Long-term outcome of asymptomatic patients with congenital cystic adenomatoid malformation

Purpose  Congenital cystic adenomatoid malformation (CCAM) represents a rare congenital anomaly of the lung. It remains controversial whether patients with asymptomatic lesions warrant early surgical intervention. Our aim was to review the outcome of asymptomatic CCAM patients at a paediatric tertiary centre. Methods  The medical case notes of all children with CCAM presenting to our institution between 1986 and 2007 were reviewed. Data on pre- and post-natal investigations, clinical presentation, lesion site, type of surgical procedure, timing, and outcomes of surgery were reviewed. Results  A total of 35 patients were diagnosed with CCAM during the 21-year study period (1986–2007). Sixty percent (n = 21) were asymptomatic at birth including eight patients with prenatal ultrasound scan confirming CCAM. In this group, 18 patients (86%) subsequently developed symptoms (median age 2 years, range 1 month–13 years) and required surgery. Symptoms included pneumonia with or without infected CCAM (43%), respiratory distress (14%) and spontaneous pneumothorax (14%). Eight patients underwent multiple hospital presentations with complications related to CCAM. Of the 21 initially asymptomatic patients, 17 (81%) underwent surgical resection. Only one of these patients was completely asymptomatic prior to surgery. There were eight post-operative complications and no mortality. One patient underwent a second thoracotomy for residual CCAM. The median length of hospital stay was 9 days (range 3–32 days). Conclusion  This study suggests patients who present with asymptomatic CCAM will subsequently become symptomatic. Early surgical referral and intervention may be beneficial to avoid the development of complications.