Esophageal perforation: a rare complication of Zollinger-Ellison syndrome

Spontaneous perforation of the esophagus is a rare manifestation of Zollinger-Ellison syndrome (ZES). Failure to recognize its existence can lead to an unsuccessful treatment of the esophageal perforation. We present a rare case of reflux esophagitis-induced esophageal perforation in a patient with ZES. Presence of a gastrinoma should be considered when recurrent or complicated reflux esophagitis is encountered.     

External perforation of the esophagus: Case report

A case of external esophageal perforation treated conservatively is reported with a brief review of the literature.     

Contributions of the Zollinger-Ellison syndrome

The salient contributions of the Zollinger-Ellison syndrome have made it unique. No pancreatic endocrine tumor described before (insulinoma) or subsequently (glucagonoma, somatostatinoma, vipoma, pancreatic-polypeptidoma) has been the topic of such a variety of studies, or has been such an inspiration and rich source of new ideas for investigation and ultimate improvement in patient care.     

Conservative surgery in the Zollinger-Ellison syndrome

Laparotomy failed to show an obvious primary lesion in two patients with the Zollinger-Ellison syndrome (ZES), one of whom had metastatic gastrinoma in a solitary lymph node which was removed. Both patients underwent a truncal vagotomy and pyloroplasty. Postoperative acid studies have shown a marked and sustained diminution in acid hypersecretion which was particularly striking in the patient with a solitary metastatic lymph node. The patient without a detectable lesion is now well-controlled on half the dose of cimetidine required pre-operatively and the other no longer requires therapy. We believe that patients in whom pre-operative investigations have shown no evidence of extensive metastases should undergo laparotomy in the hope of finding a solitary primary or isolated metastatic lesions unassociated with an obvious primary in the pancreas. Available evidence suggests that simple removal of such lesions offers the patient an excellent chance of long-term remission. However, if no such lesion is discovered, vagotomy may be the logical procedure to assist in the subsequent pharmacological control of the acid hypersecretory state.     

Occult coin perforation of the esophagus

Only a small number of ingested foreign bodies perforate the esophagus and even a smaller fraction migrate extraluminally with no symptoms. Both of these events are even rarer after coin ingestion. Between 1972 and 1984, three children (16 months to 5 years), who had unabating upper respiratory symptoms, were found to have ingested coins. In only one child could the time interval between ingestion and appearance of symptoms (3 years) be established, and in this child the diagnosis was delayed because of failure to x-ray the chest after an ingestion episode. Surgical evaluation included chest/neck films: tracheal and esophageal shadows were separated by the object and a soft tissue mass; esphagogram: deviation of the esophagus, irregularity of the lumen, and no leak or tracheoesophageal fistula (TEF) and esophagoscopy: mucosa was intact with no direct visualization of the coin. Treatment consisted of exploration: cervical, one; thoracic, two; localization: coin in extraluminal granulomatous soft tissue; removal: without resection of the soft tissue mass or esophagus, and drainage: penrose, one; chest tube, two. There was no morbidity or mortality from 1 to 13 years later. Though generally harmless, ingested coins are capable of penetrating the esophagus. Sporadic literature reports confirm that the clinical findings and operative results are typical of this seemingly intermediate stage between perforation with mediastinitis and perforation with TEF.     

Zollinger-Ellison syndrome: special considerations.

Twelve patients with Zollinger-Ellison syndrome and one patient with WDHA syndrome are reviewed. Three of the Z-E patients exhibited MEA, two having hyperinsulinism and one hyperparathyroidism. Ages ranged from nine to 71 years. Diagnosis of Z-E syndrome was established from history, gastric acid secretion, radiologic studies, serum gastrin measurements and from actual tissue biopsy in 10 of the 12 patients. Total gastrectomy was performed in 8 of the 12 Z-E patients, with abolition of the ulcer diathesis in all. However, in none of our patients was there objective evidence of subsequent tumor regression. Three patients remain alive. Four died of tumor, one from post-total gastrectomy complications, one from post-subtotal gastrectomy in another hospital, two from ulcer hemorrhage, and one from electrolyte imbalance with autopsy diagnosis of Z-E tumor. A patient is recorded in detail who exhibited both hyperinsulinemia and hypergastrinemia from a malignant islet cell tumor, had the tumor "debulked" four times over a 14 year period and whose hepatic metastases were temporarily abolished by streptozotocin infusion. The question is raised regarding relationships between chronic organic hyperinsulinism and subsequent hypergastrinemia.     

Zollinger-Ellison syndrome: a personal perspective

The odyssey of Zollinger-Ellison Syndrome is one of the most interesting in surgery. From the initial recognition of two patients with severe peptic ulcer disease in 1955 sprung an international dialogue on this unique disease culminating in discovery that gastrin was the hormone secreted by these pancreatic and duodenal tumors. The impact was the birth of a new area of science, "gastrointestinal endocrinology." Initially, surgeons were challenged by the complexity of the patients and the need to perform total gastrectomy to prevent death from complications of the severe ulcer disease. Later, after the discovery of proton pump inhibitors, total gastrectomy was no longer needed and the surgeon could focus treatment on tumor removal added by radioimmunoassay for gastrin and new imaging modalities. Today, we recognize that all gastrinomas have malignant potential; early surgical removal can reduce the incidence of metastases and improve survival, independent of biochemical cure, in both sporadic and familial forms of the disease.     

Gastrinoma: diagnosis and management of Zollinger-Ellison syndrome

Diagnosis and management of Zollinger-Ellison syndrome (ZES) do not appear to be established even today. In the present study, 143 cases of ZES (including 12 of author's experience) were collected from Japanese literature and analyzed, and diagnosis and management were discussed. (1) Although 43% of patients showed high gastrin level (greater than or equal to 500pg/ml), it is worth to be stressed that gastrin level was below 300pg/ml in 29% of patients. (2) Secretin provocation test was essential for diagnosis and particularly useful for detecting ZES with low gastrin level. (3) Angiography and/or blood samplings of PTPC were widely used for localizing gastrinoma but the informations thus obtained were not always of help for complete removal of tumors since malignant or multiple gastrinomas were frequent. (4) An aim of management for ZES consists of complete removal of tumor but it is not always feasible. Therefore, removal of target organ (total gastrectomy) should be routinely added for long-term cure. (5) Value of chemical gastrectomy (H2-receptor antagonist) is still a matter of pros and cons but substantial value of this drug should be considered adjunct to surgery.     

Cimetidine in the treatment of Zollinger-Ellison syndrome

From 1977 to 1983 we used cimetidine as primary therapy for 17 patients with the Zollinger-Ellison syndrome. All patients were treated with oral doses of cimetidine, 300 to 600 mg, four times a day, unless symptoms of hyperacidity developed or until evidence of a potentially resectable tumor became available, at which time they underwent definitive therapy. Eleven (65 percent) had no response to cimetidine therapy, 7 of whom had symptomatic recurrent ulcers, 3 of whom had esophagitis, and 2 of whom had severe diarrhea. Eight of these patients were treated with total gastrectomy, two with successful tumor resection, and one with parietal cell vagotomy (which facilitated the control of hyperacidity with cimetidine). All operations except one were performed electively and there was no operative mortality.