Natural courses and prognostic factors of pulmonary underdevelopment except for congenital diaphragmatic hernia

Abstract

Purpose: Pulmonary underdevelopment is one of the pulmonary causes of neonatal mortality, which is categorized into pulmonary agenesis, pulmonary aplasia, and pulmonary hypoplasia. Congenital diaphragmatic hernia is a well-known cause of pulmonary hy poplasia; however, further studies are needed for other etiologies. Patients with pulmonary underdevelopment show varying de grees of clinical severity. Most patients with pulmonary underdevelopment have respiratory distress at birth, while a few have only mild symptoms. Our study investigated the natural course and prognosis of pulmonary underdevelopment by analyzing multiple cases from a single tertiary medical center. Methods: A retrospective review was conducted on patients diagnosed with pulmonary hypoplasia and agenesis from January 2000 to August 2019. Cases were extracted from the clinical database of Seoul National University Children’s Hospital by searching for keywords related to pulmonary underdevelopment. Results: A total of 28 patients were identified. Four patients had pulmonary agenesis, and all were idiopathic. Twenty-four patients had evidence of pulmonary hypoplasia with diverse causes. The median age at diagnosis was 11.5 days (range, 1-240 days) and the mean gestational age was 35 weeks 2 days±3 weeks 6 days. Twenty patients received ventilator care and 11 had various degrees of pulmonary hypertension. The long-term survival rate was 50%. The median age of death among pulmonary hypoplasia patients were 11 days (range, 1-730 days). All patients without ventilator care survived. The incidence of ventilator care, pneumothorax, and pulmonary hypertension were significantly higher among patients who died. Conclusion: The prognosis of pulmonary underdevelopment depends on the severity of respiratory distress and pulmonary hyper tension in earlier life. Patients with severe distress appear to have severe degree of pulmonary hypoplasia. Patients without ventila tor support in the neonatal period survived without significant morbidities. (Allergy Asthma Respir Dis 2020;8:206-212)