The patient's GI symptoms resolved postprocedure. After she could tolerate a normal diet, she was discharged with an aggressive oral bowel regimen and motility specialist follow-up.
SSc can affect any part of the GI tract, resulting in fibrosis and dysmotility of the gut wall. SSc's GI manifestations account for 6%–12% of the mortality rate of these patients.2 The GI pathophysiology of SSc is explained by Sjogren's theory.3 In response to an unknown initial injury, collagen deposits in blood vessels within the gut wall.4 This causes recurrent vascular derangement, compression of nerves, and autoimmune-mediated injury.3 Immune cells infiltrate the smooth muscle, causing dysfunction, atrophy, and fibrosis.
The most common GI manifestation of SSc is esophageal dysfunction due to fibrosis of the lower two-thirds of the esophagus. It can lead to an incompetent lower esophageal sphincter, inadequate peristalsis, GERD, and/or stricture formation.5 Another common site of SSc involvement is the small intestine, resulting in hypomotility and stasis. Consequently, 10%–30% of these patients have SIBO, leading to malabsorption and diarrhea.6
Less commonly, SSc results in colonic hypomotility. Symptoms most often include bloating, abdominal pain, and constipation, which can ultimately result in overflow diarrhea that passes around the hardened stool. The progressive hypomotility of the colon can result in pseudo-obstruction, which refers to obstruction without a mechanical cause. However, in rare instances, a true mechanical obstruction can occur because of stool completely blocking the large intestine, but few case reports exist in the literature.1 This rarity may be because SIBO and malabsorption lead to small intestinal diarrhea, which often mitigates stool impaction.2 Nonetheless, for patients with severe colonic SSc, stool transit can be significantly impaired, allowing a fecal bezoar to accumulate over time despite overflow diarrhea.
Regarding management of bezoar-induced obstruction in patients with SSc, physicians should consider oral osmotic laxatives such as PEG, prokinetic agents, and, if the obstruction is distal to the transverse colon, mineral oil enemas.7 If conservative measures fail, colonoscopic intervention may be successful.8 In cases of mechanical obstruction, in all patients, surgical management is reserved for patients who do not respond to endoscopic treatment or who have bowel perforation. Limited resection of the colon outside of these circumstances is unlikely to be helpful, especially in patients with SSc due to the pan-colonic fibrosis inherent to the disease.9 Surgery is therefore almost never performed on these patients. For prevention of bowel obstruction, the same conservative measures (PEG, prokinetic agents and mineral oil enemas) can be used in lower doses and frequencies, both in patients with SSc and other at-risk patients.10
Because of its rarity, recurrence rates—and epidemiologic data in general—for true bowel obstruction in patients with SSc are not known, although some data exist pertaining to pseudo-obstruction. One study showed that the mortality rate from acute intestinal pseudo-obstruction in patients with SSc was 16%.11 Female patients who presented with pseudo-obstruction were more likely to have recurrence than male patients, although male patients had a higher mortality rate after pseudo-obstruction [ibid].
We present a rare case of a true mechanical bowel obstruction secondary to fecal bezoar formation in a patient with systemic SSc. The patient's obstruction was successfully relieved with medical and endoscopic therapy. Although most data on SSc-related bowel obstructions focus on pseudo-obstructions, our report highlights the potential for true mechanical obstruction in the GI tract. We demonstrate that endoscopic intervention can successfully disimpact obstructing fecal bezoars in patients with SSc.
Author contributions: R. Sarnoff and B. Girmay wrote the manuscript. R. Mocharla edited the manuscript. All other authors contributed equally to the manuscript. R. Sarnoff is the article guarantor.
Financial disclosure: None to report.
Informed patient consent was obtained for this case report.
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© 2019 by Lippincott Williams & Wilkins, Inc.
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