A case of esophageal carcinoma on esophageal varices treated by endoscopic submucosal dissection after endoscopic injection sclerotherapy.

Esophageal varices (EVs) are often treated using endoscopic injection sclerotherapy. Endoscopic submucosal dissection (ESD) has been used for early esophageal epithelial neoplasia worldwide. We report a case of early esophageal squamous cell carcinoma (ESCC) that occurred over EVs, in which the EVs were treated with endoscopic injection sclerotherapy before the early ESCC was treated with endoscopic submucosal dissection. Argon plasma coagulation was finally performed to prevent the recurrence of varices. No serious complications, such as severe bleeding or perforation, were observed. Histopathological examination revealed submucosal veins occluded with an organized thrombus for which endoscopic injection sclerotherapy with an intravariceal injection of sclerosant had been performed, but no fibrosis was observed outside the blood vessels. This explains that the injected sclerosant into EVs did not cause any tissue reaction like fibrosis in the submucosa surrounding the vein, which may have made endoscopic submucosal dissection safer and easier. Varices have not recurred, and ESCC has also not recurred for 5 years. We demonstrated a successful treatment of ESCC on EVs and no submucosal fibrosis other than inside the occluded vessels and verified it histologically.

Validation of PH and Varices Risk Scores for Prediction of High-Risk Esophageal Varix and Bleeding in Patients with B-Viral Cirrhosis.

Esophageal varices (EVs) can be accurately predicted using PH and varices risk scores. We aimed to validate their prognostic performances. Methods: We enrolled patients with B-viral cirrhosis as the training cohort (n = 503). Areas under receiver operating characteristic curves (AUROCs) for HEV were calculated for PH (=-5.953 + 0.188 × liver stiffness (LS) + 1.583 × sex (1:male/0:female) + 26.705 × spleen diameter/platelet count ratio) and varices (=-4.364 + 0.538 × spleen diameter -0.049 × platelet count -0.044 × LS + 0.001 × LS × platelet count) risk scores, and compared to LSPS (=LS × spleen diameter/platelet count). An independent cohort was recruited for further validation (n = 222). In the training cohort, the varices risk score showed the highest AUROC (0.926), followed by the PH risk score (0.924) and LSPS (0.924), but without any statistically significant differences. For varices risk scores ≤-1.70 and ≥1.48, a 95.0% negative predictive value (NPV) and 91.2% positive predictive value (PPV) were observed, respectively. At PH risk scores ≤2.25 and ≥7.71, 95.0% NPV and 90.0% PPV were observed, respectively. At LSPS ≤1.73 and ≥13.9, 95.3% NPV and 95.0% PPV were observed, respectively. The EV bleeding (EVB) risk during follow-up increased stepwise and significantly when stratified by PH, varices risk scores, and LSPS (all p < 0.001). In the validation cohort, NPVs were generally similar when stratified by PH (88.2%), varices risk scores (93.2%), and LSPS (88.9%); however, corresponding PPVs were suboptimal. PH and variceal risk scores are reliable for predicting HEV and future EVB. Patients with PH and varices risk scores ≤2.25 and ≤-1.70, respectively, may avoid endoscopy safely. For convenience, LSPS might be a good alternative, with comparable prognostic performance to these two models.

The longitudinal outcomes of applying non-selective beta-blockers in portal hypertension: real-world multicenter study.

BACKGROUND/AIM: We investigated the effect of non-selective ß-blockers (NSBB) in real-world situations and whether low-dose NSBB is beneficial compared to maximally tolerated doses. METHODS: We performed a retrospective study of 740 patients with cirrhosis requiring prophylactic treatment of esophageal varices: 473 primary prophylaxis (PP: NSBB = 349, non-NSBB = 124) and 267 secondary prophylaxis (SP: NSBB = 200, non-NSBB = 67). The NSBB group was divided into low-dose (≤ 80 mg/day) and high-dose (> 80 mg/day). RESULTS: In the PP group, NSBB treatment reduced mortality and showed the most pronounced effect in patients with moderate/severe ascites (hazard ratio [HR], 0.46; p < 0.01), HVPG ≥ 16 mmHg (HR, 0.53; p = 0.04), or CTP class B/C (HR, 0.46; p < 0.01) but not in those with no/mild ascites, HVPG < 16 mmHg, or CTP class A. Low-dose NSBB group showed a significant reduction in mortality compared with non-NSBB (moderate/severe ascites: HR, 0.61; p = 0.02 and CTP class B/C: HR, 0.41; p < 0.01) and the effect size was stronger than the high-dose NSBB. NSBB was associated with a reduced risk of infection (HR, 0.36; p = 0.01). In the SP group, NSBB prolonged survival in patients with moderate/severe ascites (HR, 0.56; p = 0.02), HVPG ≥ 16 mmHg (HR, 0.42; p < 0.01), or CTP class B/C (HR, 0.52; p < 0.01). Low-dose NSBB was more beneficial with 56% risk reduction (p < 0.01) of mortality compared with 33% risk reduction in the high-dose NSBB (p = 0.05). CONCLUSION: NSBB therapy was associated with longer survival in PP and SP groups who had an advanced stage of cirrhosis. Moreover, low-dose NSBB exhibited a better benefit than a standard-titrated high-dose NSBB with better tolerability.

Validating the BAVENO VI criteria to identify low risk biliary atresia patients without endoscopy for esophageal varix.

BACKGROUND AND AIMS: Portoenterostomy is the initial surgical treatment for biliary atresia (BA); however, no curative therapy exists for BA. Varix bleeding is a major complication of end-stage liver disease and must be determined in patients with BA, necessitating routine surveillance using esophagogastroduodenoscopy (EGD). We attempted to validate criteria to identify BA patients requiring EGD. METHODS: From January 2007 to December 2017, we selected BA patients who underwent Kasai surgery, transient elastography (TE), and EGD at Severance hospital. In total, 190 cases were included; laboratory tests and EGDs were carried out from 3 months before TE to 3 months after TE. RESULTS: Based on the cut-off value (<10) of the liver stiffness measurement (LSM), 35 (81.4%) patients with low-risk varix (LRV) and 8 (18.6%) with high-risk varix (HRV) were identified. Based on platelet counts (>150,000), 87 (77.68%) patients with LRV and 25 (22.32%) with HRV were identified. Based on this, the BAVENO VI criteria, which identify patients who can safely avoid screening EGD, missed 9/68 (13.24%) of HRV patients. The expanded BAVENO VI criteria missed 21/68 (30.88%) of HRV patients. However, the criteria using LSM <10 and platelet count >150,000 missed identifying only 4/68 (5.88%) HRV patients. CONCLUSIONS: The BAVENO criteria may be as useful in children with BA as in adults with liver cirrhosis. Regular laboratory tests, imaging studies, and EGD may avoid missing diagnoses of varices in BA patients. However, LSM<10 and platelet count>150,000 may provide more accurate criteria and help identify patients who does not need endoscopy.

Removal of a Trigger Cord Stuck between Bands during Endoscopic Multiple-Band Ligation for Treating Esophageal Variceal Hemorrhage.

Endoscopic variceal ligation is the preferred endoscopic treatment method for esophageal variceal bleeding. The incidence of complications such as chest pain, bleeding, stricture formation, and aspiration pneumonia is low. We report a case wherein a malfunctioning multiple-band ligator could have potentially caused damage to the esophageal varices and massive bleeding. The equipment was safely removed using scissors and forceps. To the best of our knowledge, this is the first published report detailing the management of a case of esophageal variceal bleeding.

Multicenter Retrospective Risk Assessment of Esophageal Variceal Bleeding in Patients with Cirrhosis: An Acoustic Radiation Force Impulse Elastography-Based Prediction Model.

Background/Aims: Acoustic radiation force impulse (ARFI) elastography predicts the presence of esophageal varices (EVs). We investigated whether an ARFI-based prediction model can assess EV bleeding (EVB) risk in patients with cirrhosis. Methods: The records of 262 patients with cirrhosis who underwent ARFI elastography and endoscopic surveillance at two institutions in 2008 to 2013 were retrospectively reviewed, and ARFI-spleen diameter-to-platelet ratio scores (ASPS) were calculated. Results: The median patient age (165 men, 97 women) was 56 years. The median ARFI velocity, spleen diameter, platelet count, and ASPS were 1.7 m/sec, 10.1 cm, 145×109/L, and 1.16, respectively. During the median 38-month follow-up, 61 patients experienced EVB. Among all patients (179 without EVs and 83 with EVs), the cutoff value that maximized the sum of the sensitivity (73.1%) and specificity (78.4%) (area under receiver operating characteristic curve [AUROC], 0.824) for predicting EVB was 2.60. The cumulative EVB incidence was significantly higher in patients with ASPS ≥2.60 than in those with ASPS <2.60 (p<0.001). Among patients with EVs (n=83), 49 had high-risk EVs (HEVs), and 22 had EVB. The cumulative EVB incidence was significantly higher in HEV patients than in low-risk EV patients (p=0.037). At an ASPS of 4.50 (sensitivity, 66.7%; specificity, 70.6%; AUROC, 0.691), the cumulative EVB incidence was significantly higher in patients with a high ASPS than in those with a low ASPS (p=0.045). A higher ASPS independently predicted EVB (hazard ratio, 4.072; p=0.047). Conclusions: ASPS can assess EVB risk in patients with cirrhosis. Prophylactic management should be considered for patients with HEVs and ASPS ≥4.50.

Multicenter Retrospective Risk Assessment of Esophageal Variceal Bleeding in Patients with Cirrhosis: An Acoustic Radiation Force Impulse Elastography-Based Prediction Model

BACKGROUND/AIMS: Acoustic radiation force impulse (ARFI) elastography predicts the presence of esophageal varices (EVs). We investigated whether an ARFI-based prediction model can assess EV bleeding (EVB) risk in patients with cirrhosis. METHODS: The records of 262 patients with cirrhosis who underwent ARFI elastography and endoscopic surveillance at two institutions in 2008 to 2013 were retrospectively reviewed, and ARFI spleen diameter-to-platelet ratio scores (ASPS) were calculated. RESULTS: The median patient age (165 men, 97 women) was 56 years. The median ARFI velocity, spleen diameter, platelet count, and ASPS were 1.7 m/sec, 10.1 cm, 145×10⁹/L, and 1.16, respectively. During the median 38-month follow-up, 61 patients experienced EVB. Among all patients (179 without EVs and 83 with EVs), the cutoff value that maximized the sum of the sensitivity (73.1%) and specificity (78.4%) (area under receiver operating characteristic curve [AUROC], 0.824) for predicting EVB was 2.60. The cumulative EVB incidence was significantly higher in patients with ASPS ≥2.60 than in those with ASPS <2.60 (p<0.001). Among patients with EVs (n=83), 49 had high-risk EVs (HEVs), and 22 had EVB. The cumulative EVB incidence was significantly higher in HEV patients than in low-risk EV patients (p=0.037). At an ASPS of 4.50 (sensitivity, 66.7%; specificity, 70.6%; AUROC, 0.691), the cumulative EVB incidence was significantly higher in patients with a high ASPS than in those with a low ASPS (p=0.045). A higher ASPS independently predicted EVB (hazard ratio, 4.072; p=0.047). CONCLUSIONS: ASPS can assess EVB risk in patients with cirrhosis. Prophylactic management should be considered for patients with HEVs and ASPS ≥4.50.

Left Gastric Vein Visualization with Hepatopetal Flow Information in Healthy Subjects Using Non-Contrast-Enhanced Magnetic Resonance Angiography with Balanced Steady-State Free-Precession Sequence and Time-Spatial Labeling Inversion Pulse.

Objective: To selectively visualize the left gastric vein (LGV) with hepatopetal flow information by non-contrast-enhanced magnetic resonance angiography under a hypothesis that change in the LGV flow direction can predict the development of esophageal varices; and to optimize the acquisition protocol in healthy subjects. Materials and Methods: Respiratory-gated three-dimensional balanced steady-state free-precession scans were conducted on 31 healthy subjects using two methods (A and B) for visualizing the LGV with hepatopetal flow. In method A, two time-spatial labeling inversion pulses (Time-SLIP) were placed on the whole abdomen and the area from the gastric fornix to the upper body, excluding the LGV area. In method B, nonselective inversion recovery pulse was used and one Time-SLIP was placed on the esophagogastric junction. The detectability and consistency of LGV were evaluated using the two methods and ultrasonography (US). Results: Left gastric veins by method A, B, and US were detected in 30 (97%), 24 (77%), and 23 (74%) subjects, respectively. LGV flow by US was hepatopetal in 22 subjects and stagnant in one subject. All hepatopetal LGVs by US coincided with the visualized vessels in both methods. One subject with non-visualized LGV in method A showed stagnant LGV by US. Conclusion: Hepatopetal LGV could be selectively visualized by method A in healthy subjects.

Direct reopening of the occluded hepatic veins of Budd-Chiari syndrome: verification of our operative method by the perioperative course of esophageal varices.

OBJECTIVE: A total of 69 patients with Budd-Chiari syndrome (BCS) were operated by direct approach under cardiopulmonary bypass (CPB). To assess the operative procedure, the perioperative course of esophageal varices (EVs) was evaluated. PATIENTS AND METHODS: Of the 69 patients, 59 (22 females) were enrolled in this study because they had complete follow-up data for endoscopic evaluation of EVs. Their mean age was 46.3 ± 13.0 years (range 21-73.3 years). EVs were found in 52 patients. Under partial cardiopulmonary bypass, the inferior vena cava (IVC) was incised. The obstruction of the IVC was excised, and the occluded hepatic veins were reopened. The incised IVC was reconstructed with an auto-pericardial patch. RESULTS: Postoperatively, the repaired IVC was patent in all patients. The average number of patent hepatic veins (HVs) increased from 1.23 ± 0.81 to 2.21 ± 0.97/patient. The pressure gradient between the IVC and right atrium (RA) decreased from 12.4 ± 5.52 to 4.46 ± 3.21 mmHg. The indocyanine green clearance test (ICG) at 15 min decreased from 31.57 ± 17.44 to 22.27 ± 15.23%. EVs had disappeared in 13 patients at discharge and in 6 patients at late postoperative follow-up. CONCLUSION: Our operative procedure for BCS is useful for decreasing portal pressure, which is reflected by disappearance of EVs. Therefore, the high risk of EV rupture could be avoided by reopening the occluded HVs.

Left Gastric Vein Visualization with Hepatopetal Flow Information in Healthy Subjects Using Non-Contrast-Enhanced Magnetic Resonance Angiography with Balanced Steady-State Free-Precession Sequence and Time-Spatial Labeling Inversion Pulse

OBJECTIVE: To selectively visualize the left gastric vein (LGV) with hepatopetal flow information by non-contrast-enhanced magnetic resonance angiography under a hypothesis that change in the LGV flow direction can predict the development of esophageal varices; and to optimize the acquisition protocol in healthy subjects. MATERIALS AND METHODS: Respiratory-gated three-dimensional balanced steady-state free-precession scans were conducted on 31 healthy subjects using two methods (A and B) for visualizing the LGV with hepatopetal flow. In method A, two time-spatial labeling inversion pulses (Time-SLIP) were placed on the whole abdomen and the area from the gastric fornix to the upper body, excluding the LGV area. In method B, nonselective inversion recovery pulse was used and one Time-SLIP was placed on the esophagogastric junction. The detectability and consistency of LGV were evaluated using the two methods and ultrasonography (US). RESULTS: Left gastric veins by method A, B, and US were detected in 30 (97%), 24 (77%), and 23 (74%) subjects, respectively. LGV flow by US was hepatopetal in 22 subjects and stagnant in one subject. All hepatopetal LGVs by US coincided with the visualized vessels in both methods. One subject with non-visualized LGV in method A showed stagnant LGV by US. CONCLUSION: Hepatopetal LGV could be selectively visualized by method A in healthy subjects.

A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum.

Neurofibromas can occur anywhere in the body, but they usually involve the head, neck, pelvis, and extremities. Abdominal visceral involvement is rare, and intrahepatic involvement is even less common. We describe a patient who suffered from plexiform neurofibromatosis with liver involvement. A 49-year-old man, who had previously been diagnosed with neurofibromatosis, underwent esophagogastroduodenoscopy and abdominal ultrasonography for screening purposes. Esophagogastroduodenoscopy showed grade 2 esophageal varices and abdominal ultrasonography showed conglomerated nodules with echogenic appearances in the perihepatic space. Magnetic resonance imaging showed presumed plexiform neurofibroma involving the lesser sac and hepatic hilum and encasing the common hepatic artery celiac trunk and superior mesenteric artery left portal triad. We report an unusual case of portal hypertension attributed to the compressive narrowing of the portal vein by presumed as plexiform neurofibroma at the lesser sac and hepatic hilum.

Biliary Cystadenoma Causing Esophageal Varices / 고신대학교의과대학학술지

Biliary cystadenomas are benign but potentially malignant cystic neoplasm. The preferred treatment is radical resection because it is difficult to differentiate a benign from a malignant biliary cystadenoma. A 40 year-old woman presented with moderate abdominal discomfort. Esophageal varix was found up to mid-esophagus on endoscopy. She has no prior history of liver disease or chronic alcohol ingestion. About 15cm sized biliary cystadenoma was diagnosed by ultrasonography, computed tomography and magnetic resonance imaging. Serum level of bilirubin, alanine aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase and tumor marker were elevated. The patient underwent US-guided aspiration. Tumor markers from the aspirated fluid are increased. Left hepatectomy was performed to completely remove the cyst. Histology of the resected specimen confirmed a biliary cystadenoma of the liver with ovary-like stroma. Without prior history of liver disease or chronic alcoholic ingestion, incidental finding of esophageal varix could show an important clue for diagnosis of biliary cystadenoma.

A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum

Neurofibromas can occur anywhere in the body, but they usually involve the head, neck, pelvis, and extremities. Abdominal visceral involvement is rare, and intrahepatic involvement is even less common. We describe a patient who suffered from plexiform neurofibromatosis with liver involvement. A 49-year-old man, who had previously been diagnosed with neurofibromatosis, underwent esophagogastroduodenoscopy and abdominal ultrasonography for screening purposes. Esophagogastroduodenoscopy showed grade 2 esophageal varices and abdominal ultrasonography showed conglomerated nodules with echogenic appearances in the perihepatic space. Magnetic resonance imaging showed presumed plexiform neurofibroma involving the lesser sac and hepatic hilum and encasing the common hepatic artery celiac trunk and superior mesenteric artery left portal triad. We report an unusual case of portal hypertension attributed to the compressive narrowing of the portal vein by presumed as plexiform neurofibroma at the lesser sac and hepatic hilum.

Prevention of Variceal Rebleeding According to the Dose of Propranolol / 대한내과학회지

Esophageal variceal bleeding is a common complication of liver cirrhosis. Non-selective beta blockers (NSBB) have been established in numerous studies as one of the medical treatment for cirrhosis, especially in the primary and secondary prevention of variceal bleeding. The dose of NSBB is adjusted for a reduction in the resting heart rate by 25%, to 55 beat/min, or until the occurrence of adverse effect. The mean adjusted dose of propranolol in Korean study is 160 mg/day. Nevertheless, low dose propranolol is frequently used in real clinical field. A study by Kwon et al. showed that effect of propranolol in the prevention for esophageal rebleeding was superior in maximally-tolerable dose group of propranolol than low dose group. In this editorial, we have reviewed the studies of prevention for variceal rebleeding focusing on the dose of propranolol.

Stroke after percutaneous transhepatic variceal obliteration of esophageal varix in Caroli syndrome.

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

Cerebral and splenic infarctions after injection of N-butyl-2-cyanoacrylate in esophageal variceal bleeding.

Variceal bleeding is the most serious complication of portal hypertension, and it accounts for approximately one fifth to one third of all deaths in liver cirrhosis patients. Currently, endoscopic treatment remains the predominant method for the prevention and treatment of variceal bleeding. Endoscopic treatments include band ligation and injection sclerotherapy. Injection sclerotherapy with N-butyl-2-cyanoacrylate has been successfully used to treat variceal bleeding. Although injection sclerotherapy with N-butyl-2-cyanoacrylate provides effective treatment for variceal bleeding, injection of N-butyl-2-cyanoacrylate is associated with a variety of complications, including systemic embolization. Herein, we report a case of cerebral and splenic infarctions after the injection of N-butyl-2-cyanoacrylate to treat esophageal variceal bleeding.