• 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.

• 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.

• The Korean Journal of Pharmacology
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• v.23 no.1
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• pp.15-23
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• 1987

To elucidate the mechanism of splanchnic hyperemia associated with chronic portal hypertension, we have investigated the alteration in visceral reflexes in conjuction with circulatory hemodynamics in portal ligated portal hypertension in cats. When capsaicin, bradykinin and vasopressin were injected via splenic artery of sham cat, respectively, they caused not only reflex excitation of systemic arterial pressure, but also elevation of splenic venous pressure with unchanged heart rates. Simultaneously, they evoked the sympathetic efferent excitation of liver (spleno-hepatic reflex) as well as of spleen (spleno-splenic reflex). Similarly, capsaicin upon pledging on the liver surface evoked a significant increase in the pressor reflex with hepatic nerve excitation (hepato-hepatic reflex). After portal ligation, the splenic venous pressure was gradually elevated in association with decrease in systemic arterial pressure. However, the excitation of pressor reflex was enhanced on the and day, thereafter, being returned to the control, and the reflexly induced spleno-splenic, spleno-hepatic and hepato-hepatic sympathetic excitations were significantly diminished on the 8th day following portal vein ligation. In conclusion, it is suggested that sympathetic reflexes to spleen and liver are specifically intervened by the same central pathways and furthermore, the diminution of these viscero-visceral reflex excitations after portal ligation may be related to the intestinal hyperemia.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.98-101
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• 2004

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures

• Journal of Veterinary Clinics
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• v.41 no.2
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• pp.95-100
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• 2024

A 6-year-old neutered male Poodle with a body weight of 2.7 kg was diagnosed with a congenital extrahepatic portoazygos shunt presented with progressive hepatic encephalopathy. Five days after surgical attenuation, the patient showed post-attenuation neurological signs (PANS) such as generalized tonic-clonic seizure, dull mentation, and tremor. PANS were successfully managed with antiepileptic drugs, but third-space fluid accumulation (ascites and peripheral edema) and phlebectasia were newly identified, suggesting marked portal hypertension (PHT). Telmisartan, spironolactone, carvedilol, and prednisolone were sequentially administered. Three months after surgery, both abnormal clinicopathological values such as anemia and hypoalbuminemia, and clinical signs completely resolved. Herein, we report successful management strategies for PANS and PHT in a dog following surgical attenuation of a congenital portoazygos shunt.

• 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.

• Korean Journal of Radiology
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• v.2 no.4
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• pp.204-209
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• 2001

Objective: To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) in patients with occluded previous TIPS. Materials and Methods: Between February 1996 and December 2000 we performed five transcaval TIPS procedures in four patients with recurrent gastric cardiac variceal bleeding. All four had occluded TIPS, which was between the hepatic and portal vein. The interval between initial TIPS placement and revisional procedures with transcaval TIPS varied between three and 31 months; one patient underwent transcaval TIPS twice, with a 31-month interval. After revision of the occluded shunt failed, direct cavoportal puncture at the retrohepatic segment of the IVC was attempted. Results: Transcaval TIPS placement was technically successful in all cases. In three, tractography revealed slight leakage of contrast materials into hepatic subcapsular or subdiaphragmatic pericaval space. There was no evidence of propagation of extravasated contrast materials through the retroperitoneal space or spillage into the peritoneal space. After the tract was dilated by a bare stent, no patient experienced trans-stent bleeding and no serious procedure-related complications occurred. After successful shunt creation, variceal bleeding ceased in all patients. Conclusion: Transcaval TIPS placement is an effective and safe alternative treatment in patients with occluded previous TIPS and no hepatic veins suitable for new TIPS.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.