G306(P) A rare case of congenital left lung aplasia diagnosed antenatally. (25th October 2020)

• Record Type: Journal Article
• Authors: Jagga, M; Thompson, P; Rasiah, SV
• Is Part Of: Archives of disease in childhood. Volume 105(2020)Supplement 1
• Page Start: A112
• Page End: A112
• Publication Date: 2020-10-25
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2020-rcpch.266
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: Pulmonary agenesis was first described in 1673 by De Pozze in a female cadaver biopsy. The condition is characterised by undeveloped pulmonary vessels, bronchi, and parenchyma. It can present bilaterally or unilaterally and, in the latter, the remaining lung contains twice as much alveoli as normal, but has normal bronchi. Although the aplastic side does not have the same structures, it has a rudimentary bronchus. It is extremely rare, occurring in 1:100, 000 births. Nearly a third of patients have associated congenital heart diseases. We report a case of unilateral left lung aplasia which was diagnosed antenatally. Case Report: A female baby was born by vaginal delivery at term, weighing 3700 grams. Antenatally the baby was noted to have mediastinal shift on 20 week scan, raising suspicion and leading to a diagnosis of complete left lung aplasia, left pulmonary artery hypoplasia, with normal right lung on subsequent MRI scan at 28 weeks gestation. Foetal ECHO showed normal cardiovascular anatomy. Baby cried immediately at birth and did not require any resuscitation but started to show signs of respiratory distress at about 15 minutes of age. Baby was transferred to the neonatal intensive care unit and managed on 6 litres of high flow until 12 hours of age. Chest x-ray shows mediastinal shift to the left, with possible right lung hyperplasia and a small undeveloped left lung. She was subsequently breathing by herself with no signs of respiratory distress and discharged home with paediatric respiratory follow-up. Conclusion: Unilateral lung aplasia is a rare congenital anomaly, usually diagnosed later in life as an incidental finding. It is very rare for antenatal diagnosis, especially if not associated with congenital heart defects. This case was identified because of the mediastinal shift. Children with unilateral lung aplasia are at an increased risk of respiratory tract infections and need regular follow up with respiratory team. Right sided lung aplasia might be associated with Scimitar syndrome. Finally, cases from the literature show poorer outcomes associated with right sided lesions (30% survival into adulthood) compared to left sided lesions (50% survival into adulthood).