Fulminant hepatic failure: a case report

Clinical History

A 64-year-old woman with no known diseases and no alcohol abuse in her history was admitted to our hospital because of hepatic dysfunction and cholestasis. She had elevated serum alkaline phosphatase, gamma-glutamyl transpeptidase, and serum aminotransferase levels. Serum viral hepatitis markers were negative.

Imaging Findings

Abdominal ultrasound showed hepatomegaly with irregular liver contour and diffuse heterogeneity of liver texture with multiple, ubiquitous hyperechoic and hypoechoic micro- and macronodules. Enlargement of the spleno-porto-mesenteric venous system and mildly enlarged celiac lymph nodes were also seen.
An MRI examination of the abdomen was then performed, showing liver stucture completely subverted by multiple, diffuse, party confluent nodules with low signal intensity on T1-weighted images, mixed signal intensity on T2-weighted images, and hypointensity compared to the surrounding liver tissue on contrast-enhanced fat suppressed T1-weighted images. Biopsy of liver lesions could not be carried out due to severe coagulopathy. The patient died of liver failure a few days later.

Discussion

Fulminant hepatic failure (FHF), or acute liver failure, is a complex syndrome with multiple aetiologies characterised by an abrupt onset of jaundice and hepatic encephalopathy in the absence of preexisting liver disease [1]. Incidence is 2000 new cases per year in the United States [2]. Generally FHF is defined by reduced synthesis parameters of the liver, usually INR>1.5 and reduced detoxification resulting in any degree of encephalopathy. This is accepted by the exclusion of preexisting cirrhosis and a duration of a liver disease for less than 26 weeks. FHF can result from a wide variety of causes (e.g. idiopathic, drug toxicity, viral hepatitis, autoimmune hepatitis, and Wilson disease) but viral or toxin-induced hepatitis are the most common [3]. Hepatic encephalopathy is the hallmark of FHF in all classifications and clearly marks the transition from a severe condition to a deadly disease. FHF often causes multisystem organ failure. Frequently the presenting symptoms are nonspecific, including fatigue, malaise, anorexia, nausea, abdominal pain, fever, and jaundice. These symptoms progress to the development of encephalopathy and/or coma. Severe coagulopathy often precedes the evolution of hepatic encephalopathy to coma [4].
Ultrasonography is primarily the preferred imaging method in these conditions because it is a cost-effective, fast, and noninvasive technique without exposure to ionising radiation. Hepatic surface nodularity (focal relatively hyperechoic areas) is a common finding on US images and usually reflects a combination of alternating foci of confluent regenerative nodules and necrosis rather than cirrhosis ("pseudocirrhosis"). The histopathologic findings are massive hepatocyte necrosis with hepatic reticulation in framework collapse around nodular hepatocyte regeneration. Such histopathologic findings differ from those of cirrhosis, which is characterised by regenerative nodules and fibrous septa [5].
CT images shows areas of liver necrosis as low attenuation before administration of iodinated contrast material and isoattenuation or hyperattenuation compared with areas of liver regeneration. On MRI, the necrotic liver parenchyma appears as hypo- and hyperintense on T1- and T2-weighted images, respectively, whereas areas of regeneration have the opposite MRI appearance [6]. Unfortunately, in our case it was impossible to elucidate the cause for hepatic failure because the patient's history was apparently unremarkable and neither liver biopsy nor autopsy was performed.
Liver transplantation is the only measure that can radically influence the course of FHF. Elucidation of the cause of hepatic failure allows some patients to benefit from specific treatments and may influence posttransplant management if transplantation is performed [2].

Differential Diagnosis List

Final Diagnosis

Fulminant hepatic failure

URL:	https://www.eurorad.org/case/10824
DOI:	10.1594/EURORAD/CASE.10824
ISSN:	1563-4086