SMALL BOWEL VOLVULUS: UNUSUAL PRESENTATION OF PROGRESSIVE SYSTEMIC SCLEROSIS

Introduction: The gastrointestinal system is preceded by skin and joints in terms of frequency of organ system involvement in Progressive Systemic Sclerosis (PSS) and can severely impair quality of life. Dysphagia and heartburn are the most common symptoms while malabsorption or pseudo-obstruction are extremely rare. Case: 51 yr old white male with history of hypothyroidism and hypertension presented with diffuse abdominal pain, distension, nausea, bilious emesis with a 10 pound weight loss for 2 weeks. Upon admission, an abdominal CT scan revealed dilated small bowel with pneumatosis cystoides intestinalis. The patient was taken for an exploratory laparotomy that revealed a small bowel volvulus twisted on its mesentery, which was reduced. Patient then continued to present with symptoms consistent with recurrent small bowel dilatation over the next 4 months with concurrent Raynaud's phenomenon and digital ulceration. He was also found to have a pleural effusion with bilateral pulmonary infiltrates with honey comb appearance with restrictive pattern of lung disease on PFTs. Transaminases & aldolase were elevated consistent with myositis. The above findings along with positive ANA and anti-Scl 70 confirmed PSS. The small bowel dilation was treated conservatively. Abdominal CT scans and small bowel follow through confirmed persistent small bowel loop dilation without obstruction. Rotating course of antibiotics were given for a period of 1 month with out response. Patient was now thought to have chronic pseudo- intestinal obstruction. Octreotide & TPN was started with resolution of symptoms. Conclusions: The esophagus is the most common organ involved in 90% of patients with small bowel involvement seen in 40% of PSS. This unique case depicts a rare triad of chronic intestinal pseudoobstruction, small bowel volvulus, and pneumatosis intestinalis in the setting of PSS without esophageal manifestations. The occurrence of small bowel volvulus and pneumatosis cystoides intestinalis as an initial presentation of PSS is rare with only a few case reports reported so far.