Prenatal Sonographic Features of Congenital Lobar Emphysema

Abstract

The prenatal sonographic features of congenital lobar emphysema (CLE) have not been well characterised. Five cases have been reported in the literature and on all these occasions either an echogenic (3) or a cystic (2) lung lesion was detected prenatally and the diagnosis was confirmed after the operation. This is the sixth case of CLE in the literature with prenatal sonographic features documented. The prenatal scans of a 23-year-old lady performed at 22 weeks of gestation revealed cystic lesions and increased echogenicity of the right fetal lung. There were no other anomalies and the karyotype was normal. The lesion decreased in size at 28 weeks and the baby was born by a normal vaginal delivery at 41 weeks. CT scan performed on day 6 confirmed cystic changes on the right lung with compression of the right lower lobe. A repeat CT scan performed at 4 months revealed extensive cystic changes in a hyper-inflated right lung and mediastinal shift to the left. At operation, abnormally inflated right upper and middle lobes were found suggesting a CLE. There were no subsequent complications after removal and histology confirmed CLE. The reported cases are reviewed and the prenatal sonographic features of CLE are discussed.

Congenital lobar emphysema with cystic lung abnormality: Antenatal ultrasound appearance

“…The pathophysiology of CLE consists of disruptions of brochopulmonary development resulting in intrinsic obstruction of bronchus [4]. Intrinsic obstruction involves dysplastic bronchial cartilage or endobronchial lesion with blockage due to mucosal fold, septa, or mucosal plugs [6]. Diffuse bronchial inflammation secondary to congenital cytomegalovirus infection has been reported as a cause of CLE [8].…”

“…Diffuse bronchial inflammation secondary to congenital cytomegalovirus infection has been reported as a cause of CLE [8]. However, the exact cause of CLE is difficult to determine, and a definitive cause cannot be identified in approximately 50% of cause [6]. Antenatal imaging with sonography has resulted in early identification and serial evaluation of congenital lung lesions.…”

“…Mostly, fetal sonographic features of CLE have a bright echogenic Youn Jeong Lee, et al CLE with cystic lung abnormality lung without abnormal blood flow. This increased echogenicity is thought to result from excessive lung fluid in the alveoli, and negative intra-thoracic pressure postnatally that results in airway collapse and air trapping leading to the manifestation of CLE [4,6]. Thus far, 3 cases of cystic lesions have been reported (Table 1) [5,6,13].…”

“…This increased echogenicity is thought to result from excessive lung fluid in the alveoli, and negative intra-thoracic pressure postnatally that results in airway collapse and air trapping leading to the manifestation of CLE [4,6]. Thus far, 3 cases of cystic lesions have been reported (Table 1) [5,6,13]. Antenatal imaging with sonography has shown polyhydramnios and fetal hydrops due to disturbed fetal circulation and impaired swallowing.…”

“…Congenital lobar emphysema (CLE) is a rare developmental anomaly of the lung, characterized by air trapping and overdistension of segments and lobes [4,5]. Prenatal diagnosis of CLE has been rarely reported in the literature and only histology later can confirm the presence of CLE [4,6].…”