Congenital lobar emphysema: 30-year case series in two university hospitals

Abstract

OBJECTIVE: To review the cases of patients with congenital lobar emphysema (CLE) submitted to surgical treatment at two university hospitals over a 30-year period. METHODS: We reviewed the medical records of children with CLE undergoing surgical treatment between 1979 and 2009 at the Botucatu School of Medicine Hospital das Clínicas or the Mogi das Cruzes University Hospital. We analyzed data regarding symptoms, physical examination, radiographic findings, diagnosis, surgical treatment, and postoperative follow-up. RESULTS: During the period studied, 20 children with CLE underwent surgery. The mean age at the time of surgery was 6.9 months (range, 9 days to 4 years). All of the cases presented with symptoms at birth or during the first months of life. In all cases, chest X-rays were useful in defining the diagnosis. In cases of moderate respiratory distress, chest CT facilitated the diagnosis. One patient with severe respiratory distress was misdiagnosed with hypertensive pneumothorax and underwent chest tube drainage. Only patients with moderate respiratory distress were submitted to bronchoscopy, which revealed no tracheobronchial abnormalities. The surgical approach was lateral muscle-sparing thoracotomy. The left upper and middle lobes were the most often affected, followed by the right upper lobe. Lobectomy was performed in 18 cases, whereas bilobectomy was performed in 2 (together with bronchogenic cyst resection in 1 of those). No postoperative complications were observed. Postoperative follow-up time was at least 24 months (mean, 60 months), and no late complications were observed. CONCLUSIONS: Although CLE is an uncommon, still neglected disease of uncertain etiology, the radiological diagnosis is easily made and surgical treatment is effective.

Congenital lobar emphysema with pulmonary extramedullary hematopoiesis

“…Recently theory of polyalveosis has been described as a cause of Cle (9). In 20% cases, Cle is known to occur with other malformations and especially cardiac malformations (5). The bronchial cartilage was normal in our case.…”

“…eighteen patients were treated by lobectomy while in two patients bilobectomy was performed, in conjunction with bronchogenic cyst resection in one case. There were no cartilaginous changes or other etiological factors except for a bronchogenic cyst compressing the upper lobe in one case (5).…”

“…90% of cases present before 6 months of age but can occur as late as 5 years. Male children are affected more commonly than females (5).…”

Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

“…On the other hand, in most cases such babies may be misdiagnosed as pneumonia leading to unnecessary antibiotic treatment and loss of valuable time in diagnosis. 14 In an Indian series, a correct diagnosis on chest radiography before referral was made in only 4 out of 10 cases even though the diagnosis could be arrived at in the 10 cases using the initial radiographs. 5 In our case, the lesion was suspected due to persistent oxygen requirement, absence of clinical and laboratory signs of septicemia and suggestive radiological findings.…”

A Case Report on Congenital Lobar Emphysema

“…Nelson RL in 1932 first described congenital lobar emphysema (CLE) [1]. In this, air enters lungs, cannot escape, and causing over inflation of lung lobes.…”