Congenital Extrahepatic Portosystemic Shunt in a Child With Caroli’s Syndrome

A 7-year-old boy presented with recurrent cholangitis, failure to thrive, and progressive splenomegaly. On examination, he had severe pallor, clubbing, and splenohepatomegaly. Lab results showed anemia (hemoglobin 7.2 g/dL) and normal liver functions. Abdominal ultrasound showed hepatosplenomegaly with coarsened liver echotexture and multifocal intrahepatic biliary radical (IHBR) dilatation. The main portal vein was markedly attenuated beyond the splenomesenteric confluence. Bilateral kidneys were enlarged with indistinct corticomedullary junction. Contrast-enhanced computed tomography demonstrated right liver lobe atrophy with compensatory left lobe hypertrophy. There were skip areas of bilobar saccular or tubular IHBR dilatation, with a central dot sign, typical of Caroli’s disease (Figure 1). Coronal maximum intensity projections showed an atretic main portal vein and its intrahepatic branches with a prominent and tortuous portosystemic (lienorenal) shunt draining portal blood into systemic circulation (Figure 2), indicative of congenital extrahepatic portosystemic shunt (CEPS, Type 2). There was asymmetric enlargement of the kidney with renal tubular ectasia and multiple renal calculi. Upper gastrointestinal endoscopy showed large esophageal varices with red color signs. The patient required 5 sessions of variceal ligation for eradication. Liver