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

Our patient with refractory HE and a large, occult SPSS successfully underwent ARTO with benefits persisting both radiologically and clinically at six weeks. This case is unique in several aspects including a) the size of the SPSS at 10 × the threshold for risk of HE and death, b) initial delay in identification of the clinical significance of the SPSS to this patient’s presentations, and c) documented clinical and cognitive improvement following angiographic intervention.

At 809mm², the SPSS identified in this case is far greater than the threshold for adverse events identified by Praktiknjo et al., who additionally found that a total surface area of greater than 83mm²was associated with higher model for end-stage liver disease score, history of overt HE, and lower 1-year survival compared to smaller SPSS (Praktiknjo et al. 2020). In addition to surface area, a diameter threshold of 8 mm (Simón-Talero et al. 2018) compared to 23 mm 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 (Simón-Talero et al. 2018). Common sites include gastrorenal, splenorenal, paraumbilical, esophageal SPSS and additional complications include variceal bleeding, portal vein thrombosis and deterioration in liver disease (Nardelli et al. 2020). Most SPSS can be visualized 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 (Nardelli et al. 2021). As demonstrated in our case, diagnosis and its clinical implications can have profound impact if angiographic intervention is feasible, demonstrating clear benefits for multidisciplinary collaboration between physician and interventional radiologist clinicians in diagnosis and management planning.

Balloon-occluded retrograde transvenous obliteration and similar approaches have long been used for treatment of gastric varices following hemorrhage and is being increasingly used for management of HE with large SPSS. Previous studies have used ammonia levels (Mukund et al. 2012), presence/grade of HE, and hospitalization (Laleman et al. 2013)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 (Weissenborn 2015). 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 s. Although there was an improvement in our patient from 62 to 55 s, 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 (Edwin et al. 2011), with subsequent improvement in visuospatial construction. The reduction in emergency hospital admissions in the three months following ARTO may have multiple contributing factors in addition to an improvement in HE, including reduction in alcohol intake, greater healthcare engagement, and improvement in nutrition. The reduction in emergency hospital admissions in the three months following ARTO may have multiple contributing factors in addition to an improvement in HE, including reduction in alcohol intake, greater healthcare engagement, and improvement in nutrition. Collateral history obtained from the patient’s wife and treating clinicians, suggested that there was a subjective improvement in mood, which is likely interlinked with the other contributing factors. This case demonstrates that both the direct and indirect consequences of accurate identification and treatment of refractory HE impacts not only the patient but their carers and support people.