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Case Reports
. 2017 Nov 16;4(2):20170054.
doi: 10.1259/bjrcr.20170054. eCollection 2018.

Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature

Romeu Duarte Mesquita  1 , Marta Sousa  1 , Filipa Vilaverde  2 , Rosa Cardoso  1
Affiliations
Case Reports

Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature

Romeu Duarte Mesquita et al. BJR Case Rep. .

Abstract

The Abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Although congenital extrahepatic portosystemic shunts are increasingly suspected and diagnosed in specialized centres, much of their clinical presentation and natural history is not fully understood. Symptoms of portosystemic shunt are mainly caused by increased levels of ammonia, which lead to signs of encephalopathy. Therapeutic options depend on the type of shunt and its clinical course, so the classification of the congenital portosystemic shunt is a key finding in these patients.

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Figures

Figure 1.
Figure 1.
Abdominal ultrasound revealed a normal-sized liver, with a diffusely heterogeneous echostructure suggesting chronic liver disease (a and b). On CT (c and d) the liver is slightly dysmorphic. There is a nodular liver lesion in the right lobe, with CT enhancement pattern in the arterial (c) and portal (d) phases suggesting a benign vascular shunt (arrow in b), which is stable on comparing with multiple previous examinations (not shown).
Figure 2.
Figure 2.
Contrast-enhanced abdominal CT scan. Coronal images showed the presence of an abnormal short portal vein, with a systemic drainage in the inferior cava vein (a), and the superior mesenteric vein and splenic vein draining in the abnormal portal vein (b and c), as depicted by the arrows.
Figure 3.
Figure 3.
Multiplanar curved reformatted CT images better depicted the short abnormal portal vein draining in the inferior vena cava (a and b).
Figure 4.
Figure 4.
Axial CT images acquired during the arterial phase show a dilated (circle in a) and tortuous hepatic artery (arrows in b).
Figure 5.
Figure 5.
MR images show the presence of the shunt with the inferior vena cava (arrows in b), and abnormal portal vein (circle in a).
See this image and copyright information in PMC

References

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