A 68-year-old male with past medical history of diabetes mellitus and two previous episodes of mild acute pancreatitis was admitted to the hospital with a 12-hour history of severe epigastric pain and nausea. He reported no recent alcohol consumption, and current medication was metformin. On initial physical examination, he had a temperature of 38.5°C, a heart rate of 94 beats per minute, and a respiratory rate of 26 breaths per minute. The patient had moderate epigastric abdominal tenderness without peritoneal irritation. Laboratory examination revealed white blood cell count 16,100/mm³, hematocrit 46.40%, glucose 26.9 mmol/L, serum amylase 1564 U/L, serum lipase 4116 U/L, serum alanine aminotransferase 380 U/L, serum lactate dehydrogenase 421 U/ L, alkaline phosphatase 248 U/L, direct bilirubin 3.0 mg/dL, BUN 12 mg/dL. Calcium, triglyceride, and electrolyte values were normal. Electrocardiography (ECG) and chest X-ray were normal. He was treated with intravenous fluids and pain relief medication. Several hours after admission the patient deteriorated, became hypotensive and dyspneic with increasing epigastric pain and peritoneal irritation, and required mechanical ventilation. Contrast-enhanced computed tomography of abdomen revealed gas in the portal vein, gastric pneumatosis, necrosis of the pancreas and pneumatosis intestinalis (Panels A and B).

The patient had acute necrotizing pancreatitis associated with hepatic portal venous gas and gastrointestinal pneumatosis. Despite aggressive resuscitation with fluids and broad spectrum antibiotics patient had an unrelenting, downhill course. Due to hemodynamic instability surgery could not be performed and patient died shortly thereafter.

Hepatic portal venous gas (HPVG) was described more than 55 years ago and is commonly associated with intramural bowel gas called pneumatosis intestinalis [1]. The frequency of detection of HPVG and pneumatosis intestinalis appears to be increasing. This increase may be the result of increased CT use. While the pathogenesis of HPVG is still unclear, and not yet well defined, various factors associated with it include bowel distention with mucosal damage, sepsis and gas embolization [2] [3]. Mechanisms proposed indicate that the gas enters the portal circulation through the veins or lymphatics of the intestinal wall reaching the hepatic veins through the hepatic sinusoids. [2] [4]. The overall mortality rate can be as high as 75–90%, depending on the underlying etiology [1] [5]. In last decades, mortality rate seems to be lower, and is considered to be the result of early diagnosis as well as its association with unimportant causes, in which conservative management is more beneficial [6]. The presence of certain clinical features in pneumatosis intestinalis may help to predict the necessity of surgical exploration. It has been reported that history and physical examination suggestive of an acute abdominal process, arterial pH <7.3, HCO3 <20 mmol/L, an elevated lactate level, an elevated serum amylase level, or the presence of portal venous gas are indicatives of the need of direct surgical intervention [7] [8] [9]. There are no specific clinical features described in HPVG to determine the treatment strategy and outcome. The patient, reported in this study, had a severe acute necrotizing pancreatitis (BISAP score of 3 points and modified Glasgow score of 6 points) and had an unrelenting, downhill course and died shortly thereafter. Algorithms to guide the management of patients with HPVG have been proposed [10]. The finding of HPVG alone cannot be an indication of urgent surgery but the presence of HPVG on plain X-ray, clinical or radiological signs of intestinal necrosis predicts a high mortality 75–85% and warrants urgent treatment. [6] [11] [12]. For patients without peritonitis mortality approaches 20–30% conservative management works well but clinicians are advised to have a low threshold for surgery and close monitoring of the patient [4] [13] [14]. However, there is limited data to determine the best treatment choice.

This case highlights a rare and potentially ominous complication of acute necrotizing pancreatitis.

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