Gastric pneumatosis (GP) and hepatic portal venous gas (HPVG) have typically been thought of as an ominous radiological sign associated with a grave prognosis, and the observation of HPVG on plain abdominal radiography, ultrasonography, or computed tomography is viewed as a significant finding. It is often associated with severe or potentially lethal conditions warranting urgent diagnosis and possible surgical intervention. Early studies of HPVG based on plain abdominal radiography found an associated mortality rate of 75% primarily due to ischemic bowel. However, modern abdominal computed tomography (CT) has resulted in the detection of HPVG in an increased proportion of nonfatal and benign conditions. We report a nonfatal case of HPVG in a patient with Noonan’s syndrome due to acute gastric dilatation in the setting of gastric outlet obstruction caused by a congenital band that is extremely rare in adults. 1. Introduction Hepatic portal venous gas (HPVG) was first described more than 55 years ago by Wolfe and Evans and was presented as a radiologic sign associated with more than 75% of mortality rate in infants with necrotizing enterocolitis (NEC) [1, 2]. Since that time, HPVG has been associated with numerous other underlying abdominal diseases, ranging from benign causes to potentially lethal diseases requiring prompt surgical intervention. Moreover, HPVG is recognized not as a specific disease entity but rather as a diagnostic clue in patients with acute abdominal pathology. Although early reports of HPVG estimated a mortality rate of 75% to 80%, more recent studies suggest mortality rates of 25% to 35% [3–5]. The observed reduction in mortality may be attributed to the increased availability of more sensitive diagnostic imaging modalities (i.e., ultrasonography (US) and computed tomography (CT)) that can detect even minute quantities of air in the portal system. In addition, there is also an increase in the proportion of nonfatal conditions reported with HPVG without associated mesenteric ischemia [6]. We report a case of HPVG in a patient with Noonan’s syndrome due to acute gastric dilatation in the setting of gastric outlet obstruction caused by a congenital band that is extremely rare in adults. This study reviews the clinical data in adults from the literature and discusses the management of underlying disease. 2. Case Report and Management A 64-year-old Caucasian female with a history of Noonan’s syndrome presented with sudden onset of coffee ground emesis and without any history of black tarry stool. She was recently treated for severe
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