Jejunal Perforations in Zollinger-Ellison Syndrome: Expect the Unexpected

The triad of refractory gastric hypersecretion along with severe peptic ulceration in the presence of a gastrinoma was first described by Zollinger and Ellison in 1955.1 GI perforation as the initial manifestation of Zollinger-Ellison syndrome (ZES) is estimated to be only around 7 per cent2 and presumed to be even lower since the advent and widespread use of proton-pump inhibitors (PPIs). After Institutional Review Board approval by our ethical institutional committee, we conducted a systematic literature review of ZES cases presenting with GI perforation, published in the English literature dating back to 1991, when Waxman first published his pioneering article about peptic ulcer perforation as the presentation of ZES.2 Because this is a retrospective review of previously published case reports, a waiver of consent was granted. [...]this study shows that perforation can occur as an initial presentation in ZES patients without prior symptoms at almost any age.