A 31-year-old black woman was admitted with a history of recurrent chest pain, dysphagia, and heartburn, no complaint of fever or weight loss, and no significant past medical history. Patient denied swallowing any foreign body. She denied alcohol consumption. On admission, physical examination revealed normal sinus rhythm, normal pulse and blood pressure, normal temperature, and mild abdominal pain, without peritonitis. BMI: 22.1 kg/m2.
Lab results: Hgb: 14.4 g/dL; WBC: 9.3 x 103/muL; serum amylase: 653 U/L; serum lipase: 544 U/L; liver enzymes were within normal limits. CT scan revealed distal esophageal wall thickening and focal confined collection with air in the lower mediastinum surrounding esophagus, and pancreas showed neither parenchymal enlargement nor changes in density (Fig. 1). Endoscopy was performed, showing reddish budging of the cardia mucosa with purulent drainage orifice (Fig. 2). Endoscopic ultrasound revealed a 31.5 x 9.1 mm subepithelial anechoic oval lesion in distal lateral esophageal wall, 37 cm from incisors (Fig. 3). The fine-needle aspiration punction revealed only a few epithelial cells. The patient was treated with antibiotics and fasting, with the resolution of infection.
The main differential diagnoses of subepithelial esophageal lesions are leiomyoma, lipoma, varices, neural tumors (i.e., schwannoma, neuroma, or neurofibroma), granular cell tumor, inflammatory fibroid polyp, duplication cyst, lymphangioma, Brunner's gland hyperplasia, GIST, carcinoid, and metastatic carcinoma. After comprehensive imaging and repeated biopsies, the differential diagnosis remained unclear, although age and subsequent follow-up endoscopic ultrasound imaging argue for benign disease.
Along two-year follow-up, the patient had four readmissions with a similar clinical presentation (Fig. 4), all of which were treated with antibiotics and fasting. After infection resolution of the last episode, the patient was submitted to a minimally invasive McKeown esophagectomy (video 1) to avoid recurrence of symptoms. Surgery was carried out via right thoracoscopic approach and laparoscopy by a team of specialized esophageal surgeons. The patient was positioned in the semi-prone position after induction anesthesia and selective bronchial intubation. Esophageal dissection was somewhat challenging due to the surrounded fibrotic tissue following repeated inflammation of adjacent structures. The subepithelial lesion was not noted during surgery. A gastric tube was made with a laparoscopic stapler, and a mechanical cervical anastomosis was performed.
The patient made an uneventful postoperative recovery, being discharged from hospital 10 days after surgery. Pathological analysis of specimens revealed a heterotopic pancreatic tissue, containing acini, ducts, without islet cells (Fig. 5).
After a two-year follow-up, the patient had no complaints, with no recurrent symptoms.