| Popis: |
Introduction Fetal anomaly scanning (FAS) increases the diagnosis of congenital lung malformations (CLMs). Uncertainty exists regarding best management; conservative versus elective removal. CLM cases following routine FAS introduction in our region were reviewed. The CLM service provides antenatal counselling at the fetal medicine centre with ongoing long term follow up in our joint medical/surgical clinic. Postnatally diagnosed patients also attend. CLM clinic includes multidisciplinary discussion (neonatalogy, surgery, respiratory, radiology and pathology) with regional teleconference. We anticipate review of all CLM babies born in our region. We aim to provide consistent advice and care with subsequent increased knowledge on the natural history of CLM since patients attend from the antenatal period throughout childhood. Methods Prospective data collection for babies with CLM born 2008–2017 reporting antenatal/postnatal diagnoses, early postnatal course, and management. Results 61 babies had CLM (M:F; 36:25). 53 antenatal/8 postnatal diagnoses. 58/61 had postnatal CT chest (2 moved area and 1 did not attend). Radiological diagnosis: 20/58 (34%) cystic, 7/58 (12%) sequestration, 6/58 (10%) hybrid, 13/58 (22%) lobar overinflation, 10/58 (17%) bronchial atresia spectrum, 2/58 (3%) normal. 11 (18%) had respiratory symptoms in the first 2 months. 5/11 required early surgery (1 antenatal: 4 postnatal). 6/11 had postnatal diagnosis with significant right upper lobe overinflation and 4 of these required emergency surgery. 5/11 were antenatally diagnosed and only 1 required early surgery. 1 had symptoms due to underlying neuromuscular condition, 3 had mild unrelated neonatal respiratory distress which settled. Parents of asymptomatic children with CLM collectively agreed to be small or without concerning features are counselled towards conservative management. Those with larger CLM or any concerning features are advised based on team discussion. 30/61 (49%) had surgery. 31/61 (51%) continue conservative management. 58/61 (95%) remain under follow up. Conclusions A dedicated CLM service provides consistent advice to families with a rare diagnosis. Long term follow-up will help inform future counselling of asymptomatic patients. At present half of families of asymptomatic children opt for elective surgery. In our series, lobar overinflation requiring urgent surgery was not identified at 20 week scanning. Bronchial atresia spectrum was more likely to be identified and less likely to cause symptoms. |
