Esophageal Perforation in Zollinger-Ellison Syndrome: A Scoping Review of Management and Outcomes.

Abstract

Esophageal perforation in Zollinger-Ellison syndrome (ZES) is an exceedingly rare and life-threatening manifestation of gastrinoma-related acid hypersecretion. While peptic ulceration and severe reflux are recognized complications of ZES, perforation represents the ultimate consequence of uncontrolled hyperacidity. Evidence remains confined to isolated case reports with no prior systematic synthesis. A scoping review was conducted in accordance with PRISMA-ScR and the Joanna Briggs Institute framework. A comprehensive search of PubMed, Embase, Scopus, and Web of Science was performed from database inception to September 2025. Eligible studies included any report describing esophageal perforation in confirmed or suspected ZES. Iatrogenic perforations were excluded. Data were extracted on patient demographics, clinical presentation, diagnostic modalities, management strategies, and outcomes. <b>S</b>even eligible cases published between 2001 and 2025 were identified. Patients were aged 44-63 years (4 males, 3 females). The distal esophagus was affected in approximately 70% of cases, usually after chronic peptic injury; three patients (≈43%) presented with spontaneous Boerhaave-type rupture. Computed tomography was diagnostic in all cases, and endoscopy was used in six. Surgical repair with mediastinal drainage or T-tube repair was the mainstay of management, while conservative therapy failed. Endoscopic stenting achieved successful leak control in one patient, whereas overall survival across all cases was approximately 86%, with one death due to delayed diagnosis and sepsis. Esophageal perforation in ZES represents a predictable but preventable endpoint of chronic acid injury. Rapid imaging, decisive surgical or endoscopic repair, and definitive acid suppression are essential to survival. Awareness of this rare complication among surgeons and gastroenterologists may facilitate early recognition and improve outcomes.