Congenital lobar emphysema: diagnostic and therapeutic challenges

Abstract

Congenital lobar emphysema (CLE), a rare condition that usually presents in the neonatal period, can be a diagnostic and therapeutic challenge for the treating clinician. If unrecognised, it is a significant risk at the time of anaesthetic induction. We describe a case of CLE in a 3-month-old boy who was initially treated for suspected aspiration pneumonia at the referring hospital. We highlight the importance of careful consideration of common childhood respiratory illness as well as pneumothorax in the differential diagnosis, and the significance of appropriate preoperative anaesthetic management. We also emphasise the importance of acknowledging a mother's concerns when taking a paediatric history.

A Four-Week-Old Infant With Respiratory Distress: An Emergency Department Case Presentation of Congenital Lobar Emphysema

“…Congenital lobar emphysema is a rare disorder with an estimated incidence of around one in 70,000 live births [1]. While the etiology of CLE varies [3][4][5], ED management and definitive treatment are the same regardless of the underlying cause [6]. Typically, emphysematous changes affect a single lobe, most commonly the left upper, followed by the right middle and right upper lobe [2,[5][6][7].…”

“…The significant challenge to the EP lies in the fact that CLE may mimic much more commonly encountered clinical entities such as pneumonia or pneumothorax [ 3 ]. The tracheal deviation is often present on physical examination, further confusing the clinical picture [ 11 ].…”

Congenital Lobar Overinflation Presenting with Recurrent Pneumothoraces

“…Diagnosis can often be established by characteristic signs on chest radiograph; therefore, a chest radiograph is the first step to evaluate the patient with respiratory distress [2,7]. Typical findings on the chest radiograph are distension and unilateral hyperlucency of the affected lobe, mediastinal shift as well as compression and atelectasis of the adjacent and contralateral lung lobes [2,4].…”

“…Selective intubation of the unaffected lung is another possible approach but is difficult to achieve. Severe neonatal respiratory distress may need emergent surgical treatment, where lobectomy of the affected lobe is performed [5,7].…”

Management of Congenital Lobar Emphysema: The Current Challenges

“…Early neonatal diagnosis is crucial and in many cases is complicated due to the variety of its clinical presentation that varies from mild tachypnea to severe respiratory distress [19].Diagnosis is often confused with pneumonia and pneumothorax [24].A case of CLE with pneumothorax due to a large bulla in an adult has been reported [25].It is a significant risk during an aesthetic induction if undiagnosed preoperatively [26].…”