P555 A rare case of congenital lobar overinflation. (June 2019)

• Record Type: Journal Article
• Authors: Finnegan, Jennifer; Corcoran, John David; Tarrant, Ailbhe
• Is Part Of: Archives of disease in childhood. Volume 104:Supplement 3(2019)
• Page Start: A375
• Page End: A375
• Publication Date: 2019-06
• Subjects: Infants -- Diseases -- Periodicals; Newborn infants -- Diseases -- Periodicals; Fetus -- Diseases -- Periodicals; 618.920105
• DOI: 10.1136/archdischild-2019-epa.889
• Languages: English
• ISSNs: 1359-2998

Abstract

Abstract : Background: AF was born by normal vaginal delivery at 38+4 weeks gestation. A normal antenatal scan was performed at 22 weeks. Septic workup was performed and antibiotics commenced due to PROM and GBS carriage in the mother. Clinical course and imaging She was admitted to NICU with tachypnoea and increased work of breathing at three hours of age. Ambient incubator oxygen up to 33% was commenced to maintain O2 saturations above 94%. The initial working diagnosis was of TTN. Initial chest x-ray showed a left lower lobe opacity and left pleural effusion. Oxygen was weaned and discontinued. Repeat imaging on day 3 showed left retrocardiac density and left lower lobe segmental atelectasis. The left upper lobe and lingula demonstrated hyperinflation. A decision was made to treat with IV antibiotics for 5 days for a suspected congenital pneumonia. Imaging was repeated on day 4 due to deterioration in clinical condition with increasing tachypnoea and a further requirement for oxygen. Hi-flow oxygen was commenced, with FiO2 of 35%. There was a further increase in hyperlucency of left upper lobe with contralateral midline shift and left lower lobe collapse. A lateral x-ray confirmed that hyperlucency was due to overexpansion and suggestive of congenital lobar overinflation (CLO) rather than pneumothorax. Management: High-frequency oxygen was discontinued and she remained on ambient O2 and was nursed right side up. She remained in a stable condition until transfer to a tertiary hospital where CT thorax confirmed the diagnosis. She awaits pulmonary lobectomy. Congenital lobar overinflation: CLO is a rare disorder characterised by hyperinflation of one or more pulmonary lobes. It has a prevalence of 1 in 20, 000 to 1 in 30, 000. It most commonly affects the left upper lobe. A narrow bronchus causes collapse and air trapping during expiration. Cases can be diagnosed antenatally during foetal ultrasound. Although 50% of cases appear in the newborn period, symptoms of tachypnoea can develop into childhood. Chest x-ray and CT are diagnostic in full-term, non-ventilated babies. X-ray typically shows increased density in the affected lobe initially, transitioning to hyperlucency as the affected lobe overinflates. The CT confirms the lobar overinflation, absence of bullae etc, and evaluates for the possibility of an aberrant mediastinal vessel as a potential cause. Lobectomy of affected lobe is a widely accepted treatment. Conclusion: CLO is typically associated with progressive respiratory distress and mediastinal shift. Although rare, CLO should remain a differential for respiratory distress in a term neonate.