Portal venous gas in a girl with chronic intestinal pseudoobstruction

Hepatic portal venous gas (HPVG) is associated with various fatal conditions requiring urgent surgical intervention. In patients with chronic intestinal pseudoobstruction (CIPO), HPVG results from intestinal dilation. CIPO is a condition that is generally not surgically remediable; however, in emergency cases, an operative approach is often considered to prevent additional complications or to restore intestinal propulsion. However, surgery in these patients is associated with high postoperative morbidity and mortality rates and frequent reoperation. Intestinal resection and adhesion formation can potentially worsen the underlying dysmotility and are associated with refractory postoperative ileus, which often requires reoperation. Here, we present a case of HPVG in a patient with chronic intestinal pseudoobstruction (CIPO), treated conservatively and discuss the management of patients with HPCG.