Anusha Agarwal, BS1, Samantha Sattler, BA1, Alla Turshudzhyan, DO2, Michael Schuster, MD3, Lee Shapiro, MD3, Daniel Cho, MD4, Micheal Tadros, MD, MPH, FACG1 1Albany Medical College, Albany, NY; 2University of Connecticut School of Medicine, Farmington, CT; 3Albany Medical Center, Albany, NY; 4Albany Medical Center, Schenectady, NY
Introduction: Scleroderma (SSc) is a chronic autoimmune disorder characterized by vascular and fibrotic changes affecting both skin and internal organs. Approximately 90% of patients with SSc present with gastrointestinal manifestations, with esophageal dysmotility being the most frequently reported. Severe forms of SSc may cause small intestinal dysmotility, leading to stasis, acute dilation, and pseudo-obstruction, a medical emergency associated with high morbidity. We present a rare case of acute gastric dysmotility and dilation manifesting as acute pseudo-obstruction in the setting of long-standing SSc.
Case Description/Methods: We present a 72-year-old female with previously well-controlled SSc on mycophenolate. Prior work-up had revealed dilated esophagus, megaduodenum, and delayed gastric emptying, but symptoms were stable on PPIs and low residue diet. She presented to clinic with two days of worsening nausea, non-bloody emesis, and severe abdominal pain with diffuse abdominal tenderness and distension on exam. The patient had no surgical history. Laboratory results were remarkable for leukocytosis and lactic acidosis. An abdominal CT revealed a markedly dilated stomach with possible volvulus (Fig.1.). Laparotomy confirmed acute gastric dilation and volvulus causing necrosis of greater curvature of the stomach. Gastric volvulus correction with partial gastrectomy was performed with subsequent placement of PEG tube for decompression. The patient’s condition stabilized, and PEG tube was later removed.
Discussion: This rare case of acute gastric dysmotility and dilation in a patient with SSc follows a pathophysiologic mechanism similar to that of small intestinal pseudo-obstruction. The mechanism may involve vascular ectasia, decreased blood flow, increased collagen deposition and fibrosis, auto-antibodies affecting myenteric neurons and neural cholinergic mediated contraction. Pseudo-obstruction in the context of SSc is a life-threatening complication that may severely reduce quality of life. Some cases have been precipitated by electrolyte derangements, anticholinergic medications, and elevation in inflammatory cytokines.
Since gastric dysmotility and potential atony in SSc are not frequently reported, we hope to make providers aware of this rare gastric manifestation of SSc. Given the severity of complications arising from gastric dysmotility, including volvulus, perforation, or gastric necrosis, providers should intervene in a timely manner when SSc patients present with signs of pseudo-obstruction.
Figure: CT of the abdomen without contrast, markedly dilated stomach (transverse view (A), coronal view (B)).
Disclosures: Anusha Agarwal indicated no relevant financial relationships. Samantha Sattler indicated no relevant financial relationships. Alla Turshudzhyan indicated no relevant financial relationships. Michael Schuster indicated no relevant financial relationships. Lee Shapiro indicated no relevant financial relationships. Daniel Cho indicated no relevant financial relationships. Micheal Tadros indicated no relevant financial relationships.
Anusha Agarwal, BS1, Samantha Sattler, BA1, Alla Turshudzhyan, DO2, Michael Schuster, MD3, Lee Shapiro, MD3, Daniel Cho, MD4, Micheal Tadros, MD, MPH, FACG1. P2066 - Acute Gastric Dilation: A Rare Complication of Scleroderma, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.