Portal-Systemic Encephalopathy Due to Congenital Intrahepatic Shunts

Raskin, Neil H.; Price, John B.; Fishman, Robert A.

doi:10.1056/nejm196401302700503

Cited by 166 publications

(93 citation statements)

References 16 publications

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“…Case studies in children and adults have shown substantial cognitive defects in patients with congenital portalsystemic shunts. [31][32][33][34][35][36][37][38][39] We investigated the cognitive function of 2 children with congenital shunts and essentially no effective portal blood flow and found major defects in attention and motor skills. 40 Staged closure of the shunts resulted in normal effective portal blood flow to the liver, and cognitive function substantially improved in follow-up.…”

Section: Figurementioning

confidence: 99%

Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability

Zelko²,

et al. 2006

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OBJECTIVES. Children with primary extrahepatic portal vein thrombosis (EHPVT) have portal-systemic shunting, which may lead to disturbed neurocognitive function similar to portal-systemic encephalopathy (PSE) seen with chronic liver disease and cirrhosis. The functions most affected are those involving fluid cognitive ability, which comprise neurocognitive domains such as attention, processing speed, and short-term memory, that are particularly vulnerable to systemic illness or diffuse neurologic insult. We determined the fluid cognitive ability of children with EHPVT and whether surgically restoring portal blood flow by mesenteric left portal vein bypass (MLPVB) improved it.DESIGN. Twelve children with EHPVT and no overt PSE underwent comprehensive neurocognitive testing before and 1 year after undergoing surgery with intent to perform MLPVB. The evaluations sampled 4 functional domains at both time points: (1) neurobehavioral (behavior, emotional, executive functioning); (2) broad cognitive (intelligence, achievement); (3) fluid ability (attention, mental speed, working memory, memory encoding); and (4) visual motor (drawing, fine motor). Tasks in the fluid-ability and visual-motor domains were expected to be especially sensitive to adverse effects of EHPVT and to be most likely to show improvement with MLPVB. The test group consisted of 8 subjects who underwent successful MLPVB, and the comparison group was composed of 3 patients who received distal splenorenal shunts and one whose MLPVB failed.RESULTS. Both groups demonstrated similar fluid cognitive ability at initial evaluation. Successful MLPVB resulted in significantly improved fluid cognitive function: in the fluid cognitive domain, significant improvements were seen for the hit reaction time variability in the Conner's Continuous Performance Test, the attention scale of the Cognitive Assessment System, and immediate verbal memory in the Children's Memory Scale. In the visual-motor domain, z scores on the Grooved Pegboard Test improved. No improvement was observed in the comparison group.www.pediatrics.org/cgi

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Section: Figurementioning

confidence: 99%

Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability

Zelko²,

et al. 2006

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“…Intrahepatic microscopic portosystemic venous shunt is sometimes observed in liver cirrhosis (1-3), however, intrahepatic macroscopic shunt has been rarely reported since the first description by Raskin et al (4). Excluding traumatic causes, the extensive intrahepatic portosystemic shunt is thought to originate either from congenital vascular malformation or as a result of massive necrosis postnatally induced by factors such as viral infection or hepatotoxic substances (2,5).…”

Section: Introductionmentioning

confidence: 99%

Extrahepatic Portosystemic Venous Shunt without Portal Hypertension.

et al. 1997

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A 72-year-old womanwas admitted for recurrent episodes of encephalopathy. Laboratory data showedmild liver dysfunction and hyperammonemia, while she had neither anemia nor splenomegaly. The dilated inferior mesenteric vein (IMV) was opacified retrogradely from the superior mesenteric vein by superior mesenteric arteriography, and IMVwas found to connect with the inferior vena cava (IVC) through a torturous shunt. Noobstruction of the extrahepatic portal vein or hepatic vein was observed by arteriography. Histological evaluation of the liver biopsy indicated remarkable fatty change without cirrhosis. Finally, we diagnosed this case as extrahepatic portosystemic venous shunt without portal hypertension.

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“…Surgical intervention (shunt ligation, shunt removal, or liver transplantation) or transcatheter embolization of the shunt vessel are the next treatment options. The traditional approach to treating a shunt vessel is surgical removal or ligation [6]. However, transcatheter embolization of the causative vessel has been widely accepted and rapidly used worldwide because of its less invasive nature [7].…”

Section: Definition Pathogenesis Classification and Therapeutics Omentioning

confidence: 99%

Efficacy and Safety of Transcatheter Embolization for Hepatic Encephalopathy Caused by Spontaneous Portosystemic Shunts

Takenaga

Aizawa

2017

Interventional Radiology

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Hepatic encephalopathy (HE) is a syndrome that occurs in patients with severe liver dysfunction or a portosystemic shunt. In patients with refractory HE caused by a portosystemic shunt, interventional closure of the shunt vessel is essential. Currently, transcatheter embolization is recognized as a less invasive and highly effective procedure, and it is considered as a first-choice method for the occlusion of shunt vessels. In this review, we discuss the role of a portosystemic shunt in the development of HE and describe the procedure and significance of transcatheter embolization of a portosystemic shunt.

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Portal-Systemic Encephalopathy Due to Congenital Intrahepatic Shunts

Cited by 166 publications

References 16 publications

Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability

Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability

Extrahepatic Portosystemic Venous Shunt without Portal Hypertension.

Efficacy and Safety of Transcatheter Embolization for Hepatic Encephalopathy Caused by Spontaneous Portosystemic Shunts

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