Clinical Case Summary

We present the case of a 45-year-old man with medical history of dilated cardiomyopathy and ethanolic chronic pancreatitis complicated by portal cavernoma, who came to the Emergency Department with a history of melena accompanied by lipothymia. On admission, he was hemodynamically unstable, and analytically had hemoglobin of 5.3g/dL, being stabilized after fluid therapy and blood transfusion.
Upper digestive endoscopy identified two bulbar ectopic varices with recent bleeding stigmata. Cyanoacrylate (2cc) was injected triggering active high output hemorrhage. A further 2cc of cyanoacrylate was injected, without success, so it was decided to inject polidocanol (4cc), with resolution of the bleeding, and subsequent application of hemostatic powder. Abdominal CT angiography revealed multiple hyperdense images in the hepatic hilum and duodenal bulb and varices in the cephalic pancreatic region and duodenal arch. Given the high risk of haemorrhagic recurrence the case was discussed with Interventional Radiology team and later at a multidisciplinary hepato-bilio-pancreatic meeting, to enhance the approach. In the absence of the possibility of TIPS or surgical revascularization, given the patient's history and CT angiography findings, partial splenic embolisation was decided. The patient evolved favorably and, on an outpatient basis, embolisation of the splenic artery with coils was performed, showing a reduction in splenic flow on arteriography. Short-term follow-up showed no major complications or haemorrhagic recurrence.
Partial embolisation of the splenic artery is an uncommon procedure for reducing portal hypertension that is described in the literature as an alternative in clinical situations where there is contraindication to TIPS. We present a case of portal hypertension secondary to portal vein thrombosis with ruptured ectopic varices, which was approached in a multidisciplinary manner using an unconventional therapy to successfully treat portal hypertension.

Clinical Case Summary

Clinical presentation: A 45-year-old male with a history of recurrent episodes of variceal bleeding due to decompensated liver cirrhosis of unknown etiology and esophageal variceal ligation (EVL) elsewhere two weeks ago presented to our emergency department with large volume hematemesis and hemodynamic instability.
Therapeutic approach: Immediate resuscitation was offered, with airway intubation and management as per massive bleeding protocol. Somatostatin and antibiotics iv were also administered. Urgent upper gastrointestinal endoscopy was performed once the patient was stabilized, and it revealed active bleeding from a deep, large ulcer at a large esophageal varix just above the gastroesophageal junction. EVL was unsuccessfully attempted twice, due to the disproportionately big size of the variceal ulcer compared to the band device. Emergency transjugular intrahepatic portosystemic shunt (TIPS) and dedicated esophageal stents for varices were not available at the time in our Unit. A decision was made to use an over-the scope clip (OTSC®) as a rescue therapy in this life-threatening condition. OTSC® was successfully applied on variceal ulcer bed and hemostasis was immediately achieved. The patient remained hemodynamically stable after the procedure without clinical signs of further bleeding. He underwent a second endoscopy 48 hours later without endoscopic signs of bleeding or clinically relevant lumen stenosis, while OTSC® was in place. TIPS was planned for the following day.
Discussion: We report the successful use of an over-the-scope clip as a rescue therapy for a patient with massive esophageal variceal bleeding in the absence of established rescue therapies. No clip-related adverse events occurred at the post-treatment period.

References

3 endoscopic images of active variceal bleeding and over-the scope clip placement.

Clinical Case Summary

We present a 27-year-old man with sickle cell disease (SCD), treated with hydroxyurea. Over the previous year, he was hospitalized three times due to vaso-occlusive crisis (VOC). He was referred to Gastroenterology for persistent abnormal liver tests for the last five years, which exhibited a cholestatic pattern and showed further worsening during VOC. AST was 2 times the upper limit of normal (ULN), ALT was normal, alkaline phosphatase (ALP) 4 times ULN, and total bilirubin 3-4 times ULN. After the last VOC, cholestasis worsened with conjugated hyperbilirrubinemia up to 10 times ULN and ALP up to 6-7 times ULN with ALT 2 times ULN and AST 5 times ULN. Coagulation was normal.
Abdominal ultrasound showed hepatomegaly with normal blood flow on doppler study. Abdominal MRI also revealed a heterogeneous liver with regenerative nodules, but no bile duct dilation.
Further investigation ruled out alcohol or drug use. ANA was positive (1:320), IgG was elevated (2230 mg/dL), suggesting polyclonal gammopathy, while other autoimmune markers were negative. Ferritin was elevated with normal transferrin saturation. Urinary copper and ceruloplasmin were increased, likely reflecting an acute-phase response. Viral serologies were negative.
A liver biopsy revealed portal fibrosis with porto-central bridging and significant fibrosis in the spaces of Disse. Dilated sinusoids were obstructed by abnormally shaped erythrocytes. These findings are consistent with chronic hepatitis secondary to chronic sinusoidal obstruction syndrome, likely due to recurrent VOC.
The case was discussed with both his hematologist and a liver transplant centre. He is now awaiting enrollment in a clinical trial for genetic therapy for SCD.
SCD can lead to liver damage through multiple mechanisms, and these complications are often underrecognized. We present this case due to the severity of the chronic liver disease and the rare occurrence of chronic sinusoidal obstruction syndrome associated with SCD.

References

This case will be illustrated with laboratory results, imaging studies, and liver biopsy images.

Introduction

Hepatic encephalopathy (HE) is a group of neuropsychiatric disorders caused by liver dysfunction or portosystemic shunting, and it is a common complication in the progression of liver cirrhosis. Pathophysiologically, there is an imbalance between the hepatic production and clearance of ammonia, leading to an increase in serum ammonium levels, which can lead to neurological dysfunction, intracranial hypertension, cerebral oedema and ultimately death (1). There are multiple triggering factors; one of the most well-known is upper gastrointestinal bleeding (UGIB) (2). Therefore, we suggest that the coexistence of UGIB and HE is associated with an increased risk of in-hospital mortality and prolonged hospital stay. This study aims to analyse the risk factors for HE during an episode of UGIB and its impact on the patient's prognosis.

Aims & Methods

Retrospective analysis of a prospective registry of patients with UGIB treated at the Hospital Universitario Virgen de las Nieves (2013-2022). Statistical analysis was performed using R v4.3.2. Categorical variables were compared using the Chi-square or Fisher's exact test. Odds ratios (OR) and 95% confidence intervals (95%CI) were calculated using the Woolf-Haldane formula for significant variables. Continuous variables were assessed using the Shapiro-Wilk test for normality, and Levene's test was used to check for homogeneity of variances. The t-test for independent samples, with or without Welch's correction, and the Mann-Whitney U test were used for hypothesis testing. Multivariate analysis is performed to identify independent risk factors for variables with statistically significant differences, calculating Odds Ratios (OR) and their 95% confidence intervals (95%CI).

Results

A total of 343 patients with liver cirrhosis and UGIB were analysed, of whom 260 were male and 83 were female, with an overall mean age of 61.51 ± 12.38, developing HE in a total of 66 patients.
After comparing the cirrhotic population that developed HE with those that did not, we observed a series of variables that correlated significantly, as shown in Table 1. Subsequently, after performing multivariate analysis, we noticed that only four variables had a significant independent relationship: bilirubin with an OR (95% CI) of 1.07 (1-1.16) with p0.064; albumin with an OR (95%CI) of 0.41 (0.23-0.7) with p0.0017; ascites with an OR (95%CI) of 1.65(1.01-2.71) with p0.04; and urea on admission with an OR (95% CI) of 1.01 (1-1.102) with p0.0005.

Conclusion

We conclude that patients with cirrhosis admitted for UGIB and who develop HE have higher mortality rates during admission without an apparent increase in hospital stay. Our study indicates that bilirubin, albumin, ascites, and urea are the most accurate predictors of HE development. These findings aligns with Rattanasupar et al. (2), concluding that the Child-Pugh score, which also considers the abovementioned variables, best predicts hepatic encephalopathy after UGIB. Further research is necessary to explore additional risk factors that aid in predicting and modelling the disease based on our findings.

References

1. Elsaid MI, Rustgi VK. Epidemiology of Hepatic Encephalopathy. Clinics in Liver Disease. 2020 May;24(2):157–74.
2. Rattanasupar A, Tiawijit N, Rachatapantanakorn B. Predictive factor for hepatic encephalopathy in cirrhotic patients who presented with acute variceal bleeding. J Med Assoc Thai. 2014 Jun;97(6):567–73.