Introduction: Dermatomyositis is an autoimmune disease characterized by proximal myopathy, cutaneous Gottron papules and heliotrope rash; intestinal involvement associated to acute vasculitis is less common but could be a life-threatening condition.
Methods: A 21-year-old woman, affected by dermatomyositis, presented to our attention with a three-day story of severe abdominal pain, no bowel movement and biliary vomit. She was diagnosed with acute abdomen. A CT scan with bowel contrast demonstrated the presence of a leakage from the retroperitoneal aspect of duodenum. The surgical and clinical management in the light of literature review is presented.
Results: Our first approach consisted in primary repair of the duodenal perforation with omentopexy. Post-operative course was complicated by hemorrhage. A reintervention showed a new perforation associated with multiple ischemic intestinal areas. We performed a gastroenteric anastomosis with functional exclusion of the damaged duodenum and positioning of drainages to create a biliary fistula. A nutritional enteric tube and an open abdomen vacuum-assisted closure system to monitor the fistula creation and to prevent abdominal contamination and collections were positioned. To reduce the amount of biliary leakage, a percutaneous transhepatic biliary drainage was placed, with progressive fistula flow disappearance in four months.
Conclusions: In patients with dermatomyositis, when clinical findings and symptoms suggest abdominal vasculitis, it is very important to be aware of the risk of bowel and particularly duodenal perforations. Open abdomen treatment favors control of contamination by gastrointestinal contents, offers temporary abdominal closure, helps ICU care and delays definitive surgery.
Keywords: Acute abdomen; Dermatomyositis; Duodenal perforations; Emergency surgery; Intestinal ischemia; Open abdomen; Retroperitoneal air.