Functional recovery from refractory hepatic encephalopathy following angiographic obliteration of a large, spontaneous portal-umbilical portosystemic shunt: a case report

Abstract

Background: Hepatic encephalopathy (HE) as a consequence of cirrhosis with portal hypertension has a profound impact on quality of life for both patients and caregivers, has no gold-standard diagnostic test, and is a risk factor for mortality. Spontaneous portosystemic shunts (SPSS) are common in patients with cirrhosis, can be challenging to identify, and in some cases, can drive refractory HE. Cross-sectional shunt size greater than 83mm² is associated with liver disease severity, overt HE, and mortality.

Case presentation: We report a patient with refractory HE and frequent hospitalization in the context of an occult spontaneous portal-umbilical portosystemic shunt with an estimated cross-sectional area of 809mm². Following identification and angiographic retrograde transvenous obliteration of the SPSS using plugs, coils and sclerosant, there was improvement in neurocognitive testing and no further hospitalization for HE.

Conclusion: SPSS in the context of cirrhosis with portal hypertension can contribute to the debilitating effects of refractory HE. This case highlights the opportunity to search for SPSS in patients with HE unresponsive to therapy as angiographic obliteration is usually safe, well-tolerated, and clinically effective.

Keywords: Angiography; Hepatic encephalopathy; Portal hypertension; Portosystemic shunt; Retrograde transvenous obliteration.

Fig. 1

a Axial views of SPSS origin and upper abdominal trajectory. b Sagittal views of SPSS origin and upper abdominal trajectory

Fig. 2

a Axial views of inferior SPSS. b Sagittal views of inferior SPSS

Fig. 3

Portoumbilical shunt widest diameter assessment = 23 mm

Fig. 4

a and b Representative images of shunt diameter measurement technique

Fig. 5

a-d Intraoperative images with sequential placement of Amplatzer plugs and pushable coils

Fig. 6

a Axial images demonstrating absence of the previously seen SPSS with embolization material in situ. b Sagittal images demonstrating absence of the previously seen SPSS with embolization material in situ

Fig. 7

Number Connection Test a) Prior to ARTO (62 s to complete) and b) At 2 weeks from ARTO (taking 55 s to complete)

Fig. 8

Clockface drawing with patient instructed to draw the hands at “10 to 2” a) Prior to ARTO and b) At 2 weeks from ARTO