Congenital Cystic Adenomatoid Malformation: Is There a Need for Pregnancy Termination?

Abstract

Aim. Congenital cystic adenomatoid lung malformation is a rare unilateral dysplasia of the lung. Three pathologic types are described in the literature: type I with cysts >2 cm, type II with cysts <1 cm, and type III with microcysts. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the necessity for pregnancy termination according to its prognosis and future mortality. Case. A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side. Other findings were cysts of the choroid plexus and echogenic intracardiac foci. The parents after genetic counseling decided pregnancy termination. The pregnant received cabergoline for ablactation. Conclusion. Congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). Fetal hydrops, cardiac and skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are common cofindings. Genetic counseling is necessary, and pregnancy termination is proposed to the cases with poor prognosis.

Fetal congenital cystic adenomatoid malformation

“…The second reason is that although the incidence of CCAM is reported as rare, I encountered another, more severe case of CCAM within a year, and a sonographer, who has worked in the Torres Strait, for the last 10 years, has encountered several cases within that time. This report outlines the second case and provides evidence in support of prior literature that the routine use of advanced antenatal ultrasound has played a large role in the increasing incidence of the diagnosis of CCAM …”

“…The true incidence of CCAM before and after birth is still uncertain but has been reported as rare (1:2500 to 1:35 000), with no gender difference . The prenatal detection of CCAM with ultrasound is very accurate, and it is evident that the incidence of CCAM has recently increased because of more frequent detection of it through the routine use of advanced ultrasound techniques …”

Congenital cystic adenomatoid malformation: Antenatal ultrasonography features

“…Karyotype was not done as there is no known association of CCAM with chromosomal anomalies. 6 On antenatal sonography bronchopulmonary sequestration (BPS) and congenital diaphragmatic hernia (CDH) are the two most important differential diagnosis of CCAM. CCAM (other than type III) can be differentiated from BPS by demonstrating cyst in lung mass.…”

"Prenataly Diagnosed Bilateral Congenital Cystic Adenoid Malformation, Class 2, a Case Report"

“…They are generally characterized as benign hamartomatous or dysplastic overgrowth of terminal bronchioles with a reduction in the number of alveoli. It results from abnormal maturation of the bronchopulmonary tree [2].…”