Two-Year Outcomes in Children Born With a Congenital Pulmonary Malformation

BACKGROUND: Families and clinicians need more information about complications associated with congenital pulmonary malformations (CPMs) to engage in shared decision-making about surgery for asymptomatic children. The study's aim was to prospectively describe symptoms in children with a CPM and to identify markers associated with surgical removal because of symptoms. METHODS: MALFPULM is a prospective population-based nationally representative cohort including pregnant women whose fetus had a CPM. Events occurring between birth and 2 years of age were compared between children who were not operated on, those with elective surgery (reference group), and those operated on because of symptoms. RESULTS: Of the 322 children with complete clinical follow-up at 2 years, 48 (15%) were operated on because of symptoms. When compared with children with elective surgery, they had a higher prenatal maximal CPM Volume Ratio (CVRmax) (P < .001) as well as a higher rate of neonatal respiratory distress (P < .001), eating difficulties (P = .002), pneumothorax (P < .05), or hospitalization for respiratory complication (P < .001). CVRmax greater than 1 cm2 was observed in 44% of children operated on because of symptoms, compared with 14% in other subgroups (P < .001). Only 3% of the children had an infection of the CPM, with no significant influence of the cystic nature of the CPM. Symptom frequency did not differ significantly between children who were not operated on and children with elective surgery, with recurrent cough and wheezing being the most frequent symptoms. CONCLUSION: Symptoms leading to surgical removal were observed in only 15% of children with CPM, with CVRmax greater than 1 cm2 being a significant risk factor. Elective surgery did not modify the frequency of less severe symptoms.