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. 2022 Aug 24;5(1):45.
doi: 10.1186/s42155-022-00320-3.

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

Natalie L Y Ngu  1   2 , Edward Saxby  3 , Caitlin C Farmer  4   5 , Stuart Lyon  4 , Suong Le  3   6
Affiliations

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

Natalie L Y Ngu et al. CVIR Endovasc. .

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.

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Conflict of interest statement

None.

Figures

Fig. 1
Fig. 1
a Axial views of SPSS origin and upper abdominal trajectory. b Sagittal views of SPSS origin and upper abdominal trajectory
Fig. 2
Fig. 2
a Axial views of inferior SPSS. b Sagittal views of inferior SPSS
Fig. 3
Fig. 3
Portoumbilical shunt widest diameter assessment = 23 mm
Fig. 4
Fig. 4
a and b Representative images of shunt diameter measurement technique
Fig. 5
Fig. 5
a-d Intraoperative images with sequential placement of Amplatzer plugs and pushable coils
Fig. 6
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
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
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
See this image and copyright information in PMC

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