100 Clinical Brief Congenital Malformations of the Lung Shahryar Hashemzadeh, Saeid Aslanabadi, Amir H. Jafari Rouhi, Ramin Azhough and Neda Asghari Kaleibar Tabriz University of Medical Sciences Children Hospital, Tabriz, Iran ABSTRACT Congenital malformations of the lung are rare and vary widely in their presentation and severity. The most common manifestation of the congenital cystic disease of the lung at newborn and early infancy is respiratory distress. Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, while producing cough, dyspnea, and thoracic pain. The purpose of this study is to review authors institutional experience of congenital cystic lung disease, with specific reference to diagnosis, treatment, as well as outcome, furthermore, to present some cases with unusual clinical manifestations. [Indian J Pediatr 2007; 74 (2) : 192-194] E-mail: jafariroohi@yahoo.com Key words : Lobar emphysema; Pulmonary sequestration; Bronchogenic cyst; CCAM Congenital malformations of the lung and mediastinum vary considerably in their embryology, presentation, management, and out come. 1 They consist of pulmonary sequestration (PS), cystic adenomatoid malformation (CCAM), congenital lobar emphysema (CLE), and bronchogenic cyst (BC). These four anomalies present a different clinical picture, are often difficult to diagnose, and require surgical management. 2 In two referral centers of Tabriz University of Medical Sciences, The authors evaluated the medical records of surgery realm patients across the March 1996 and March 2005 records with the discharge diagnosis of lung congenital malformation was documented with exclusive attention upon the manifestation which related to the age, method of diagnosis and outcome. CASE REPORTS Congenital lobar emphysema (n=7) Through these patients, 4 were referred by main feature of respiratory distress from birth. Three remainder patients were misdiagnosed, 2 of them were refered as a refractory pneumonia and the other one misdiagnosed as a pneumothorax at a peripheral hospital and an intercostals drain was inserted increasing the respiratory distress, requiring an emergency lobectomy. There was patent ductus arteriosus in 1 case of CLE. All the patients Correspondence and Reprint requests : Dr. Amir H. Jafari Rouhi, Department of Pediatrics, Division of Pediatric Emergency Medicine, Children’s Hospital, Sheshgelan Street, Tabriz, Iran. underwent surgery procedures. About the location of the lesion; 2 in right upper lobe, 1 in right lower lobe and in 4 subjects the lesion was located in left upper lobe. The recovery was uneventful in all seven patients. Bronchogenic cyst (n=6) The most common presentation comprised cough, suppurative bronchitis and hemoptysis with one case of massive hemoptysis. In all cases, the delay between appearance of symptoms, discovery of the cyst, and operation varied from a few hours to 6 years (mean,2 years and 2 months). Plain X-ray along side chest CT scans were chosen as diagnostic modalities for all of patients, which revealed involvement of right upper lobe in two cases, right middle lobe in 1 case, right lower lobe in 1 and left lower lobe in 2 cases. Partial absence of pericardium was seen in one of our cases in this group as a very unusual associated anomalies. All the subjects were asymptomatic and long-term follow-up of pulmonary function is good despite minor radiological anomalies such as compensatory lobar hyperinflation. Congenital cystic adenomatoid malformation (n=3) The presenting symptom was respiratory difficulty or distress. Resembling the prior groups, chest radiography and computerized tomography was the choice of evaluation. In two patients left - upper lobe were affected and one subject had bilobar involvement of upper and middle lobes of left side. Patients had no complications. Indian Journal of Pediatrics, Volume 74—February, 2007 192