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Pneumatosis Intestinalis With Fatal Air Emboli in a Scleroderma Patient FREE TO VIEW

George Adesina, MD; Callan Mason, MD; Adam Mora, MD
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Baylor University Medical Center, Dallas, TX

Chest. 2013;144(4_MeetingAbstracts):340A. doi:10.1378/chest.1702450
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SESSION TITLE: Critical Care Student/Resident Case Report Posters III

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pneumatosis intestinalis (PI) is described as gas within the walls of the intestine. This radiological sign may herald significant morbidity and mortality.

CASE PRESENTATION: An 87 year old woman with scleroderma reported to her PCP with a four day history of worsening lower abdominal pain, anorexia, and intermittent non-bloody diarrhea. She was in acute renal failure and was sent to the emergency department where she acutely decompensated with hypotension and acute respiratory failure. She had a distended and rigid abdomen. An NG tube placed to suction drained bright red blood. Abdomen/pelvis CT revealed extensive PI involving the right colon and majority of the small bowl as well as portal venous gas extending to the splenic vein and multiple mesenteric veins. Air was identified within the main pulmonary artery and right atrium. Significant lab values showed metabolic acidosis, thrombocytopenia and elevated INR. She was admitted to our ICU for aggressive IV fluid resuscitation, levophed and continued empiric antibiotics. A code blue was called approximately 30 minutes later when she developed PEA. She never regained a pulse and the patient’s family requested that life sustaining measures stop.

DISCUSSION: PI can be seen in an array of disease processes. Pathogenesis is poorly understood. One hypothesis postulates that gas enters the intestinal wall through small breaches in the serosal or laminal surfaces. These areas of disrupted mucosal integrity allow gas to track down the mesenteric arteries, dissect throughout the intestinal wall and spread to distant sites. (1) The idea of a disrupted mucosal integrity however simple may in fact be the unifying connection between PI and scleroderma. Higher risk of intestinal tearing in scleroderma patients may be explained by Raynaud-like vasospasms causing intestinal ischemia and stasis leading to an increase in intramural pressure and overgrowth of gas forming bacteria. (2) Clinical manifestations of PI range from asymptomatic patients to extensive damage culminating in death. Our case was complicated by an air embolism likely derived from the PI and rapid deterioration due to suspected DIC as indicated by bleeding, acute thrombocytopenia and an elevated INR. This case illustrates Scleroderma producing PI leading to air embolism and DIC resulting in death.

CONCLUSIONS: PI associated with scleroderma requires a high index of suspicion. Abdominal symptoms may be an indicator of potential catastrophic events and should prompt abdominal imaging.

Reference #1: AU Pieterse AS, Leong AS, Rowland R. The mucosal changes and pathogenesis of pneumatosis cystoides intestinalis. Human Pathology 1985; 16(7):683.

Reference #2: Lock G, Hostege A, Lang B, Scholmerich J. Gastrointestinal manifestations of progressive systemic sclerosis. American Journal Gastroenterology 1997; 92(5):763-71.

DISCLOSURE: The following authors have nothing to disclose: George Adesina, Callan Mason, Adam Mora

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