Antenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and Magnetic Resonance Imaging (MRI)

Susanne E Gruessner,Corinna Peter,Gerhard Alzen,Hermann Hertel,Charles O A Omwandho,Eva Bültmann

doi:10.4236/ojog.2012.24076

Abstract

Purpose: Antenatal diagnosis of congenital cystic adenomatoid lung malformation (CCAM) is vital for disease surveillance and postnatal care. Ultrasonography (US) has been the imaging gold standard for antenatal CCAM assessment. However, one of the limitations of US is the “vanishing phenomenon” caused by isoechogenicity of CCAM tissue and adjacent normal lung parenchyma. Methods: Antenatal serial US were concurrently used with magnetic resonance imaging (MRI) to monitor macro- and microcystic lesions. Results: In both pregnant women, antenatal US and MRI confirmed the presence, in the fetus, of cystic lesions and predicted disease regression/progression as well as the need for postnatal surgical intervention. Several advantages were detected by using both—serial US and MRI (over serial US alone)—including improved signal intensity, exact volume size measurements, precise CCAM location in particular for patients with adverse ultrasound conditions. Both neonates underwent surgical resection and had an uneventful post-operative course. Conclusions: Antenatal use of MRI as well as serial US improved information regarding tissue resolution and delineation of CCAM. The information from two imaging modalities was complementary. Our literature review confirmed the emerging role of prenatal MRI for postnatal monitoring and management of CCAM.

Highlights

Antenatal serial US were concurrently used with magnetic resonance imaging (MRI) to monitor macro- and microcystic lesions
Congenital cystic adenomatoid lung malformation (CCAM) is characterized by various cystic intrathoracic masses caused by proliferative terminal respiratory bronchioles and by a reduction in the number of normal alveoli
Type II is the second most common form, detected in up to 20% to 35% of patients with CCAM: the multiple cysts are less than 20 mm in diameter and are lined by cylindrical or cuboidal epithelium, with prominent smooth or striated muscle

Summary

INTRODUCTION

Congenital cystic adenomatoid lung malformation (CCAM) is characterized by various cystic intrathoracic masses caused by proliferative terminal respiratory bronchioles and by a reduction in the number of normal alveoli It usually affects a single pulmonary lobe; multi-lobular or bilateral involvement is rare [1]. Type III is the least common form, detected in up to 10% of patients with CCAM: the solid microcysts are rarely larger than 2 mm in diameter and are lined by cuboidal epithelium, often intricately folded [1]. The advantages of magnetic resonance imaging (MRI) especially in cases of attenuate ultrasound signals include exact measurement of the lesion(s)-tolung volume ratio, superior tissue resolution, with determination of disease progression or regression and precise determination of CCAM location, and need for a low number of studies [17,18,19]. Ethical clearence was obtained from the relevant office and experiments conducted in accordance with the Helsinki declaration of 1982 regarding use of human subjects

Case 1

Case 2

DISCUSSION

CONCLUSION