TBA (19A180)

Pneumatosis cystoides intestinalis in a patient with advanced scleroderma, a case report


Shamma Ahmad Al-Nokhatha, Muddassar Ahmad, Diana Gheta, David Kane, Ronan H Mullan


Department of Rheumatology, Tallaght University Hospital, Dublin, Ireland


A 74-year old female fulfilling both anti-centromere positive limited systemic sclerosis and seropositive rheumatoid arthritis diagnostic criteria, presented to surgical services with nausea, vomiting, abdominal pain and constipation.


Examination revealed abdominal distension without peritonism. Radiographic imaging showed volvulus with intestinal obstruction. Emergency laparotomy revealed a large portion of dilated and ischemic bowel without perforation. Bowel resection with end stoma formation was performed.


Four months later the patient was readmitted with recurrent abdominal discomfort and subacute obstruction. Abdominal computed tomography (CT) showed both small bowel pneumatosis and residual small volume pneumoperitoneum attributed to recent surgery. Conservative management with bowel rest, fluid, electrolyte supplementation and parenteral nutrition were instituted with full resolution of symptoms. One-month later, the patient represented with a second episode of subacute small bowel obstruction. Repeat imaging showed interval progression of small bowel pneumatosis and new small bowel dilatation (Figure 1A, B).


Rheumatology services were contacted and the patient was managed using high-flow oxygen therapy and antibiotics (rifaximine) to manage anaerobic bacterial overgrowth and the somatostatin analogue octreotide for intestinal pseudoobstruction. The patient’s bowel symptoms resolved and she was weaned off oxygen. To date, her symptoms have not recurred.


Scleroderma is an autoimmune connective tissue disease affecting multiple organ systems. Gastrointestinal features are common, ranging from mild gastroesophageal reflux disease to life-threatening bowel dysfunction. Pneumatosis cystoides intestinalis (PCI) is a rare intestinal feature of scleroderma, characterized by gaseous cysts in intestinal submucosa, subserosa and surrounding tissues. PCI is presumed to occur secondary to overgrowth of anaerobic gas-forming bacteria, which proliferate as a result of bowel stasis in the setting of intestinal fibrosis. Treatment strategies involve creating a toxic environment for anaerobic bacteria using rotating antibiotics and high-flow oxygen, as well as the use of pro-kinetic agents. An increased awareness of PCI among treating rheumatologists, along with the prompt institution of conservative management strategies, can prevent the requirement for surgical intervention leading to improve long-term outcomes.