TY - JOUR T1 - Prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS): Long-term follow-up of 119 cases JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA3146 VL - 48 IS - suppl 60 SP - PA3146 AU - James Cook AU - Lyn S. Chitty AU - Paolo De Coppi AU - Michael Ashworth AU - Colin Wallis Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA3146.abstract N2 - Background: A paucity of evidence regarding the natural history of CCAM and PS has resulted in a divergence in management strategy of asymptomatic cases.Methods: Retrospective review of all cases referred to a single institution from 1996 and followed-up for at least 5 years. Study entry required prenatal identification of a cystic lung lesion on ultrasound scan and confirmation of CCAM/PS on postnatal CT imaging.Results: 51 (43%) patients were managed surgically; 8 (6.7%) as a neonatal emergency. Indications for elective surgery included: concerning features on CT, 6 (5.1%); medical advice, 20 (17%); other, 6 (5%). Recurrent respiratory infection was the indication in 11 (9.2%) with a median age at surgery of 1.6 years (range 0.4 to 4.6 years). No cases of malignancy were identified on histological examination.68 (57%) patients were managed conservatively for a median period of 9.9 years (range 5.2 years to 18 years). Of these patients 52 (76%) continue follow-up and remain asymptomatic. Sixteen patients are not followed-up; 4 (5.9%) following spontaneous resolution and 12 (17%) for other reasons.Conclusion: Conservative management of asymptomatic CCAM and PS is a reasonable option during the paediatric period.The risk of recurrent infection is <10% and declines following the second year [figure 1],spontaneous resolution is possible and malignancy was not described in this series. ER -