Esophageal epiphrenic diverticulum treated with laparoscopic surgery in a patient with systemic sclerosis: A rare case report

Abstract

Introduction and importance: Systemic sclerosis is a disease characterized by autoimmune inflammation, fibrosis of the skin and internal organs, and vasculopathy. Diverticula found in the intestines are a common feature in patients with systemic sclerosis, but esophageal epiphrenic diverticulum is extremely rare. We present a rare case of esophageal epiphrenic diverticulum treated with laparoscopic diverticulectomy and Heller myotomy in a patient with systemic sclerosis.

Case presentation: A 73-year-old woman had been treated with prednisolone for diffuse systemic sclerosis with interstitial pneumonia. The patient had complained of chronic dysphagia and reflux symptoms. A small and asymptomatic diverticulum was first detected four years ago. Endoscopy repeated because of exacerbation of symptoms revealed an enlarged diverticulum. Therefore, the patient underwent laparoscopic diverticulectomy and Heller myotomy with partial fundoplication. Her postoperative course was uneventful, and her symptoms were relieved.

Clinical discussion: Although patients with systemic sclerosis commonly present with reflux esophagitis, they rarely develop achalasia-like change that leads to an esophageal diverticulum. There are several treatment options for esophageal diverticulum, including transhiatal surgery, thoracic surgery, or endoscopic treatment.

Conclusion: Clinicians must pay attention to patient symptoms because the worsening of dysphagia might suggest an underlying achalasia-like change or epiphrenic diverticulum in the esophagus. Surgeons should determine the treatment approach with considerations of the patient's background, the location and size of the diverticulum, and other factors.

Keywords: Diverticulectomy; Esophageal epiphrenic diverticulum; Heller myotomy; Laparoscopic surgery; Systemic sclerosis.

Fig. 1

Preoperative findings. a. Contrast esophagography shows a 50-mm epiphrenic diverticulum on the right wall of the lower esophagus. b. Contrast-enhanced computed tomography shows a diverticulum (yellow arrow) at the lower mediastinum. c. Upper endoscopy shows the orifice of the diverticulum (yellow arrow).

Fig. 2

High-resolution esophageal manometry shows persistent high internal pressure in the lower esophagus. Increased internal pressure is also observed in the area consistent with a diverticulum (30–36 cm from the incisors).

Fig. 3

Port placement and intraoperative findings. a. The operator used the two ports on the right side of the patient. b. The diverticulum (yellow arrow) was dissected from surrounding tissues and completely exposed. c. The diverticulum was transected using a linear stapler with endoscopic bougie. d. Intraoperative upper endoscopy showed complete resection of the diverticulum and no stenosis in the esophagus. e. The stapler line was covered and reinforced by suturing (yellow arrow). f. Heller myotomy was performed for over 2 cm from the EGJ on both the esophageal side and gastric side. g. Partial fundoplication and fixation to the diaphragmatic crus were performed.

Fig. 4

Postoperative images. a. Postoperative contrast esophagography shows smooth flow of contrast medium to the stomach. b. Photo of the specimen.