State University of New York Upstate Medical University Syracuse, New York
Sundus Mian, MD1, Hiba Bilal, DO1, Michelle Bernshteyn, MD2, Andras Perl, MD, PhD1; 1State University of New York Upstate Medical University, Syracuse, NY; 2SUNY Upstate Medical University, Syracuse, NY
Introduction: Scleroderma is a progressive autoimmune disorder that causes microvasculopathy and organ fibrosis leading to deformity and dysfunction. The gastrointestinal (GI) system is affected in up to 90% of patients. Vascular damage induces enteric neuropathy and myopathy which causes GI hypomotility and intestinal bacterial overgrowth. Upper GI manifestations include gastro-esophageal reflux disease (GERD), Barrett’s esophagus, and gastroparesis. Lower GI manifestations include small intestinal bacterial overgrowth (SIBO), pseudo-obstruction, malabsorption, and rarely pneumatosis intestinalis (PI) in advanced disease.
Methods: A 55-year-old woman with limited scleroderma (CREST syndrome) presents with three days of constipation, nausea, and vomiting. She suffered from CREST syndrome for 26 years with recent advancing skin, lung, and GI disease. Computed Tomography (CT) of the abdomen/pelvis demonstrated diffuse bowel distension with intramural gas-filled sacs without free air or fluid. Labs revealed normal white blood cell count and lactate. She lacked fever and abdominal tenderness. Conservative measures of bowel rest, serial abdominal exams, and monitoring for bowel movement were pursued. Rheumatology was consulted and felt her PI was a complication of advanced scleroderma. The patient was given one week of Metronidazole and Erythromycin and high flow nasal cannula (HFNC) oxygen therapy inpatient. She was discharged after constipation resolution. Discussion: About 85% of PI cases are secondary to other diseases such as scleroderma while 15% are idiopathic. Secondary PI follows a subacute, progressive course. PI typically involves the small and/or large intestine. It is associated with pseudo-obstruction and overgrowth of intestinal bacteria. CT abdomen/pelvis is the imaging of choice to identify the characteristic gas-filled cysts within the bowel wall which are thought to be due to bacterial invasion and gas production. Management involves ruling out life-threatening surgical emergencies such as bowel ischemia, perforation, or peritonitis. PI carries an overall estimated mortality of 20-25%. Afterward, PI is managed conservatively with bowel rest to relieve pseudo-obstruction, antibiotics such as Metronidazole to target SIBO, use of motility agents (i.e. Erythromycin, Metoclopramide, or Octreotide), and HFNC or hyperbaric oxygen therapy to resorb intramural gas. However, the efficacy of these therapies are controversial due to lack of substantial evidence.
IMAGE 1 shows the axial view of the CT abdomen/pelvis without contrast which highlights dilated large bowel loops with Pneumatosis intestinalis in the wall of the ascending colon.
IMAGE 2 shows the coronal view of the CT abdomen/pelvis without contrast which highlights dilated large bowel loops and Pneumatosis intestinalis in the wall of the ascending colon.
Disclosures: Sundus Mian indicated no relevant financial relationships. Hiba Bilal indicated no relevant financial relationships. Michelle Bernshteyn indicated no relevant financial relationships. Andras Perl indicated no relevant financial relationships.