Diagnostic Criteria and Treatment Options for Portosystemic Encephalopathy Syndrome

Diagnostic Criteria

Portosystemic encephalopathy (PSE) is classified as Type B hepatic encephalopathy, occurring due to portosystemic shunting without significant liver disease 3
The syndrome can be distinguished from Type A (acute liver failure) and Type C (cirrhosis with or without portosystemic shunting) 3
Symptoms include fatigue, cognitive deficits, and recurrent episodes of encephalopathy 1, 2
Neuropsychological tests reveal cognitive deficits similar to those in cirrhosis with minimal hepatic encephalopathy 1
Patients may develop symptoms early in life or remain asymptomatic until the sixth or seventh decades 1, 2
Unexplained hyperammonemia is a key diagnostic finding 1, 2
MRI is recommended for diagnosis and classification of portosystemic shunts 1, 2
Complete blood tests including electrolytes, renal function, and liver function tests are essential 4
West Haven criteria should be used for grading when temporal disorientation is present (grades ≥2) 3
Glasgow Coma Scale should be added for patients with grades III-IV encephalopathy 3

Rifaximin is effective as an add-on therapy when lactulose alone is insufficient 6
Rifaximin prophylaxis is recommended before non-urgent TIPS procedures to prevent post-TIPS encephalopathy 7, 8
Portosystemic shunt ligation/embolization should not be performed systematically but considered on a case-by-case basis 7, 8
Radiological embolization or surgical ligation of large portosystemic shunts appears feasible in selected patients with HE, resulting in recovery in 46-100% of cases 7, 8
MELD score is the strongest predictor of HE recurrence, with a cut-off of 11 used for patient selection 7, 8
Liver transplantation should be offered to patients with disabling symptoms of hepatic encephalopathy refractory to drug and non-drug treatments 7, 8
Liver transplantation may be the only treatment option when there is an absent portal vein or with intractable portosystemic encephalopathy 1, 2, 9

Congenital portosystemic shunting should be investigated in patients with unexplained hyperammonemia, mental retardation, or clinical picture compatible with hepatic encephalopathy in the absence of cirrhosis 1, 2
Two types of congenital portosystemic shunts exist: extrahepatic and intrahepatic (includes patent ductus venosus) 1, 2
Shunt diameter may determine clinical course: large shunts cause persistent manifestations from childhood, while small intrahepatic shunts lead to recurrent episodes beginning in adulthood 1, 2
TIPS-related HE occurs in approximately 35% of cases 7, 8
A single episode of overt HE is not a formal contraindication to elective TIPS creation, but should be discussed case-by-case 7, 8
For cases of refractory gastrointestinal bleeding, there is no contraindication to TIPS as there is no therapeutic alternative 7, 8
Mental status changes may be subtle in early stages, requiring formal neuropsychological testing 3
Avoid benzodiazepines and other sedatives that can worsen symptoms 5, 6
Always rule out other causes of altered mental status before attributing symptoms to PSE 4
Recognize that hyponatremia and sepsis can produce encephalopathy independently and precipitate hepatic encephalopathy 4