Case Presentation: A 51-year-old woman presented with recurrent confusion and altered mental status over the past 6 months. She reported associated hallucinations, altered sleep cycle and hyper-arousal symptoms. She was treated with lactulose and rifaximin for suspected hepatic encephalopathy with no improvement. She denied any other symptoms. Past medical history was significant for cirrhosis secondary to NASH (non-alcoholic steatohepatitis), type 2 diabetic mellitus, hypothyroidism, and essential hypertension. Medications included glargine, lisinopril and levothyroxine. She smoked, and denied alcohol or illicit drug use. Her vital signs were normal. She appeared irritable with mild distress. She had anicteric sclerae, and benign abdominal exam with no ascites. Cranial nerves were intact. She had a slow mentation with no focal deficits in strength or sensation. She was alert, oriented to person and place only. All muscle strength, tone and reflexes were normal in all extremities. Peripheral pulses were normal. No skin rash or joint swelling.
Hemoglobin was 12 g/dl, leukocyte count 9k/mm3, and platelets 120 k/mm3. Comprehensive metabolic panel showed urea 17 mg/dl and creatinine 0.8 mg/dl, Na 137, albumin 3.1, AST 21, ALT 31, ALK 120, and total bilirubin 0.4. Thyroid-stimulating hormone was 1.09 mIU/ml (0.27–4.2 uIU/ml). HbA1c was 7.9%. Hepatitis A, B and C serology and HIV antibodies were negative. Blood cultures were sterile. Abdominal ultrasound showed cirrhotic appearance of the liver with no masses and no free fluid. Lumbar puncture showed normal cell count, cytology, and protein, negative cultures, negative herpes simplex viral serology, and negative paraneoplastic antibodies. Magnetic resonance imaging (MRI) of the brain demonstrated a few scattered punctate foci of increased T2/FLAIR signal in the cerebral white matter.
During her hospital course, she was having an intermittent worsening confusion and behavioral. Her Ammonia level was fluctuating between 70 to 400 µ/dL (11 to 32 µmol/L) despite having frequent bowel movements and taking lactulose and rifaximin. Further imaging testing with abdominal angiography were pursued looking for possible portosystemic shunts. She was found to have a large spontaneous portosystemic shunting between superior mesenteric vein and the right gonadal vein measured 12 mm in diameter. She underwent successful embolization of the superior mesenteric varix at the confluence with the gonadal vein. Patient tolerated the procedure with no complication. She started to be less confused and being more active as she was working more with physical therapists. She was discharged home in a stable condition with hepatology follow up.
Discussion: A spontaneous extrahepatic portosystemic shunt (SPSS) is a rare malformation of the vessels which leads to the development of hepatic encephalopathy (HE) due to excessive shunting of blood from the portal vein to the inferior vena cava. Portosystemic shunt syndrome is a clinical triad of HE, no ascites, and a relatively low MELD score as it is shown in our patient. Diagnosis is with high clinical suspicion and aided with computed or magnetic resonance angiography.
Conclusions: Hospitalist should be mindful of the possibility of SPSS in patients with hepatic encephalopathy. Early diagnosis could limit unnecessary medical management and lessen potential harm. Endovascular management by occluding the portosystemic shunt should be considered in patient with type B hepatic encephalopathy.