Novel variant syndrome associated with congenital hepatic fibrosis

Figure 1 Liver biopsy showing an increased number of abnormal bile ducts, with nodularity of liver parenchyma accentuated by fibrous septa.

Figure 2 Eyes of the first patient.

Figure 3 Mouth of the first patient. This appearance helps us to make a differential diagnosis of Cohen’s syndrome, in which a distinct cheerful facial expression is noted.

Figure 4 Electroretinography of case 3 showing no response in eyes bilaterally. VEP: Visual evoked potantial; ERG: Electroretinography.

Figure 5 Liver showing nodular appearance due to fibrous bands in which elongated and angulated bile ducts are seen.

Figure 6 Liver showing nodular appearance due to fibrous bands in which bile ducts are elongated and periportal ductular proliferation is seen.