• 한국간담췌외과학회지
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• 제26권4호
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• pp.298-307
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• 2022

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권4호
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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.

• 한국간담췌외과학회지
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• 제27권4호
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• pp.350-365
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• 2023

Backgrounds/Aims: Extra hepatic portal venous obstruction (EHPVO) is the most common cause of portal hypertension in Indian children. While endoscopy is the primary modality of management, a subset of patients require surgery. This study aims to report the short- and long-term outcomes of EHPVO patients managed surgically. Methods: All the patients with EHPVO who underwent surgery between August 2007 and December 2021 were retrospectively reviewed. Postoperative complications were classified after Clavien-Dindo. Binary logistic regression in Wald methodology was used to determine the predictive factors responsible for unfavourable outcome. Results: Total of 202 patients with EHPVO were operated. Mean age of patients was 20.30 ± 9.96 years, and duration of illness, 90.05 ± 75.13 months. Most common indication for surgery was portal biliopathy (n = 59, 29.2%), followed by bleeding (n = 50, 24.8%). Total of 166 patients (82.2%) had shunt procedure. Splenectomy with esophagogastric devascularization was the second most common surgery (n = 20, 9.9%). Nine major postoperative complications (Clavien-Dindo > 3) were observed in 8 patients (4.0%), including 1 (0.5%) operative death. After a median follow-up of 56 months (15-156 months), 166 patients (82.2%) had favourable outcome. In multivariate analysis, associated splenic artery aneurysm (p = 0.007), isolated gastric varices (p = 0.004), preoperative endoscopic retrograde cholangiography and stenting (p = 0.015), and shunt occlusion (p < 0.001) were independent predictors of unfavourable long-term outcome. Conclusions: Surgery in EHPVO is safe, affords excellent short- and long-term outcome in patients with symptomatic EHPVO, and may be considered for secondary prophylaxis.