BACKGROUND: Congenital lobar emphysema (CLE) is characterised by over distension of one lobe and pressure on the adjacent lobe and mediastinum. In this study, we review the pathological results of our paediatric patients with CLE, highlighting the early and late complications that occurred in these patients. PATIENTS AND METHODS: In a prospective study from 1996 to 2008, we evaluated 30 patients with CLE diagnosis. Variables collected included sex, age at the time of diagnosis, radiological diagnostic method, type of treatment, pathological analysis, surgical findings and early postoperative complications. Parents were asked to refer to our clinic for follow-up and evaluation of late complications. RESULTS: Thirty patients and males accounted for majority of the study population (n = 20, 67%). The mean age of male and female patients (at admission) was 7.2 ± 2.3 and 4.7 ± 1.2 months respectively (P = not significant). The main diagnostic method was chest x-ray (CXR) in all patients. Abnormal bronchial cartilage was found in 71% of patients. The most affected lobe was left upper lobe (50%). Associated anomalies were seen in four patients. Early postoperative periodhadtwo cases of pneumothoraces. At six month follow up, five (25%) males and four females (40%) had delayed weight gain. Permanent oxygen dependency was seen in two patients. Twenty- six patients underwent thoracotomy. Mortality rate was 13%. Base deficit at the time of admission was greater in those patients who eventually died, (-8.6 ± 1.2 versus -3.1 ± 0.4) (P = 0.0003). There were two deaths in the bilobar involvement group and two in the unilobar involvement group (P = 0.07, near significant). CONCLUSION: This study confirms that the number of affected lobes and base deficit at the time of admission were associated with significantly increased mortality.
BACKGROUND:Congenital lobar emphysema (CLE) is characterised by over distension of one lobe and pressure on the adjacent lobe and mediastinum. In this study, we review the pathological results of our paediatric patients with CLE, highlighting the early and late complications that occurred in these patients. PATIENTS AND METHODS: In a prospective study from 1996 to 2008, we evaluated 30 patients with CLE diagnosis. Variables collected included sex, age at the time of diagnosis, radiological diagnostic method, type of treatment, pathological analysis, surgical findings and early postoperative complications. Parents were asked to refer to our clinic for follow-up and evaluation of late complications. RESULTS: Thirty patients and males accounted for majority of the study population (n = 20, 67%). The mean age of male and female patients (at admission) was 7.2 ± 2.3 and 4.7 ± 1.2 months respectively (P = not significant). The main diagnostic method was chest x-ray (CXR) in all patients. Abnormal bronchial cartilage was found in 71% of patients. The most affected lobe was left upper lobe (50%). Associated anomalies were seen in four patients. Early postoperative periodhadtwo cases of pneumothoraces. At six month follow up, five (25%) males and four females (40%) had delayed weight gain. Permanent oxygen dependency was seen in two patients. Twenty- six patients underwent thoracotomy. Mortality rate was 13%. Base deficit at the time of admission was greater in those patients who eventually died, (-8.6 ± 1.2 versus -3.1 ± 0.4) (P = 0.0003). There were two deaths in the bilobar involvement group and two in the unilobar involvement group (P = 0.07, near significant). CONCLUSION: This study confirms that the number of affected lobes and base deficit at the time of admission were associated with significantly increased mortality.