PMC7477041_01
Historical classification of CEPS according to Abernethy and the Morgan and Superina scheme. Type I CEPS was defined as an end-to-end communication of the portal system and the IVC, including type Ia (SMV and SV draining separately into the IVC) and type Ib (SMV and SV join to form a common vessels that feeds into the IVC). Type II CEPS was defined as an end-to-side communication of the PV with the IVC.
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F1
CEPS type II portosystemic shunt with a hypoplastic PV (Figures 2A,B). Conventional angiography confirmed the presence of a short, patent shunt and a hypoplastic PV, which was visible without full shunt occlusion (Figure 2C, Videos 1, 2).
Nine years-old male with Abernethy type II portosystemic shunt and hepatic adenoma. CT-angiography demonstrated (A) confluens of the SV, SMV, and IVC (arrow), and (B) a hypoplastic PV (arrow). (C) The shunt (marked by an arrow) and a hypoplastic portal vein (marked by an X) were clearly visualized during a shunt occlusion test (see also Videos 1, 2). (D) MRI imaging confirmed the presence of a large tumor in the liver. (E) Histology was consistent with HA with steatosis (lower part), surrounded by normal liver tissue (upper part). (F) Negative L-FABP staining in HA tissue indicated HNF1-alpha inactivation. Portal tracts contained (G) unpaired arterioles (maked by an arrow) and one bile duct (marked by an X), or (H) two arteriolas (marked by an arrow) and one venule (marked by an X). The size of the HA decreased from (I) a maximum diameter of 11.5 cm at the time of diagnosis to (J) a maximum diameter of 10 cm 4 months after shunt closure.
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F2
CEPS type II portosystemic shunt with a hypoplastic PV (Figures 2A,B). Conventional angiography confirmed the presence of a short, patent shunt and a hypoplastic PV, which was visible without full shunt occlusion (Figure 2C, Videos 1, 2). Figure 2D shows size of the adenoma within the right hepatic lobe. Relevant ductal proliferation was absent (Figure 2E). Portal tracts contained unpaired arterioles and one bile duct, or two arterioles and one venule (Figures 2G,H). This was accompanied by loss of liver fatty acid binding protein (L-FABP) (Figure 2F) in neoplastic cells, while L-FABP expression was preserved in the surrounding non-neoplastic liver parenchyma. Four months after shunt closure, MRI imaging demonstrated a small, patent PV and, notably, a marked decrease in the size of the HA (10 x 8 x 6 cm compared to initial diameters of 11.5 x 10 x 9 cm) (Figures 2I,J, 3).
Graphical depiction of the patient's disease course. The patient first presented with urinary urgency in May 2019. Surgical closure of the shunt was performed in October 2019. By March 2019, the size of the adenoma decreased from initially 11.5 x 10 x 9 cm to 10 x 8 x 6 cm.
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F3
Four months after shunt closure, MRI imaging demonstrated a small, patent PV and, notably, a marked decrease in the size of the HA (10 x 8 x 6 cm compared to initial diameters of 11.5 x 10 x 9 cm) (Figures 2I,J, 3).