Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung

Abstract

The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients. Fourteen patients were reviewed regarding the antenatal ultrasonography findings and postnatal clinical course. They were then classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 2), group B had respiratory symptoms just after birth (n = 6), and group C was asymptomatic at birth (n = 6). The postnatal clinical courses in three groups were reviewed. In group A, all two patients with hydrops fetalis died just after birth. In group B, six patients had a severe respiratory distress and underwent operation during the neonatal period. In group C, five out of six patients were asymptomatic and received elective operation during the early infant period. In the remaining one patient, the lesion spontaneously disappeared over time after birth. The mean age at the time of operation in group B and group C was 4.5 days and 4.5 months of age, respectively. In almost all patients in group C, we performed an operation within the first 6 months. During this observation period, we did not experience any complications associated with CCAM. In patients with hydrops fetalis, fetal intervention is thought to be needed. In patients with asymptomatic CCAM, an elective operation during the early infant period is recommended to prevent the risk of complications associated with CCAM before 6 months of age. In addition, we recommend the performance of a partial lung resection using an axillary skin crease incision in order to obtain a good postoperative quality of life.