An Interesting Case of Congenital Intrahepatic Porto-hepatic Shunt as a Cause of Unexplained Encephalopathy

Alvi, Arsalan Ahmad; Pichardo, J. E. Hernández; Gupta, Sonali; Goyal, Pradeep; Mattana, Joseph

doi:10.7759/cureus.7639

Cited by 5 publications

(4 citation statements)

References 15 publications

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“…1 Abdominal CT angiography found congenital portosystemic shunts connecting the superior mesenteric vein and the inferior vena cava, without liver cirrhosis signs (figure 2). 2…”

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confidence: 99%

A 73-Year-Old Woman With Hyperammonemic Encephalopathy Caused by Noncirrhotic Congenital Portosystemic Shunts

et al. 2021

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A 73-year-old woman with hyperammonemic encephalopathy presented with recurrent, transient episodes of diminished memory and confusion that lasted for 2-3 days. Except for ammonia levels of 208.7 μ mol/L (normal range 10.71-32.13 μmol/L), the laboratory tests, including all liver tests, were normal. Neuropsychological assessment showed cognitive decline (4/30 on Mini-Mental State Examination and 1/30 on Montreal Cognitive Assessment). EEG showed triphasic waves, which disappeared along with clinical remission. MRI showed bilateral symmetrical lesions in cortex, cingulate, lentiform nucleus, and insular (figure 1). 1 Abdominal CT angiography found congenital portosystemic shunts connecting the superior mesenteric vein and the inferior vena cava, without liver cirrhosis signs (figure 2). 2

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“…1 Abdominal CT angiography found congenital portosystemic shunts connecting the superior mesenteric vein and the inferior vena cava, without liver cirrhosis signs (figure 2). 2…”

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confidence: 99%

A 73-Year-Old Woman With Hyperammonemic Encephalopathy Caused by Noncirrhotic Congenital Portosystemic Shunts

et al. 2021

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“…Shunts can increase in size over a patient’s lifespan and become symptomatic, explaining why the incidents of shunts are greatest in the elderly. 2 , 6 …”

Section: Discussionmentioning

confidence: 99%

Hepatic Encephalopathy Secondary to Non-cirrhotic Portosystemic Shunt

Trivedi,

Parikh,

Botros

et al. 2024

Journal of Investigative Medicine High Impact Case Reports

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Hepatic encephalopathy is uncommon in the absence of cirrhosis. We report a 71-year-old woman who presented with altered mental status in the setting of hyperammonemia for the second time in 6 months. Magnetic resonance imaging of the abdomen revealed an uncommon portosystemic shunt involving an enlarged posterior branch of the right portal vein and an accessory right hepatic vein, with no features of cirrhosis. Appropriate management of these patients with ammonia-lowering therapy can reduce repeat episodes and improve quality of life. This case demonstrates the importance of diagnosing non-cirrhotic hepatic encephalopathy in patients with altered mental status.

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“…The gold standard for the diagnosis of these vascular abnormalities is a liver biopsy, but it is not always possible to perform it. The patient's age, comorbidities, poor cooperation (often due to altered level of consciousness during hyperammonaemia), increase the peri-and intraprocedural risk, making this route unfeasible [7]. Consequently, these malformations remain poorly understood.…”

Section: Discussionmentioning

confidence: 99%

“…A decisive role in the characterization of these malformations is also played by CT (Figure 2) and MRI scans, which are used to confirm the diagnosis and to provide a precise vascular map; these can be done with or without contrast injection at the arterial and portal phase [8]. In support of how crucial it is for the correct characterization and to establish the severity of the malformation, today, thanks to a procedure called Per-rectal scintigraphy, it is possible to estimate the shunt ratio, which is the amount of portal blood that has shunted away from the liver directly into the systemic circulation [7]. Additionally, the improvement of interventional radiology techniques such as intravascular embolization using plugs or coils, have been described in several cases of SIHPSS surgery management [9].…”

Section: Discussionmentioning

confidence: 99%

Unusual Case of Hyperammonemic Encephalopathy in Elderly Patient Without Liver Disease

2024

Ann Case Report

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Here we describe a case of an 87-year-old man who was admitted to the hospital with clinical evidence of encephalopathy and was found to have hyperammonemia. Abdominal ultrasound (US) with color doppler showed a rare vascular anomaly consisting in a shunting of portal blood flow directly into the systemic circulation. Abdominal computed tomography scan with contrast injection was performed and revealed a spontaneous intrahepatic porto-systemic shunt (SIHPSS). Portosystemic shunts are rare and often detected in adulthood but should be considered an important cause of unexplained encephalopathy in the absence of cirrhotic liver disease or hepatic trauma. This case illustrates that, albeit rare, SIHPSS may express themselves for the first time in the elderly, a patient population that is frequently affected by many more common causes of altered mental status. Our patient was treated with medical conservative therapy which led to an impressive amelioration of his symptoms.

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An Interesting Case of Congenital Intrahepatic Porto-hepatic Shunt as a Cause of Unexplained Encephalopathy

Cited by 5 publications

References 15 publications

A 73-Year-Old Woman With Hyperammonemic Encephalopathy Caused by Noncirrhotic Congenital Portosystemic Shunts

A 73-Year-Old Woman With Hyperammonemic Encephalopathy Caused by Noncirrhotic Congenital Portosystemic Shunts

Hepatic Encephalopathy Secondary to Non-cirrhotic Portosystemic Shunt

Unusual Case of Hyperammonemic Encephalopathy in Elderly Patient Without Liver Disease

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