Follow-up of Congenital Lung Malformations (CLM) in a Pediatric tertiary center

Introduction: CLM represent a heterogeneous group of rare developmental disorders with poor prognostic description. Objectives: Describe the follow-up of patients with CLM followed in a tertiary center. Methods: Review of clinical records of patients extracted from the database of a Pediatric Respiratory Unit (2002-17). Descriptive analysis of demographic and clinical data at diagnosis, surgical intervention and follow-up including lung function tests(LFT). Results: We report 58 cases: 18 diaphragmatic hernia with pulmonary hypoplasia(DH); 9 congenital cystic adenomatoid malformation(CCAM); 7 pulmonary sequestration(PS); 5 lobar emphysema(LE); 5 bronchial anomalies(BA); 4 tracheal stenosis(TS); 4 pulmonary vascular malformations(PVM); 3 tracheoesophageal fistula(TEF); 2 bronchogenic cyst(BC) and 1 pulmonary cystic malformation not otherwise specified. Current mean age is 7,2±5,1 years; 57% male. Prenatal diagnosis by ultrasound was made in 28(48%), almost half in 2nd trimester. The median age at post-natal diagnosis(PoND) was 4 (1-96)months. PoND was made by x-ray or CT-scan in 12(75%) and bronchoscopy in 3(19%). Neonatal respiratory distress syndrome was the most common presentation(40%). Globally, 47(81%) underwent surgery (50% pulmonary), all cases of DH, CCAML, PS, TEF and BC, 3/4 TS, 3/5 LE and 2/4 PVM and none of BA. Pathology results were obtained in 18/47(38,3%). 86.2% children remain on follow-up, 17(29%) persist symptomatic and 12/22(55%) performed LFT: 9(75%) obstructive (3 with asthma); 2(17%) restrictive, both having scoliosis. Four patients were lost to follow-up. Conclusions: Markers of evolution of CLM are difficult to homogenize. Clinical registries may help design more comprehensive care.