• 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.

• 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.