Unique Case of Congenital Duodenal Atresia and a Choledochal Cyst and the Hypothesis of Their Embryological Evolution

Abstract

The concomitant occurrence of duodenal atresia (DA) and a choledochal cyst (CC) has rarely been reported. Knowledge of both the presentation and management of this rare co-occurrence is imperative in avoiding potential complications and sequelae, such as biliary metaplasia. Herein we describe a female infant born at 32 weeks gestational age who was diagnosed with duodenal atresia and annular pancreas postnatally, who had subsequent findings of malrotation and a choledochal cyst, as seen from contrast imaging. Uncomplicated repair of the DA and obstruction was performed at 4 days of life. She re-presented 2 years later with non-bloody, nonbilious emesis and was found to have elevated amylase, lipase and liver enzymes. Imaging revealed dilated intra-hepatic ducts, a distended gallbladder and a large choledochal cyst. She underwent a cholecystostomy tube placement followed by a definitive choledochal cyst excision with immediate improvement following surgery and full resolution of symptoms before discharge.

Keywords: cholecystectomy; choledochal cyst; duodenal atresia; embryology; metaplasia.

Figure 1

Plain abdominal radiograph taken soon after birth, showing a double-bubble in the lower abdomen.

Figure 2

Upper GI contrast study on day-of-life 2, demonstrating the duodenal obstruction with reflux into the dilated biliary tree and a choledochal cyst approximately 14 × 10 mm.

Figure 3

Ultrasound scan of the abdomen, revealing a choledochal cyst with dilatation of the intrahepatic biliary system.

Figure 4

Intra-operative cholangiogram performed at two years of age, demonstrating a persistently dilated biliary tree with a revisualization of the choledochal cyst approximately 59 × 37 mm.