case_images: PMC5727568_01
case_images: Abdominal ultrasound demonstrating a present gallbladder.
case_images: CRIS2017-9348461.001.jpg
case_images: PMC5727568_01_CRIS2017-9348461.001.jpg
case_images: fig1
case_images: An abdominal ultrasound had no evidence of choledochal cyst and demonstrated the presence of a gallbladder but was equivocal for presence of the common bile duct (Figure 1).
case_images: HIDA scan without excretion into the intestine.
case_images: CRIS2017-9348461.002.jpg
case_images: PMC5727568_01_CRIS2017-9348461.002.jpg
case_images: fig2
case_images: A hepatobiliary iminodiacetic acid (HIDA) scan was performed to assess for biliary patency, and this failed to demonstrate excretion of the radioactive tracer into the duodenum (Figure 2).
case_images: Intraoperative biopsy demonstrating bile duct proliferation.
case_images: CRIS2017-9348461.003.jpg
case_images: PMC5727568_01_CRIS2017-9348461.003.jpg
case_images: fig3
case_images: Histopathology demonstrated bile duct proliferation (Figure 3), cholestasis (Figure 4), chronic inflammation, and periportal fibrosis without bridging, consistent with the diagnosis of BA; additionally, hepatocytes with PAS-positive, diastase-resistant globules (Figure 5) were seen supporting the additional diagnosis of A1AT.
case_images: Intraoperative biopsy showing cholestasis and bile duct plugging.
case_images: CRIS2017-9348461.004.jpg
case_images: PMC5727568_01_CRIS2017-9348461.004.jpg
case_images: fig4
case_images: Histopathology demonstrated bile duct proliferation (Figure 3), cholestasis (Figure 4), chronic inflammation, and periportal fibrosis without bridging, consistent with the diagnosis of BA; additionally, hepatocytes with PAS-positive, diastase-resistant globules (Figure 5) were seen supporting the additional diagnosis of A1AT.
case_images: Intraoperative biopsy, hepatocytes with PAS-positive, diastase-resistant globules.
case_images: CRIS2017-9348461.005.jpg
case_images: PMC5727568_01_CRIS2017-9348461.005.jpg
case_images: fig5
case_images: Histopathology demonstrated bile duct proliferation (Figure 3), cholestasis (Figure 4), chronic inflammation, and periportal fibrosis without bridging, consistent with the diagnosis of BA; additionally, hepatocytes with PAS-positive, diastase-resistant globules (Figure 5) were seen supporting the additional diagnosis of A1AT.
case_images: PMC5727568_02