Hepatic Encephalopathy Without Cirrhosis


American Journal of Transplantation Images in Transplantation – Continuing Medical Education (CME)

Each month, the American Journal of Transplantation will feature Images in Transplantation, a journal-based CME activity, chosen to educate participants on current developments in the science and imaging of transplantation. Participants can earn 1 AMA PRA Category 1 Credit™ per article at their own pace.

This month's feature article is titled: “Hepatic Encephalopathy Without Cirrhosis.”

Accreditation and Designation Statement

This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Blackwell Futura Media Services, the American Society of Transplant Surgeons and the American Society of Transplantation. Blackwell Futura Media Services is accredited by the ACCME to provide continuing medical education for physicians.

Blackwell Futura Media Services designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 Credit™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Statement of Need

Hepatic encephalopathy or portosystemic encephalopathy is one of the major complications of liver cirrhosis. It is characterized by an alteration of neurological function resulting from the accumulation of toxic substances in blood, such as ammonia, that are normally filtered by a functioning liver. Hepatic encephalopathy can also be a symptom of inherited urea cycle enzyme defects, Reye's syndrome, sodium valproate toxicity, or vascular anomalies. When evaluating hepatic encephalopathy in a patient without obvious liver cirrhosis, these other causes need to be considered.

Purpose of Activity

This activity is designed to increase the participants' knowledge and competency in understanding the evaluation and management of a patient with hepatic encephalopathy without liver cirrhosis, thus optimizing patient outcomes.

Identification of Practice Gap

Most cases of hepatic encephalopathy are due to liver cirrhosis. The diagnosis might be delayed if other causes are not considered.

Learning Objectives

Upon completion of this educational activity, participants will be able to:

  • Know the diagnostic algorithm to determine the cause of hepatic encephalopathy.
  • Recognize causes of hepatic encephalopathy in patients without cirrhosis.
  • Be familiar with the radiologic appearance of portosystemic shunt.
  • Know the various causes of portosystemic shunts and their treatment.

Target Audience

This activity is designed to meet the educational needs of hepatologists, surgeons, radiologists, and others involved in the treatment of patients with liver disease.


No commercial support has been accepted related to the development or publication of this activity. Blackwell Futura Media Services has reviewed all disclosures and resolved or managed all identified conflicts of interest, as applicable.


Allan D. Kirk, MD, PhD, FACS, has no relevant financial relationships to disclose.


Sandy Feng, MD, PhD, discloses stock and/or equity interest in Abbott, Amgen, Charles River Labs, Eli Lily, Glaxo-Smith-Klein, Hospira, Johnson & Johnson, Express Scripts, Medco, Merck, Pfizer, and Stryker; research support from Cumberland and Quark; and research support and consulting work for Novartis.

Douglas W. Hanto, MD, PhD, has no relevant financial relationships to disclose.


Lena Sibulesky, MD, J. Mark McKinney, MD, Justin H. Nguyen, MD, and Raj Satyanarayana, MD, have no relevant financial relationships to disclose.

ASTS Staff

Mina Behari, Director of Education

This manuscript underwent peer review in line with the standards of editorial integrity and publication ethics maintained by the American Journal of Transplantation. The peer reviewers have no relevant financial relationships to disclose. The peer review process for the American Journal of Transplantation is blinded. As such, the identities of the reviewers are not disclosed in line with the standard accepted practices of medical journal peer review.

Instructions on Receiving CME Credit

This activity is designed to be completed within an hour. Physicians should claim only those credits that reflect the time actually spent in the activity. This activity will be available for CME credit for twelve months following its publication date. At that time, it will be reviewed and potentially updated and extended for an additional twelve months.

Follow these steps to participate, answer the questions and claim your CME credit:

  • Log on to https://www.wileyhealthlearning.com/ajt
  • Read the learning objectives, target audience, and activity disclosures.
  • Read the article in print or online format.
  • Reflect on the article.
  • Access the CME Exam, and choose the best answer to each question.
  • Complete the required evaluation and print your CME certificate.

A 66-year-old woman with end-stage renal disease due to membranoproliferative glomerulonephritis began hemodialysis in 2010. She remained in her usual state of health until 2012 when she was hospitalized with several episodes of severe encephalopathy with ammonia levels as high as 674 µg/dL (normal: 20–80 µg/dL). Her symptoms worsened despite lactulose treatment. The patient denied antecedent history of ascites, gastrointestinal bleeding, jaundice or liver disease. Nevertheless, because of high ammonia levels in conjunction with her symptoms, she underwent work-up for liver disease. The laboratory studies showed normal hepatic function. Viral and autoimmune hepatitis serologies were negative. Percutaneous liver biopsy revealed intact hepatic architecture with minimal steatosis without inflammation or fibrosis (Figure 1). Contrast enhanced abdominal computed tomography (CT) scan demonstrated a vascular structural anomaly. This finding prompted a referral to interventional radiology for a transhepatic portal venogram (Figure 2).

L. Sibulesky1,*, J. M. McKinney2, J. H. Nguyen1 and R. Satyanarayana1

1Department of Transplantation, Mayo Clinic, Jacksonville, FL

2Department of Radiology, Mayo Clinic, Jacksonville, FL

*Corresponding author: Lena Sibulesky, sibulesky.lena@gmail.com

Figure 1.

Liver biopsy (hematoxylin and eosin stain 100x).

Figure 2.

Transhepatic portal venogram.


  1. Transhepatic portal venogram demonstrated:
    1. Arteriohepatic venous shunt
    2. Diffuse hepatic venovenous shunts
    3. Diffuse bilobar intrahepatic portosystemic venous shunts
    4. Arterioportal fistula
  2. The most likely cause of this condition in this patient is:
    1. Trauma
    2. Liver biopsy
    3. Congenital malformation
    4. Portal hypertension
  3. This condition is rare and the most common presentation is:
    1. Gastrointestinal bleeding
    2. Hepatic encephalopathy
    3. Acute liver failure
    4. Ascites
  4. The most sensitive diagnostic modality is:
    1. Doppler sonography
    2. CT
    3. MRI
    4. Percutaneous transhepatic portography and retrograde venography
  5. Based on the interventional radiology findings and clinical presentation, the most effective therapeutic approach in this patient is:
    1. Diet modification
    2. Surgical intervention
    3. Transcatheter embolization
    4. Liver transplant

To complete this activity and earn credit, please go to https://www.wileyhealthlearning.com/ajt