Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung

Abstract

Introduction: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung disease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding prenatal management and postpartum treatment.

Objective: To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution.

Patients and Method: Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the prenatal ultrasound or that the postnatal chest X-ray showed no lesion.

Results: 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after birth. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor prognosis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0.

Conclusions: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is preferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.