Emphysematous hepatitis: A case report and review of literature

Abstract

Emphysematous hepatitis (EH) is a recently described gas forming liver infection, often associated with diabetes mellitus. It is often associated with poor prognosis, with rapidly fatal course in most reported cases. Its diagnosis is based on CT findings when an extensive gas replacing the liver parenchyma is present. We report the case of a 77-year-old woman with history of diabetes mellitus who presented in the emergency department with acute onset of cutaneomucous jaundice and fever, and in whom an abdominopelvic CT scan showed findings consistent with emphysematous hepatitis. EH is a rare condition with high mortality rate, however early diagnosis can be crucial in order to decrease mortality.

Introduction

Abdomino-pelvic gas-forming infections represent potentially life-threatening conditions that require aggressive medical and often surgical management. They have been widely recognized in the urinary tract, gallbladder, uterus, stomach, and pancreas [1]. Emphysematous hepatitis (EH) is a rare and recently described entity, first described in 2002 by Blachar et al. [2], and only few cases of emphysematous hepatitis have been described after that, most of them had a rapidly fatal course [3]. The pathogenesis of EH is poorly understood, although diabetes mellitus commonly coexists [4]. Its diagnosis is based on typical radiological findings on computed tomography and characterized by the presence of extensive gas replacing of the liver parenchyma without mass effect of any fluid collection [4]. Early diagnosis of this entity is crucial in order to decrease mortality.

We report a case of an emphysematous hepatitis in a 77-year-old woman.

Case presentation

A 77-year-old female patient with a history of treated hypertension and diabetes mellitus presented to the emergency department with acute onset cutaneomucous jaundice, associated with abdominal pain and fever. The initial clinical examination revealed a conscious patient, normotensive at 120 mmHg with a heart rate of 110 beats per minute, and stable respiratory status. The abdominal examination revealed rigidity of the right upper quadrant. Blood glucose was 5 g/L, and urinary dipstick was positive, suggestive of diabetic ketoacidosis (DKA). Laboratory investigations showed leukocytosis at 19,000/mm³, thrombocytopenia at 50,000/mm³, CRP at 343 mg/l, AST at 753 UI/l, ALT at 647 UI/l, conjugated bilirubin at 104 mg/l, gamma-GT at 108 UI/l, and ALP at 707 UI/l.

Abdominal ultrasound revealed a large acoustic shadow measuring 123 mm in the right liver lobe, primarily suggestive of a liver abscess (Fig. 1).

Fig. 1.

US images showing large acoustic shadow (asterisk) in the right liver lobe (green arrow).

Additional CT imaging with contrast enhancement (Fig. 2) confirmed the presence of a poorly limited area in the sixth hepatic segment containing multiple gas bubbles without enhancement wall nor collection, measuring 120 × 113 mm, associated with infiltration of the perihepatic fat. These findings were consistent with the EH.

Fig. 2.

CT findings of emphysematous hepatitis: Coronal (A) and axial (B) contrast enhanced CT images showing extensive gas (asterisk) replacing the liver parenchyma in the sixth segment with no enhancing wall nor fluid collection. Note the associated infiltration of the perihepatic fat in image (asterisk in C). Lung window better shows the extensive gas (asterisk in D) in the liver parenchyma.

The patient was managed initially with DKA rehydration protocol and broad-spectrum antibiotic therapy (ceftriaxone + metronidazole) and scheduled for radiological drainage. However, few hours after admission, she rapidly altered her consciousness and developed a septic shock with hypotension of 70/30 mmHg and tachypnea of 26 cycles per minute. The patient was immediately intubated and placed on norepinephrine with broadening of the antibiotic spectrum by introduction of vancomycin and fluconazole while maintaining metronidazole. Despite these resuscitative measures, the septic shock was refractory and the patient subsequently passed away 24 hours after admission.

Blood cultures revealed later on the presence of a multidrug-resistant Escherichia coli.

Discussion

Emphysematous hepatitis (EH) is an exceedingly rare yet severe and life-threatening infection. To our best knowledge, it was reported in only 18 cases in the literature [[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18]]. Among these cases, 11 were female, and diabetes mellitus was noted in 11 patients. The age range varied between 38 and 82 years old, with a mean age of 66 years old. EH proved fatal for 12 patients. Those who survived were diagnosed promptly and underwent aggressive treatment with antibiotics, radiological drainage and/or urgent laparotomy with surgical debridement. In our case, our patient was female and had a history of diabetes. Our patient passed away after rapid installation of refractory septic shock.

The pathogenesis of this necrotizing infection remains unclear, it is probably due to mixed acid fermentation from tissue necrosis by bacteria resulting in formation of nitrogen (60%), hydrogen (15%), carbon dioxide (5%), oxygen (5%) and the impaired transport of these products of catabolism from the production site [1,4,7]. Some authors also suppose that this mixed acid fermentation is due to a superinfection of an infarcted liver parenchyma by one of the following germs: Streptococcus mutans, Enterococcus faecalis, E. coli, Klebsiella, Enterobacter, Pseudomonas, and Proteus [1].

Clinical symptoms in EH are often nonspecific, with fever and abdominal pain being the most common symptoms [3,4]. Biological investigations often show increased white cells and CRP, and abnormal liver function tests with cytolysis and increased bilirubin [3].

The diagnostic criteria for EH rely on computed tomography findings, which are crucial for prompt and accurate diagnosis. These criteria include the replacement of liver parenchyma with gas, without evidence of fluid collections, abscess formation, or mass effect [[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18]].

Early recognition is crucial in attempts to decrease mortality, although the fatality of this condition remains very high. For the rare cases reported who survived, management of EH included aggressive antibiotics therapy and radiological drainage, sometimes followed by a laparotomy with surgical debridement [1,3,4,15,17].

Patient consent

Written informed consent was obtained from a legally authorized representative(s) for anonymized patient information to be published in this article.