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

doi:10.21203/rs.3.rs-1620209/v1

Download PDF

Research Article

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

https://doi.org/10.21203/rs.3.rs-1620209/v1

This work is licensed under a CC BY 4.0 License

You are reading this latest preprint version

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 hospitalisation 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 hospitalisation 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.

Portal hypertension

portosystemic shunt

hepatic encephalopathy

retrograde transvenous obliteration

angiography

Hepatic encephalopathy (HE) is defined as “brain dysfunction caused by liver insufficiency and/or portosystemic shunts” and occurs in 30–45% with cirrhosis(1). It can have 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(2). Spontaneous portosystemic shunts (SPSS) are a common consequence of portal hypertension, with increasing size correlating with liver disease severity, risk of HE and mortality(3).

Angiographic retrograde transvenous obliteration (ARTO), with or without balloon-occlusion has been developed for treatment of both gastric varices(4) and refractory HE(5) by impeding collateral flow through shunts. Alternative methods of venous obliteration include plugs, coils and sclerosing agents. ARTO for refractory HE has found to be safe and effective for at least 4–12 months in patients with cirrhosis failing medical management(5, 6).

A 53-year-old man with cirrhosis and ongoing alcohol excess presented to our tertiary hospital with decreased conscious state clinically consistent with Westhaven Grade 2–3 HE, which improved but did not resolve following treatment with lactulose, rifaximin and nutritional supplementation. Investigations for other causes for the altered conscious state including brain computed tomography (CT), blood glucose levels, and biochemistry were unremarkable, and there was no history of sedating medication use or active infection. Investigations for an underlying precipitant included multiphase abdominal computed tomography (CT), which demonstrated extensive anterior abdominal wall varices. The patient had three further admissions for HE over the next four months, was unable to work, and his partner experienced significant carer stress. A large, portal-umbilical shunt was subsequently identified by an experienced interventional radiologist (Figs. 1 & 2a). The large paraumbilical vein, reflecting recanalization of the ligamentum teres, can be seen draining inferiorly into engorged pelvic veins in coronal and axial views. The SPSS was 23mm at widest diameter and had a total cross-sectional area of 809mm² using Baveno VI-SPSS Group criteria(7), calculated by summing the maximal cross-sectional areas of all connected vessels greater than 6mm diameter.

The patient subsequently underwent an ARTO. The umbilical vein was accessed under ultrasound guidance with introduction of an 8 French sheath through which satisfactory occlusion of the portal-to-venous umbilical shunt was achieved using 12mm type 2 and 3, and 14mm type 2 Amplatzer plugs; 15mm, 12mm and 10mm pushable coils and Fibrovein sclerosant (Fig. 2b & 2c). There were no periprocedural complications. Follow-up ultrasound at 7 days confirmed successful SPSS occlusion. A further CT was performed at 6 weeks, which confirmed eradication of the likely culprit SPSS and presence of smaller, portal system collateral vessels reflecting the extent of the patient’s underlying portal hypertension (Fig. 2d).

Neurocognitive testing was conducted at baseline and at two weeks post-ARTO (Figs. 3 & 4). Baseline tests demonstrated impairment including delayed time to completion of the Number Connection Test at 62 seconds and an improvement to 55 seconds at 2 week follow up. The baseline clockface drawing demonstrated incomplete numbers and inaccurate hand placement with subsequent improvement at 2 weeks. This improvement correlated with absence of emergency hospital admissions in the three months post-ARTO.

This case report did not require institutional ethics committee approval. Written informed consent was obtained from the patient for publication of this case report and any accompanying media.

Our patient with refractory HE and a large, occult SPSS successfully underwent ARTO with benefits persisting both radiologically and clinically at 6 weeks. This case is unique in several aspects including a) the size of the SPSS at 10x the threshold for risk of HE and death, b) difficulty in identification of the SPSS, and c) documented clinical and cognitive improvement following angiographic intervention.

At 809mm², the SPSS identified in this case is far greater than the threshold identified by Praktiknjo et al, who additionally found that a total surface area of greater than 83mm² was associated with higher model for end-stage liver disease score, history of overt HE, and lower 1-year survival compared to smaller SPSS(7). In addition to surface area, a diameter threshold of 8mm(3) compared to 23mm in our patient, as well as total number of SPSS, are both associated with presence of overt HE.

SPSS are present in 30–60% of those with cirrhosis(3). Common sites include gastrorenal, splenorenal, paraumbilical, oesophageal SPSS and additional complications include variceal bleeding, portal vein thrombosis and deterioration in liver disease(8). Most SPSS can be visualised on CT, as demonstrated in a study of 222 patients with cirrhosis who underwent CT abdomen with portal venous phase contrast, with an SPSS identified in 63.5% by two experienced radiologists(9). As demonstrated in our case, diagnosis can be challenging yet identification can have profound implications if angiographic intervention is feasible.

Balloon-occluded retrograde transvenous has long been used for treatment of gastric varices following haemorrhage and is being increasingly used for management of HE with large SPSS. Previous studies have used ammonia levels(5), presence/grade of HE, and hospitalisation(10) as markers of success. We used two forms of neurocognitive testing to contrast the patient’s ability pre- and post-ARTO. The Number Connection Test is a widely used assessment of visuospatial orientation and psychomotor speed, and is a component of the Psychometric Hepatic Encephalopathy Score(11). The patient was shown a sheet of paper with 15 numbered circles randomly spread across the paper and asked to draw a line connecting the circles in order from 1–15. A healthy subject should be able to complete the task within 30 seconds. Although there was an improvement in our patient from 62 seconds to 55 seconds, the persistent impairment may reflect minimal HE or another cognitive impairment. The baseline clockface drawing demonstrated constructional apraxia and conceptional deficits (incomplete numbers, inaccurate hand placement) associated with a positive predictive value of 0.96 for HE in patients with cirrhosis(12), with subsequent improvement in visuospatial construction. Additionally, the reduction in emergency hospital admissions in the three months following ARTO may reflect an improvement in functional status.

ARTO is well-tolerated, safe, and effective for refractory HE in the context of a spontaneous portosystemic shunt. Such shunts can be challenging to diagnose despite the profound impact on quality of life and need for hospitalisation. Application of Baveno VI-SPSS criteria to estimate cross-sectional area can assist in identifying shunts likely to contribute to complications including HE and death. Successful obliteration of a culprit SPSS and reduction in HE may be reflected by improvement in neurocognitive testing and reduction in emergency hospitalisation.

ARTO: Angiographic, retrograde transvenous obliteration.

CT: Computed tomography

HE: Hepatic encephalopathy

SPSS: Spontaneous portosystemic shunt

Ethics approval and consent to participate: This case report did not require institutional ethics committee approval.

Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying media.

Availability of data and material: Not applicable.

Competing interests: None

Funding: None

Authors’ contributions

Study concept and design: NN, ES, CF, SL, SLe

Cognitive testing: ES

Interventional radiology procedure: CF, SL

Image and video acquisition: CF, SL, ES

Drafting and revision of manuscript: NN, ES, CF, SL, SLe

Acknowledgements: Not applicable

Hepatic Encephalopathy in Chronic Liver Disease (2014) 2014 Practice Guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases. J Hepatol 61(3):642–659
D'Amico G, Morabito A, Pagliaro L, Marubini E (1986) Survival and prognostic indicators in compensated and decompensated cirrhosis. Dig Dis Sci 31(5):468–475
Simón-Talero M, Roccarina D, Martínez J, Lampichler K, Baiges A, Low G et al (2018) Association Between Portosystemic Shunts and Increased Complications and Mortality in Patients With Cirrhosis. Gastroenterology 154(6):1694–705e4
Kumamoto M, Toyonaga A, Inoue H, Miyakoda K, Morita Y, Emori K et al (2010) Long-term results of balloon-occluded retrograde transvenous obliteration for gastric fundal varices: hepatic deterioration links to portosystemic shunt syndrome. J Gastroenterol Hepatol 25(6):1129–1135
Mukund A, Rajesh S, Arora A, Patidar Y, Jain D, Sarin SK (2012) Efficacy of balloon-occluded retrograde transvenous obliteration of large spontaneous lienorenal shunt in patients with severe recurrent hepatic encephalopathy with foam sclerotherapy: initial experience. J Vasc Interv Radiol 23(9):1200–1206
Lynn AM, Singh S, Congly SE, Khemani D, Johnson DH, Wiesner RH et al (2016) Embolization of portosystemic shunts for treatment of medically refractory hepatic encephalopathy. Liver Transpl 22(6):723–731
Praktiknjo M, Simón-Talero M, Römer J, Roccarina D, Martínez J, Lampichler K et al (2020) Total area of spontaneous portosystemic shunts independently predicts hepatic encephalopathy and mortality in liver cirrhosis. J Hepatol 72(6):1140–1150
Nardelli S, Riggio O, Gioia S, Puzzono M, Pelle G, Ridola L (2020) Spontaneous porto-systemic shunts in liver cirrhosis: Clinical and therapeutical aspects. World J Gastroenterol 26(15):1726–1732
Nardelli S, Riggio O, Turco L, Gioia S, Puzzono M, Bianchini M et al (2021) Relevance of Spontaneous Portosystemic Shunts Detected with CT in Patients with Cirrhosis. Radiology 299(1):133–140
Laleman W, Simon-Talero M, Maleux G, Perez M, Ameloot K, Soriano G et al (2013) Embolization of large spontaneous portosystemic shunts for refractory hepatic encephalopathy: a multicenter survey on safety and efficacy. Hepatology 57(6):2448–2457
Weissenborn K (2015) Diagnosis of minimal hepatic encephalopathy. J Clin Exp Hepatol 5(Suppl 1):S54–S59
Edwin N, Peter JV, John G, Eapen CE, Graham PL (2011) Relationship between clock and star drawing and the degree of hepatic encephalopathy. Postgrad Med J 87(1031):605–611

Download PDF

Reviewers agreed at journal
19 May, 2022
Reviewers invited by journal
09 May, 2022
Editor assigned by journal
06 May, 2022
First submitted to journal
03 May, 2022

You are reading this latest preprint version

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

Status:

Version 1

Abstract

Figures

Background

Case presentation

Discussion

Conclusion

Abbreviations

Declarations

References

Status:

Version 1