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.