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Paediatric Respiratory Disease
P186 Incidence and outcome of congenital lung agenesis in the north of england
  1. N Robertson1,
  2. N Miller2,
  3. J Rankin3,
  4. M McKean4,
  5. M Brodlie5,
  6. M Thomas4
  1. 1James Cook University Hospital, Middlesbourgh, UK
  2. 2National Congenital Anomaly and Rare Disease Registration Service, Public Health England, Newcastle Upon Tyne, UK
  3. 3Institute of Health and Society, Newcastle University, Newcastle Upon Tyne, UK
  4. 4Great North Children’s Hospital, Newcastle Upon Tyne, UK
  5. 5Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK

Abstract

Introduction Congenital lung agenesis is a rare abnormality which has been reported to have an estimated incidence of 1 in 15,000 pregnancies and to carry a poor prognosis, especially when associated with congenital heart anomalies. (1) However, precise incidence data has previously been unavailable and clinical management has improved in recent years with an unknown impact on outcomes.

Methods We used the North of England register of congenital anomalies (NorCAS) cross-referenced with clinical data from our regional paediatric respiratory centre to calculate the first known accurate incidence estimate for this condition. Detailed clinical and outcome data were then collected with informed consent from the families of affected infants.

Results The incidence of lung agenesis was 0.12 per 100,000 live births (95% confidence interval 0.03–0.31). Four cases were identified with a median age at follow-up of 5 years. Lung agenesis was associated with complex congenital heart disease (complete atrioventricular septal defect with left atrial isomerism) in one case, and with aortic coarctation and atrial septal defect in another. Both these patients had their heart defects successfully repaired. The third patient had a normal heart but musculoskeletal problems, while lung agenesis was the only anomaly in the fourth patient. All four patients are well and not on home oxygen.

Conclusions We reviewed the course of the four patients with lung agenesis born in the region between 2004 and 2013, and report that medium term outcomes have been good, even when associated with congenital heart disease and other anomalies. This information will provide a useful starting point when counselling parents whose unborn baby has an antenatal diagnosis of lung agenesis. We aim to follow-up these patients to report long-term outcomes as these remain unknown and there is concern about the potential for the development of late onset pulmonary hypertension.1

Reference

  1. Muensterer O, Abellar R, Otterburn D, et al. Pulmonary agenesis and associated pulmonary hypertension: A case report and review on variability, therapy, and outcome. Eur J Pediatr Surg Rep 2015;3:33–9.

https://doi.org/10.1136/thoraxjnl-2016-209333.329

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