Congenital hepatic cyst: Prenatal and postnatal imaging findings

Abstract

Introduction: Congenital hepatic cyst is a rare hepatobiliary malformation that can present as an asymptomatic, unilocular, upper abdominal cystic mass in the fetus.

Cases: We report two cases of congenital hepatic cyst in which the diagnosis was made by prenatal ultrasound at 25 and 33 weeks' gestation. The diagnosis was confirmed postnatally by abdominal ultrasound and radiologic imaging studies. Although the infants remained asymptomatic, laparoscopic excision was performed due to the increasing size of the cyst in both cases. Pathological examination of the resected specimens confirmed a simple cyst in one case and an epidermoid cyst in the other.

Conclusions: Our cases and those described in the literature demonstrate the usefulness of incidental prenatal detection of congenital hepatic cyst, especially during late pregnancy. Such a diagnosis can allow for proper perinatal surveillance, selection of the route of delivery, and timely postnatal surgical intervention if required.

Keywords: Congenital anomalies; fetal ultrasound; hepatic cyst; prenatal diagnosis.

Figure 1.

Case 1. Axial (a) and parasagittal (b) ultrasound views of the fetal abdomen at 34 weeks’ gestation show a 44 × 43 × 31 mm cystic mass (asterisk) between the stomach (st) and the umbilical vein (uv). Note that the cyst is in close contact with the fetal anterior abdominal wall and displaces, but does not compress, the umbilical vein. (c) Postnatal abdominal ultrasound shows a hepatic cyst (between calipers) measuring 51 × 30 mm. L, liver.

Figure 2.

Case 1. Postnatal axial (a) and coronal (b) contrast-enhanced abdominal computed tomography images at six weeks show a hypodense mass (asterisk) within the liver (L), consistent with a nonparasitic simple congenital hepatic cyst. st, stomach.

Figure 3.

Case 1. Laparoscopic view of the hepatic cyst (C) before excision.

Figure 4.

Case 1. Histopathology of the resected specimen revealed a simple cyst. (a) Note cyst lining, composed of a monolayer cubic epithelium, and the surrounded fibrous wall and hepatic tissue (H&E, 10×). (b) Immunohistochemistry staining of the epithelia was positive for CK7 (arrows).

Figure 5.

Case 2. Abdominal ultrasound views of a congenital hepatic cyst. (a) At one day of life, a simple unilocular cyst located in liver and measuring 23 × 19 × 13 mm was confirmed. (b) At 15 months, the hepatic cyst (between calipers) had grown considerably, now measuring 85 × 56 mm. Note internal debris probably representing desquamated epithelial cells.

Figure 6.

Case 2. Preoperative abdominal MRI at 15 months. (a) Coronal and (b) axial T2-weighted images show a hepatic cyst (asterisk). L, liver. (c) Magnetic resonance cholangiography shows the hepatic cyst (asterisk). Note that there is no connection between the cyst and the biliary tree (arrows).

Figure 7.

Case 2. Histopathology of the resected specimen revealed an epidermoid cyst. (a) Note cyst lining composed of a keratinized squamous epithelia (H&E, 40×). (b) Epithelial cells displayed immunoreactivity for CK AE1/AE3, stained as dark brown.