Gastroenterology Case Scenario An Uncommon Cause for a Common Scenario

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Waleed Mahrous

Case Scenario

A 59-year-old woman presented to the emergency department with nausea and epigastric pain for 2 days preceded by 2 weeks of anorexia. Several years ago, she was diagnosed as glucose intolerant, but did not receive regular follow-up. On examination, her temperature was 34.8°C and her blood pressure was 81/53 mm Hg. She had icteric sclera, palpated tenderness in the upper right quadrant of the abdomen, and hepatomegaly (liver span of 14 cm at the midclavicular line).

Case Scenario

Blood tests showed a leukocyte count of 5700/mm3, (55% neutrophils; 19% lymphocytes), a glucose level of 347 mg/100 mL, an aspartate aminotransferase level of 10,920 U/L, an alanine aminotransferase level of 3,651 U/L, a total bilirubin of 4.8 mg/dL, and a creatinine level of 3.4 mg/100 mL. Plain film of the chest was normal, but plain film of the abdomen revealed a mottled appearance (Figure A, arrowheads) with branching radiolucencies (Figure A, arrows) in the liver. Hypotension persisted despite maximum inotropic support and the use of broad-spectrum antibiotics. She was hemodynamically unstable and died within 20 hours. A blood culture grew Klebsiella pneumoniae.

Dr Waleed Kh. Mahrous

Dr Waleed Kh. Mahrous

Case Scenario

Case Scenario

What's your diagnosis?

Case Scenario

Case Scenario

Noncontrast computed tomography showed extensive replacement of the liver parenchyma with interstitial (Figure B, arrowheads) and bilateral peripheral portal venous gas (Figure B, arrows).

The patient was diagnosed with emphysematous hepatitis (EH), which is equivalent to a severe gas-forming liver infection and septic shock.

In contrast with other affected organs, such as emphysematous pyelonephritis or cholecystitis, emphysematous changes in the liver are extremely rare.

Case Scenario

EH is fundamentally different from gas-forming pyogenic liver abscesses, which are usually characterized by cluster signs, septal breakage, or collections of pus.

Computed tomography would show hepatic portal venous gas and "alveolarization" of the hepatic parenchyma if the hepatic structure adjacent to the portal system were also destroyed.

To our knowledge, the previously reported cases of EH were all fatal in a fulminant course within 8-72 hours after arriving at the hospital.

The potential etiologies in these reported cases were bacteremia caused by suppuration from neighboring organs or following a hepaticojejunostomy owing to orthotopic liver transplantation or lobectomy.

Case Scenario

The pathogenesis of EH remains unclear; however, some factors have been implicated in our case, including gas-forming bacteria (K. pneumoniae) associated with an intra-abdominal infection, a high serum glucose level, a thrombus or gas embolus (during tissue necrosis and disseminated intravascular coagulation) that in turn alters the bowel wall, permitting intraluminal gas to pass through the portal venous system into the liver parenchyma in a vicious cycle.

There is no definite treatment guideline for EH.

Portal venous gas

Portal venous gas is merely the accumulation of gas in the portal vein and its branches. It needs to be distinguished from pneumobilia, pattern of gas (i.e. peripheral in portal venous gas, central in pneumobilia).

Although traditionally considered a harbinger of death, portal venous gas is increasingly recognized in a variety of conditions, many of which do not carry as high mortality or morbidity risks.

Portal venous gas

Adult Alterations of bowel wall

Ischaemic bowel (usually mural gas as well as mesenteric gas : mortality of 75 - 90% : but gas is not an independent predictor)

Necrotic / ulcerated colorectal carcinoma (CRC)

Inflammatory bowel disease (IBD) Perforated peptic ulcer

Portal venous gas

bowel luminal distention Iatrogenic gastric and bowel dilatation

(e.g upper and lower endoscopic procedures, enemas)

Paralytic ileus / mechanical obstruction Acute gastric dilatation Barotrauma.

Portal venous gas

Intra-abdominal sepsis Diverticulitis Pelvic abscess Cholecystitis and Cholangitis Appendicitis

unknown mechanism Pneumatosis intestinalis Chronic obstructive pulmonary disease (COPD) Corticosteroid usage