Congenital Lobar Emphysema: A Clinicopathologic Evaluation of 14 Cases

Abstract

Controversy still exists concerning the diagnosis and treatment of congenital lobar emphysema (CLE). Although surgical removal of the affected lobe is the most commonly accepted form of treatment, detection of milder or even asymptomatic cases is usually followed by a more conservative management of patients, i. e. non-surgical treatment and follow-up. We therefore decided to evaluate our patients with CLE, placing special emphasis on treatment and diagnostic techniques. We also evaluated quantitative analyses of alveolar diameters. Fourteen children with CLE were analysed retrospectively, including age, sex, clinical picture, localisation, diagnostic and surgical modalities and histopathologic diagnosis. The alveolar diameters of affected lobes were compared with those of the lobectomised patients with other non-obstructive respiratory diseases. All children but one had severe respiratory distress as an initial symptom. All patients, except newborns, had a history of pulmonary infection. All cases underwent thoracic CT examination as the main radiologic method. In all of the patients, only one lobe was affected. We found an obvious mediastinal shift and atelectasis of adjacent lobes due to compression of the affected lobe. The affected lobe was therefore surgically removed in all of the children. In one case, we had to carry out a partial lobectomy to reduce the duration of the operation, due to an intraoperative fall of oxygen saturation. All of the children had an uneventful clinical course postoperatively. The alveolar diameters of the cases with CLE were significantly greater than those of the control patients. We think that the majority of cases with CLE have too severe respiratory distress to avoid surgical removal of affected lobe. Conservative management should be reserved only for patients with milder symptoms or no distress at all.

Congenital lobar emphysema: Pitfalls in diagnosis

“…Early surgical intervention has been found to significantly improve the condition of the patient. [ 9 10 ] However, the number of lobes affected may also determine the outcome of surgery. Nazem et al .…”

Congenital Lobar Emphysema Diagnosed in Adult Age: A Case Report

“…The disease is generally seen in the neonatal period and patients are usually asymptomatic (3)(4)(5)(6). Progressive respiratory distress occurs in hours or sometimes in days after birth.…”

“…Airway obstruction is the most common cause of the disease, but the cause of the disease cannot be detected in 25% of the patients. It manifests with progressive dyspnoea and tachypnea a few days after birth in more than half of the cases (3)(4)(5). It is more prevalent among males (6) with an M/F ratio of 3/1.…”

Congenital lobar emphysema

“…Pathologically two forms are recognized: hypoalveolar and polyalveolar. 2 The chest radiographs reveal a radiodense lobe initially at birth that becomes progressively hyperlucent and hyperexpanded. CT of the chest reveals a hyperlucent lobe with attenuated vascular markings with contralateral mediastinal shift and compression atelectasis of the ipsilateral lung.…”

“…Treatment is conservative for cases with minimal symptoms and consist of surgical lobectomy in severe cases (as in our case). 2 …”