Evaluation of congenital lung and airway diseases

275 patients diagnosed with congenital lung and airway diseases between 1 January 2000-31 December 2021 were reviewed retrospectively. While 84 (%30,5) patients were in congenital cystic lung disease (CCLD) group, 191 (%69,4) patients were in other congenital lung and airway disease group. In CCLD group, %61,9 of patients had prenatal diagnosis and %38,1 of patients had postnatal diagnosis. In other congenital lung and respiratory tract disease group, %10,5 of patients had prenatal diagnosis and %89,5 of patients had postnatal diagnosis. In CCLD group, most common diagnosis was congenital cystic adenomatoid malformation (CCAM) in 44(%52,3) patients (4 patients had hybrid lesions), followed by pulmonary sequestration (PS) in 20 (%23,8) patients, congenital lobar emphysema (CLA) in 18 (%21,4) patients, bronchogenic cysts (BC) in 3 (%3,6) patients and focal hyperinflation in 3 (%3,6) patients. Among the patients diagnosed with CCAM, %70,45 were asymptomatic and %86,36 had no physical examination findings. Nine (%27,27) patients had normal chest x-ray, while the most common finding was cyst/cysts appearence in 16 (%48,5) patients, followed by mediastinal shift and hyperinflation. Among patients diagnosed with CCAM %77,27 had surgical resection. Pulmonary function test findings were consistent with obstruction in %10 of the patients and small airway disease in %30 of the patients. Among the patients diagnosed with PS, 12 (%63,15) had normal chest x-ray, other patients had findings such as mediastinal shift, cyst, atelectasis, increase in echogenicity and consolidation. Patients treated with pulmonary embolisation and surgical resection had the highest recovery rate as %80. CLA lesions were most commonly (%44) located in left upper lobe. %91,6 of the patients operated with CLA diagnosis had full recovery. Two thirds of the patients diagnosed with BC had cough and wheezing symptoms but none had physical examination findings. All patients had surgical resections and full recovery. In other congenital lung and airway disease group, the most common diagnosis was tracheoesophageal fistula (TEF) in 86 (%45) patients, followed by congenital diapraghmatic hernia (CDH) in 39 (%20,4) patients, tracheomalacia in 30 (%15,7) patients, laryngomalacia in 18 (%9,42) patients, pulmonary hypoplasia (PH) in 11 (%5,75) patients, congenital tracheal stenosis (CSS) in 6 (%3,14) patients, pulmonary agenesia (PA) in 5 (%2,61) patients, subglottic hemangioma (SGH) in 2 (%1,04) patients, vascular ring in 2 (%1,04) patients and congenital tracheal stenosis (CTS) in one patient. Most common accompanying anomali was esophegeal atresia (EA) (%46,51) in patients diagnosed with TEF. Median value for gestational week of prenatal diagnosis was 28,5 weeks and median value for age at time of surgery was 12 days in patients diagnosed with CDH. Half of the patients diagnosed with tracheomalacia had accompanying bronchomalacia. Most common symptom was wheezing in 10 (%55,55) patients among patients diagnosed with laryngomalacia. Median age of diagnosis was 3 months for laryngomalacia. Most common physical examination finding was unilateral decrease in breathing sounds in 7 (%63,63) patients among patients diagnosed with PH. In patients diagnosed with PA, dextrocardia was the most common (%40) accompanying anomaly. One of the patients diagnosed with SGH had beta blocker treatment, the other had no treatment. Hemangiomas disappeared in both patients at follow-up. Both patients diagnosed with vascular ring had diagnosis in the first year of life, one had cough as a symptom. The patient diagnosed with CTS required to have tracheostomy after slide tracheoplasty. In conclusion, congenital lung and respiratory diseases may have varying clinical, radiological features that lead to diagnosis in prenatal or postnatal period while some patients may be diagnosed incidentally during an asymptomatic course. Additional diseases of different systems may accompany. Although surgical treatment may provide a safer follow-up, treatment should be planned individually in each patient.