• Journal of Yeungnam Medical Science
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• v.35 no.1
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• pp.89-93
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• 2018

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.400-406
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• 2019

We report a 12-month-old female infant who had a history of neonatal sepsis with liver micro-abscesses that resolved with intravenous antibiotics during neonatal period. During her neonatal admission period, no umbilical vein catheter was inserted. Also, she did not undergo any abdominal surgeries or had a postnatal history of necrotizing enterocolitis. However, the child developed upper gastrointestinal bleeding in form of hematemesis and melena secondary to esophageal varices at the age of 12 months with an extra-hepatic portal vein obstruction with cavernous transformation and portal hypertension subsequently. The child underwent a successful endoscopic injection sclerotherapy. She is now 20-month-old and has portal hypertension but otherwise asymptomatic. We are proposing the possibility of a delayed-onset portal hypertension as a complication of liver abscess and neonatal sepsis.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.3
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• pp.307-312
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• 2023

Hepatic arterioportal fistulae are abnormal communications between the hepatic artery and portal vein. They are reported to be congenital or acquired secondary to trauma, iatrogenic procedures, hepatic cirrhosis, and hepatocellular carcinoma, but less likely to occur spontaneously. Extrahepatic portal venous obstruction (EHPVO) can lead to pre-hepatic portal hypertension. A spontaneous superimposed hepatic arterioportal fistula can lead to pre-sinusoidal portal hypertension, further exacerbating its physiology. This report describes a young woman with long-standing EHPVO presenting with repeated upper gastrointestinal variceal bleeding and symptomatic hypersplenism. Computed tomography scan demonstrated a cavernous transformation of the portal vein and a macroscopic hepatic arterioportal fistula between the left hepatic artery and portal vein collateral in the central liver. The hepatic arterioportal fistula was associated with a flow-related left hepatic artery aneurysm and a portal venous collateral aneurysm proximal and distal to the fistula, respectively. Endovascular coiling was performed for the hepatic arterioportal fistula, followed by proximal splenorenal shunt procedure. This case illustrates an uncommon association of a spontaneous hepatic arterioportal fistula with EHPVO and the utility of a combined endovascular and surgical approach for managing multifactorial non-cirrhotic portal hypertension in such patients.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.4
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• pp.361-364
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• 2018

Accessory hepatic lobe is noted as and considered a rare disease in children. It can manifest with various symptoms and complications depending on the location, volume, type and position of the disease as presented on a child. The patient presented as a 14-month-old girl who was seen with a notable hepatosplenomegaly and portal hypertension. A diagnosis was made after taking an extensive medical history, observation and radiological examinations. The formal diagnosis was a prehepatic portal hypertension associated with accessory hepatic lobe.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.221-225
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• 2007

A previously healthy 3-year-old girl was admitted to the Department of Pediatrics in Severance Hospital with sudden symptoms of melena. The vital signs were stable, and splenomegaly was found in a physical examination. The patient had moderate thrombocytopenia. There was no evidence of autoimmune disease. A upper gastrointestinal endoscopy and esophagogram showed a varix on the lower esophagus. Coarse liver parenchymal echoes and increased periportal echogenicity were seen on a Doppler sonogram. The velocity of the portal vein mildly increased. Magnetic-resonance-cholangiopancreatogram (MRCP) demonstrated normal portal structures. A sono-guided liver biopsy was performed, but the pathological findings were unremarkable. Based on these findings, we diagnosed the patient with idiopathic portal hypertension. The patient was discharged and was treated with oral beta blocker. We report a case of idiopathic portal hypertension with a brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.48 no.1
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• pp.67-71
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• 2000

Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2 % incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances, normally metabolized by the liver, may have gained access to pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.

• Journal of the Korean Society of Radiology
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• v.79 no.5
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• pp.264-270
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• 2018

Purpose: To evaluate the effect of portal hypertension on the tumor recurrence in patients with hepatocellular carcinoma (HCC) and without hepatic decompression following radiofrequency ablation (RFA). Materials and Methods: Treatment-naïve HCC patients within the Milan criteria and with Child-Pugh class A were included in this study, who had performed RFA in our hospital between January 2010 and March 2017. Univariate and multivariate analyses using the Cox proportional hazard model were performed to find the predictors of local or distant tumor recurrence. Results: Overall, 178 patients were included in this study. Median follow-up period was 40.2 months. The difference in the local tumor progression rates depending on the absence or presence of portal hypertension was not statistically significant (p = 0.195). The 1-, 3-, and 5-year distant intrahepatic tumor spread rates were 6.6%, 29.5%, and 537% in patients without portal hypertension, and 23.4%, 51.9%, and 63.6% in patients with portal hypertension, respectively. The difference was statistically significant (p = 0.011). Univariate and multivariate analysis showed that portal hypertension was an independent predictor for distant intrahepatic tumor spread (p = 0.008). Conclusion: For HCC patients with Child-Pugh class A, portal hypertension adversely affected distant intrahepatic tumor progression.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.3
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• pp.264-270
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• 2023

Backgrounds/Aims: Proximal splenorenal shunt (PSRS) is a commonly performed procedure to decompress portal hypertension, in patients with refractory variceal bleed, especially in non-cirrhotic portal hypertension (NCPH). If conventional methods are hindered by any technical or pathological factors, alternative surgical techniques may be required. This study analyzes the effectiveness of various unconventional shunt surgeries performed for NCPH. Methods: A retrospective analysis of NCPH patients who underwent unconventional shunt surgeries during the period July 2011 to June 2022 was conducted. All patients were followed up for a minimum of 12 months with doppler study of the shunt to assess shunt patency, and upper gastrointestinal endoscopy to evaluate the regression of varices. Results: During the study period, 130 patients underwent shunt surgery; among these, 31 underwent unconventional shunts (splenoadrenal shunt [SAS], 12; interposition mesocaval shunt [iMCS], 8; interposition PSRS [iPSRS], 6; jejunal vein-cava shunt [JCS], 3; left gastroepiploic-renal shunt [LGERS], 2). The main indications for unconventional shunts were left renal vein aberration (SAS, 8/12), splenic vein narrowing (iMCS, 5/8), portalhypertensive vascular changes (iPSRS, 6/6), and portomesenteric thrombosis (JCS, 3/3). The median fall in portal pressure was more in SAS (12.1 mm Hg), and operative time more in JCS, 8.4 hours (range, 5-9 hours). During a median follow-up of 36 months (6-54 months), shunt thrombosis had been reported in all cases of LGERS, and less in SAS (3/12). Variceal regression rate was high in SAS, and least in LGERS. Hypersplenism had reversed in all patients, and 6/31 patients had a recurrent bleed. Conclusions: Unconventional shunt surgery is effective in patients unsuited for other shunts, especially PSRS, and it achieves the desired effects in a significant proportion of patients.

• Clinical Endoscopy
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• v.51 no.6
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• pp.563-569
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• 2018

Background/Aims: To evaluate patients with portal hypertension (PH) of varied etiologies for portal hypertensive enteropathy (PHE) using the PillCam SB3 capsule endoscopy (CE) system. Methods: Consecutive patients with PH presenting with unexplained anemia and/or occult gastrointestinal bleeding were evaluated using the PillCam SB3 CE system. Abnormal findings were categorized as vascular or non-vascular. The patients with ongoing bleeding caused by PHE were treated. The correlation of the CE scores of PHE with the clinical, laboratory, and endoscopic features was determined. Results: Of the 43 patients included in the study, 41 (95.3%) showed PHE findings. These included varices (67.4%), red spots (60.5%), erythema (44.2%), villous edema (46.5%), telangiectasia (16.3%), and polyps (16.3%). The CE scores varied from 0 to 8 ($mean{\pm}standard$ deviation, $4.09{\pm}1.8$). Five patients (11.6%) showed evidence of ongoing or recent bleeding due to PHE. Three of these five patients underwent endotherapy, and one patient underwent radiological coil placement. Conclusions: The PillCam SB3 CE system revealed a high prevalence of PHE in the patients with PH. Using this system, evidence of bleeding due to PHE was found in a small but definite proportion of the patients.

• Journal of the Korean Society of Radiology
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• v.84 no.1
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• pp.291-297
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• 2023

Isolated left-sided portal hypertension is a rare clinical syndrome caused by splenic veno-occlusive disease. Splenic vein thrombosis and extrinsic compression causes proximal splenic vein hypertension, and the splenic blood flows into the superior mesenteric or portal vein through the upper stomach's collateral vessels, such as the short gastric, coronary, and gastroepiploic veins. Open splenectomy is recommended to treat gastrointestinal bleeding caused by isolated left-sided portal hypertension. Interventional management could be a clinically useful option for selected patients who want to avoid surgical corrections. The report presents two cases of left-sided portal hypertension with gastric variceal bleeding.