PURPOSE: To review the clinical and pathologic features, diagnosis and managemenent of congenital cystic malformation of the lung in infants, children and adolescents.
METHODS: A 30 year retrospective review of patients medical records diagnosed of congenital cystic lung malformation based on the histopathologic confirmation admitted at Philippine Heart Center between 1975 to 2005 was done.
RESULTS: Histopathologic examination revealed the following: congenital cystic adenomatoid malformation (n= 9) accounted for 45%, congenital lobar emphysema (n= 5) 25%, bronchogenic cyst (n=3) 15% and pulmonary sequestration (n= 3) 15%. Twelve patients were under 1 year of age 5 of whom where neonates. There was no sex preponderance in all four diseases. Most common symptoms were dyspnea, acute respiratory tract infection, fever and cyanosis while tachypnea, intercostals retractions, decreased breath sounds and tachycardia were the most common physical findings. The duration of illness ranged from 2 days to 6 years. Dyspnea was noted in 80% of patients. Lobectomy was done in 70% of patients. Immediate post-operative complication in decreasing frequency were pneumonia, atelectasis, pneumothorax, septicemia, bronchopleural fistula and bleeding were noted in 80% of patients.
CONCLUSION: There was no significant correlation between survival with the number of lobes resected.Poor post-operative outcome was not associated with the types of congenital cystic lung malformation.There was also no significant correlation between presence of post-operative complications and poor outcome.
CLINICAL IMPLICATIONS: Today congenital cystic lung malformation is a well-known disease in infants and children because of an increase in number referred and admitted in our institution. It is therefore the intent of this papaer to present the clinical profile of patients with congenital cystic lung malformation with the hope that these data may help in future assessment and management of these patients.
DISCLOSURE: Jean Marie Jamero, None.