Zollinger-Ellison Syndrome

journal homepage: /locate/vjgienAvailable online at SUPPLEMENTATION TO THE ENCYCLOPEDIAZollinger –Ellison Syndrome $$Shou-jiang Tang a ,n ,Ruonan Wu a ,Feriyl Bhaijee baDivision of Digestive Diseases,Department of Medicine,2500North State Street,University of Mississippi Medical Center ,Jackson,MS 39216,USA bDepartment of Pathology,2500North State Street,University of Mississippi Medical Center ,Jackson,MS 39216,USAReceived 17April 2013;received in revised form 12June 2013;accepted 17June 2013KEYWORDSGastrinoma;Zollinger –Ellison syndrome;Endoscopy;Gastrin;Ulceration;VideoAbstractBackground:Zollinger –Ellison (ZE)syndrome is characterized by gastric acid hypersecretionand ulcer disease from autologous gastrin secretion by a gastrinoma.Patient and methods:A 43year old man underwent upper endoscopy for a 6week history of recurrent nausea,vomiting,heartburn,weight loss,and watery diarrhea.Results:Endoscopic findings included severe re flux esophagitis with multiple linear esopha-geal ulcerations,thickened gastric folds with mosaic pattern mucosa,distinctive gastric corpus and antrum junction,numerous antral erosions with traces of coffee ground substance,bulbar erosions and ulcerations,and post-bulbar erosions and ulcerations.Based on these symptoms and endoscopic findings,a gastrinoma work-up was instituted and the diagnosis was con firmed.Conclusions:Endoscopists need to be aware of the classical symptoms and clinical findings associated with ZE syndrome in order to appropriately diagnose and manage affected patients.&2013The Authors.Published by Elsevier GmbH.Video Related to this ArticleVideo related to this article can be found online at http:///10.1016/j.vjgien.2013.06.005.1.BackgroundA 43year old man was referred for a 6week history of recurrent nausea,vomiting,heartburn,weight loss of 80lbs,and watery diarrhea.The patient had no signi ficant past medical or surgical history.The patient reported no signi ficant family history.2.MaterialsDiagnostic gastroscope (Olympus GIF-Q180,OlympusAmerica,Center Valley,PA).2212-0971&2013The Authors.Published by Elsevier GmbH./10.1016/j.vjgien.2013.06.005☆☆The terms of this license also apply to the correspondingvideo.nCorresponding author .Tel.:+16019844540;fax:+16019844548.E-mail addresses:stang@ (S.-j.Tang),rwu@ (R.Wu),Fbhaijee@ (F .Bhaijee).Video Journal and Encyclopedia of GI Endoscopy (2014)1,666–668Open access under CC BY license .Open access under CC BY license .3.Endoscopic procedureEndoscopicfindings:○Severe reflux or erosive esophagitis with multiplelinear esophageal ulcerations.○Thickened gastric folds with mosaic pattern mucosa.○Distinctive gastric corpus and antrum junction.○Numerous antral erosions with traces of coffeeground substance.○Bulbar erosions and ulcerations.○Post-bulbar erosions and ulcerations.Endoscopic intervention:○Endoscopic biopsy was performed at the stomachand duodenum.○Pathology:▪Chronic gastritis with parietal cell hyperplasia.▪No evidence of Helicobacter pylori infection.▪Acute peptic duodenitis.Fasting serum gastrin level:2305pg/mL(normal o100).Whole body octreotide scintigraphy:○Focal increased uptake in the mid-abdomen,corre-sponding to the head and uncinate process of thepancreas.○Physiologic activity in the liver,spleen,bowel,kidneys,and bladder.The patient underwent surgical enucleation of the pancreatic gastrinoma and truncal vagotomy.Surgical pathology:1.5cm gastrinoma in the pancrea-tic head.○Low grade,well-differentiated gastrinoma withoutatypia or mitoticfigures.○Positive immunostains,confirming neuroendocrineorigin:▪Chromogranin.▪Synaptophysin.▪Gastrin.▪CD56.Outcome:at last follow-up,the patient's gastrointest-inal(GI)symptoms had resolved.4.Key learning points and tips and tricksIn1995,Zollinger and Ellisonfirst described the Zollinger–Ellison(ZE)syndrome as gastric acid hyper-secretion and severe ulcer disease from autologous gastrin secretion by a gastrinoma[1–4].More than90%of the gastrinomas are located in the “gastrinoma triangle,”which is formed by the con-fluence of:○the cystic and common bile ducts superiorly,○the second and third portions of the duodenuminferiorly,and○the pancreatic neck and body medially.Mostly appear in the fourth to sixth decades.May be sporadic or associated with multiple endocrine neoplasia type1.About60%of gastrinomas are malignant.Presentations:▪Hypergastrinemia and gastric acid hyersecretion.Esophageal acid reflux.Thickened gastric folds with hypertrophic parietal cell mass.Gastric and duodenal erosions and ulcerations.Post-bulbar erosions and ulcerations.▪MetastasesEndoscopicfindings:mucosal changes and injury related to gastric acid hypersecretion.Diagnostic and localization studies:○Elevated fasting serum gastrin level,usually41000pg/mL.○Secretin stimulation test.▪Serum gastrin increases by4200pg/mL aftergiven an injection of secretin.○Low gastric pH measured during endoscopy orsecretin measurement.○Serum markers for neuroendocrine neoplasms suchas chromogranin A and neuron-specific enolase.○Octreotide scintigraphy.○Endoscopic ultrasound.○Magnetic resonance imaging or computerized tomo-graphy scan.○Selective arteriography.Treatment options:○The optimal treatment is surgical excision.○High dose proton pump inhibitor(usually twice aday dosing).○Somastostatin.○Chemotherapeutic agents.5.Scripted voiceoverVoiceover TextA67year old male patient who received oral anticoagulant therapy due to implanted mechanical aortic and mitral valves was admitted to our department with melena six days after a successful sphincterotomy and stone extraction for choledocholithiasis.During emergency duodenoscopy post-papillotomy bleeding was controlled by local injection of diluted epinephrine.24hours later recurrent and refractory post-papillotomy bleeding occurred.At the time of repeated emergency endoscopy a large clot with fresh blood concealing the papilla is visible in the descending duodenum.Dormia basket and highflow water jetflushing is used to remove the adherent clot and clean the site of bleeding.We introduce a5Fr4cm long prophylactic pancreatic stent first to prevent post-procedure pancreatitis.After repeated washing the bleeding point is coagulated by using a hemostatic forceps.Coagulation process is made step-by-step throughout the whole margin of the post-sphincterotomy wound by using a hemostatic forceps(Coagrasper)similarly to ESD with a soft coagulation mode,effect6,plete667ZE SyndromeVoiceover Texthemostasis is achieved by electrocautery forceps coagulation as a new endoscopic modality.No re-bleeding observed next day.No complication occured including post-procedure pancreatitis.The prophylactic stent was removed few days later.Normal post-sphincterotomy conditions observed11days later.A77year old woman with previous cholecystectomy had an enlarged papilla suggesting a papillary adenoma. Papillotomy caused mild bleeding,but it was controlled by injection of diluted epinephrine.Cholangiography showed dilated common bile duct with an obstruction at the level of papilla.8hours after the initial procedure emergency duodenoscopy was performed due to haematemesis suggesting recurrent post-papillotomy rge amount of fresh clot and fresh blood was observed in the descending duodenum at the level of the papilla.Initially,it is always difficult to make a clear view due to massive bleeding.Dormia basket is usedfirst for removing the large clot from the papilla pushing it distally into the duodenum.Now we are using Fujinon highflow water jet for thorough washing.As it seems that the bleeding originates from the whole circumference of the sphinterotomized papilla and the first line haemostatic therapy of injecting diluted epinephrine failed,we decided to use the Coagrasper to coagulate the papilla.Coagulation process should be made step-by-step throughout the whole margin of the post-sphincterotomy wound,but always start at the actively bleeding point.It is also crucial that the site of the forceps coagulation should always be kept within the edge of the papilla to avoid perforation.At the time of the coagulation we pull the tissue a little towards the lumen to prevent transmural burn of the duodenal wall then we gently release the coagulated parts from the Coagrasper.At the end of the procedure primary hemostasis is clearly achieved.Voiceover TextThe following day endoscopy showed no signs of bleeding. The pancreatic stent is in place.The additionally placed haemoclips are visible.A week later a gastroscopy was performed to remove the pancreatic stent.Histology revealed papillary adenocarcinoma and multiple small liver metastases were detected on abdominal CT.Finally a biliary SEMS was applied for long term endoscopic palliation. Electrocautery forceps coagulation is an effective second line treatment in refractory post-papillotomy bleeding. Highflow water jetflushing is useful in optimal visualization of the bleeding point.Prophylactic pancreatic stents prevent post-procedure pancreatitis in these cases bination of prophylactic pancreatic stenting with thermal coagulation with coagulation forceps might be suggested as a rescue method in severe post-papillotomy bleeding.Conflict of interestAll authors,Shou-jiang Tang,Ruonan Wu,Feriyl Bhaijee, have nothing to declare and we have no conflict of interests.References[1]Zollinger RM,Ellison EH.Primary peptic ulcerations of thejejunum associated with islet cell tumors of the pancreas.Ann Surg1955;142(4):709–23.[2]Metz DC,Jensen RT.Gastrointestinal neuroendocrine tumors:pancreatic endocrine tumors.Gastroenterology2008;135(5): 1469–92.[3]Morrow EH,Norton JA.Surgical management of Zollinger–Ellisonsyndrome;state of the art.Surg Clin North Am2009;89(5): 1091–103.[4]Ellison EC.Zollinger–Ellison syndrome:a personal perspective.Am Surg2008;74(7):563–71.[review].S.-j.Tang et al.668。