Systemic Scleroderma and Colonic Volvulus

Abstract

Introduction: Patients with scleroderma may have significant gastrointestinal symptoms due to intestinal dysmotility and pseudo-obstruction. Abnormal bowel motility is a risk factor for volvulus, a lifethreatening condition with a similar presentation as pseudo-obstruction. The literature shows the rare occurrence of colonic volvulus in scleroderma. The aims of this study were to examine the occurrence of colonic volvulus in scleroderma and its associated clinical features. Methods: Patients with documented ICD-9 or ICD-10 codes for both scleroderma and colonic volvulus were identified from the electronic and archived medical records using institutional software between July 1950 and January 2017. Records eligible were limited to patients between the ages of 18 and 99 at time of diagnosis. This data was combined with similar patients from the medical literature using PubMed. Results: From our database, 8 patients were identified with concomitant scleroderma and colonic volvulus. In combination with the 8 cases from the literature, a total of 16 patients were analyzed. The mean age at diagnosis of volvulus was 54.3 (SD +/-15.5) and the mean disease duration from onset of scleroderma diagnosis to colonic volvulus was 16.1 years (SD +/-12.3). There were 11 females and 5 males, with most having limited disease versus diffuse (81.3% vs. 18.7%, respectively). The most frequent associated disease was universally Raynaud's phenomenon (n=16), followed by interstitial lung disease (n=6). As for chronic GI symptoms, half the patients had documented gastroesophageal reflux disease (GERD), and 37.5% had chronic constipation. The most common site for initial volvulus was sigmoid (50%), followed by cecum (25%), and the majority required surgery for successful final treatment of volvulus (81.3%). While half the patients had a good outcome, 37.5% had poor outcomes including 2 deaths. Conclusion: While scleroderma with colonic volvulus is a rare occurrence, it is critical to consider this diagnosis in scleroderma patients presenting with symptoms of pseudo-obstruction because it is lifethreatening and requires surgery. The misdiagnosis of pseudo-obstruction in a patient with colonic volvulus can be catastrophic.