Zollinger-Ellison Syndrome Presenting as Esophageal Rupture

Abstract

Zollinger-Ellison syndrome (ZES) is caused by a gastrin-secreting neuroendocrine tumor and classically presents with distal duodenal ulcers, severe esophagitis, epigastric pain and diarrhea. ZES is a rare disorder with incidence of 1-3 cases per million. Here we discuss a case of ZES presenting with Boerhaave syndrome, illustrating the importance of early clinical suspicion and prompt diagnosis in order to prevent ZES complications. A 50-year-old diabetic woman was admitted for intractable abdominal pain and vomiting after having intermittent symptoms for over a year. Twenty-four hours after admission she developed sudden hypoxia after emesis. CT chest showed bilateral hydropneumothorax and mediastinal free air. Urgent EGD revealed severe circumferential ulcerative esophagitis with perforation 1-2cm above GE junction, multiple distal duodenal ulcers, and a small submucosal lesion in the duodenal bulb. A partially covered esophageal metal stent was deployed sealing the perforation, and the patient underwent chest tube placement and laparoscopic mediastinal wash-out. Her hospital course was complicated by severe bleeding from a distal duodenal ulcer requiring endoscopic therapy for hemostasis. Four weeks post placement, her embedded partially covered stent was removed using “stent-in-stent” technique (1 week of fully covered esophageal metal stenting inside the partially covered stent). Leak closure was confirmed radiographically. Work up for ZES was initiated with a fasting gastrin level, off of PPIs, of 784 and an Octreoscan revealing abnormal uptake in the duodenal bulb. After evaluation by bariatric surgery, she underwent D2 Gastrectomy. The final pathology returned positive for a low-grade gastrinoma with 1 of 15 lymph nodes positive for metastatic disease. Her nutrition was optimized, her symptoms improved and was discharged with close follow-up. Esophageal perforation is a late manifestation of unrecognized ZES and is described in only a handful of cases. Other esophageal manifestations, such as heartburn and esophagitis, are seen in up to 45% of patients while strictures are found in 4-10%. Given the non-specific symptoms of ZES, prompt diagnosis is often difficult. Patients with intractable symptoms, severe esophagitis and multiple distal duodenal ulcers, should raise clinical suspicion for ZES followed by expedited workup in order to prevent many of the complications seen in our patient.Figure: Gastrinoma, endoscopic view.Figure: Esophageal perforation.Figure: Stent-in-stent.