 , 3 ]; CLE in adults is quite rare. Adults with CLE are usually asymptomatic, and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress [ 4 , 5 ]. Imaging studies are regarded as the gold standard for the diagnosis of CLE, which is characterized by overinflation of the involved lobes [ 1 ].…”

An unusual case of unilateral hyperlucent lung

“…Similar results were seen when surgical and medical management were compared in a long-term follow-up study of CLE patients [ 16 ]. Hence, a severely symptomatic patient or progression of disease should be managed with surgical intervention whereas conservative management can be tried with close follow-up for mild or asymptomatic disease [ 16 , 19 , 20 ].…”