Therapeutic Utilization of In Vivo Histopathology in Scleroderma

Abstract

Esophageal involvement in scleroderma is characterized by atrophy of the smooth muscle of the muscularis propria. This atrophy leads to insufficiency of the lower esophageal sphincter, increased acid reflux and poor acid clearance. Scleroderma patients have a greater prevalence of Barrett's esophagus (BE) and esophageal adenocarcinoma compared to the general population. When considering endoscopic mucosal resection (EMR), there is concern for the risk of perforation due to the atrophy and decreased wall thickness seen in scleroderma. We present a case of endoscopic mucosal resection of an early esophageal adenocarcinoma in a scleroderma patient with diagnosis aided by confocal endomicroscopy and risk stratification performed with volumetric laser endomicroscopy (VLE). 65 year old male with scleroderma presents with weight loss and anemia. On endoscopy, there were mucosal changes consistent with long-segment Barrett's esophagus and an ulcerated 1 cm lesion in the distal esophagus, which under confocal endomicroscopy showed dysplasia. On endoscopic ultrasound (EUS), a hypoechoic lesion measured up to 3 mm in thickness and 9 mm in length with possible extension into the submucosa. There was no surrounding lymphadenopathy. VLE was used to evaluate the patient's submucosal anatomy and assess the risk of EMR. This showed thinning of the submucosa and a heterogeneous appearance to the muscularis propria with disruption of the outermost layer. There was also full visualization of the adventitia which is often not visible via VLE in a normal esophagus (Figure 1). Despite this thinning, we felt there was adequate submucosa for EMR. Band ligation and snare mucosal resection were successfully performed. Pathology showed moderately differentiated adenocarcinoma invading into the submucosa (pT1b SM1) with lateral and deep margins negative for carcinoma. The patient was discharged home without complication. The esophagus is one of the most commonly affected regions of the gastrointestinal tract in scleroderma. A case review of 92 cases found nearly the entire group had atrophy of the circular smooth muscle while 2/3 also had concurrent atrophy of the longitudinal smooth muscle, particularly in the lower esophagus. Volumetric laser endomicroscopy was helpful in assessing the submucosal changes in the esophageal muscle affected by this patient's scleroderma, allowing us to risk stratify and ensure safe resection of an early esophageal adenocarcinoma.Figure 1