journal homepage: www.elsevier.com/locate/vjgien Available online at www.sciencedirect.com SUPPLEMENTATION TO THE ENCYCLOPEDIA Zollinger–Ellison Syndrome $$ Shou-jiang Tang a,n , Ruonan Wu a , Feriyl Bhaijee b a Division of Digestive Diseases, Department of Medicine, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA b Department of Pathology, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA Received 17 April 2013; received in revised form 12 June 2013; accepted 17 June 2013 KEYWORDS Gastrinoma; Zollinger–Ellison syndrome; Endoscopy; Gastrin; Ulceration; Video Abstract Background: Zollinger–Ellison (ZE) syndrome is characterized by gastric acid hypersecretion and ulcer disease from autologous gastrin secretion by a gastrinoma. Patient and methods: A 43 year old man underwent upper endoscopy for a 6 week history of recurrent nausea, vomiting, heartburn, weight loss, and watery diarrhea. Results: Endoscopic findings included severe reflux 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 confirmed. 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. & 2013 The Authors. Published by Elsevier GmbH. Video Related to this Article Video related to this article can be found online at http:// dx.doi.org/10.1016/j.vjgien.2013.06.005. 1. Background A 43 year old man was referred for a 6 week history of recurrent nausea, vomiting, heartburn, weight loss of 80 lbs, and watery diarrhea. The patient had no significant past medical or surgical history. The patient reported no significant family history. 2. Materials Diagnostic gastroscope (Olympus GIF-Q180, Olympus America, Center Valley, PA). 2212-0971 & 2013 The Authors. Published by Elsevier GmbH. http://dx.doi.org/10.1016/j.vjgien.2013.06.005 ☆☆ The terms of this license also apply to the corresponding video. n Corresponding author. Tel.: + 1 601 984 4540; fax: + 1 601 984 4548. E-mail addresses: [email protected] (S.-j. Tang), [email protected] (R. Wu), [email protected] (F. Bhaijee). Video Journal and Encyclopedia of GI Endoscopy (2014) 1, 666–668 Open access under CC BY license . Open access under CC BY license .
Zollinger–Ellison Syndrome
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Zollinger–Ellison SyndromeVideo Journal and Encyclopedia of GI Endoscopy (2014) 1, 666–668
Available online at www.sciencedirect.com
2212-0971 & 2013 T http://dx.doi.org/1
fax: +1 601 984 454 E-mail addresses
[email protected] (R. W
journal homepage: www.elsevier.com/locate/vjgien
aDivision of Digestive Diseases, Department of Medicine, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA bDepartment of Pathology, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA
Received 17 April 2013; received in revised form 12 June 2013; accepted 17 June 2013
KEYWORDS Gastrinoma; Zollinger–Ellison syndrome; Endoscopy; Gastrin; Ulceration; Video
he Authors. Publis 0.1016/j.vjgien.20
is license also app thor. Tel.: +1 601 8. : [email protected] u), Fbhaijee@umc
Abstract Background: Zollinger–Ellison (ZE) syndrome is characterized by gastric acid hypersecretion and ulcer disease from autologous gastrin secretion by a gastrinoma. Patient and methods: A 43 year old man underwent upper endoscopy for a 6 week history of recurrent nausea, vomiting, heartburn, weight loss, and watery diarrhea. Results: Endoscopic findings included severe reflux 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 confirmed. 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.
& 2013 The Authors. Published by Elsevier GmbH. Open access under CC BY license.
Video Related to this Article
Video related to this article can be found online at http:// dx.doi.org/10.1016/j.vjgien.2013.06.005.
hed by Elsevier GmbH. 13.06.005
ly to the corresponding video. 984 4540;
(S.-j. Tang), .edu (F. Bhaijee).
Open access
1. Background
und
2. Materials
er CC BY license.
linear esophageal 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. Post-bulbar erosions and ulcerations.
Endoscopic intervention: Endoscopic biopsy was performed at the stomach
and duodenum. Pathology:
o100).
Whole body octreotide scintigraphy:
Focal increased uptake in the mid-abdomen, corre- sponding to the head and uncinate process of the pancreas.
Surgical pathology: 1.5 cm gastrinoma in the pancrea- tic head. Low grade, well-differentiated gastrinoma without
atypia or mitotic figures. Positive immunostains, confirming neuroendocrine
origin:
Outcome: at last follow-up, the patient's gastrointest- inal (GI) symptoms had resolved.
4. Key learning points and tips and tricks
More than 90% 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 duodenum
inferiorly, and the pancreatic neck and body medially.
41000 pg/mL. Secretin stimulation test.
Serum gastrin increases by 4200 pg/mL after
given an injection of secretin.
Low gastric pH measured during endoscopy or
secretin measurement. Serum markers for neuroendocrine neoplasms such
as 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 a
day dosing). Somastostatin. Chemotherapeutic agents.
5. Scripted voiceover
Voiceover Text
A 67 year 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. 24 hours 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 high flow water jet flushing is used to remove the adherent clot and clean the site of bleeding.
We introduce a 5 Fr 4 cm 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, effect 6, 80 Watts. Complete
S.-j. Tang et al.668
Voiceover Text
hemostasis 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 observed 11 days later.
A 77 year 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.
8 hours after the initial procedure emergency duodenoscopy was performed due to haematemesis suggesting recurrent post-papillotomy bleeding. Large 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 used first for removing the large clot from the papilla pushing it distally into the duodenum.
Now we are using Fujinon high flow 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 Text
The 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. High flow water jet flushing is useful in optimal visualization of the bleeding point. Prophylactic pancreatic stents prevent post-procedure pancreatitis in these cases too. Combination of prophylactic pancreatic stenting with thermal coagulation with coagulation forceps might be suggested as a rescue method in severe post-papillotomy bleeding.
Conflict of interest
All 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 the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955;142(4):709–23.
[2] Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology 2008;135(5): 1469–92.
[3] Morrow EH, Norton JA. Surgical management of Zollinger–Ellison syndrome; state of the art. Surg Clin North Am 2009;89(5): 1091–103.
[4] Ellison EC. Zollinger–Ellison syndrome: a personal perspective. Am Surg 2008;74(7):563–71. [review].
Background
Materials
Scripted voiceover
Available online at www.sciencedirect.com
2212-0971 & 2013 T http://dx.doi.org/1
fax: +1 601 984 454 E-mail addresses
[email protected] (R. W
journal homepage: www.elsevier.com/locate/vjgien
aDivision of Digestive Diseases, Department of Medicine, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA bDepartment of Pathology, 2500 North State Street, University of Mississippi Medical Center, Jackson, MS 39216, USA
Received 17 April 2013; received in revised form 12 June 2013; accepted 17 June 2013
KEYWORDS Gastrinoma; Zollinger–Ellison syndrome; Endoscopy; Gastrin; Ulceration; Video
he Authors. Publis 0.1016/j.vjgien.20
is license also app thor. Tel.: +1 601 8. : [email protected] u), Fbhaijee@umc
Abstract Background: Zollinger–Ellison (ZE) syndrome is characterized by gastric acid hypersecretion and ulcer disease from autologous gastrin secretion by a gastrinoma. Patient and methods: A 43 year old man underwent upper endoscopy for a 6 week history of recurrent nausea, vomiting, heartburn, weight loss, and watery diarrhea. Results: Endoscopic findings included severe reflux 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 confirmed. 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.
& 2013 The Authors. Published by Elsevier GmbH. Open access under CC BY license.
Video Related to this Article
Video related to this article can be found online at http:// dx.doi.org/10.1016/j.vjgien.2013.06.005.
hed by Elsevier GmbH. 13.06.005
ly to the corresponding video. 984 4540;
(S.-j. Tang), .edu (F. Bhaijee).
Open access
1. Background
und
2. Materials
er CC BY license.
linear esophageal 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. Post-bulbar erosions and ulcerations.
Endoscopic intervention: Endoscopic biopsy was performed at the stomach
and duodenum. Pathology:
o100).
Whole body octreotide scintigraphy:
Focal increased uptake in the mid-abdomen, corre- sponding to the head and uncinate process of the pancreas.
Surgical pathology: 1.5 cm gastrinoma in the pancrea- tic head. Low grade, well-differentiated gastrinoma without
atypia or mitotic figures. Positive immunostains, confirming neuroendocrine
origin:
Outcome: at last follow-up, the patient's gastrointest- inal (GI) symptoms had resolved.
4. Key learning points and tips and tricks
More than 90% 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 duodenum
inferiorly, and the pancreatic neck and body medially.
41000 pg/mL. Secretin stimulation test.
Serum gastrin increases by 4200 pg/mL after
given an injection of secretin.
Low gastric pH measured during endoscopy or
secretin measurement. Serum markers for neuroendocrine neoplasms such
as 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 a
day dosing). Somastostatin. Chemotherapeutic agents.
5. Scripted voiceover
Voiceover Text
A 67 year 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. 24 hours 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 high flow water jet flushing is used to remove the adherent clot and clean the site of bleeding.
We introduce a 5 Fr 4 cm 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, effect 6, 80 Watts. Complete
S.-j. Tang et al.668
Voiceover Text
hemostasis 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 observed 11 days later.
A 77 year 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.
8 hours after the initial procedure emergency duodenoscopy was performed due to haematemesis suggesting recurrent post-papillotomy bleeding. Large 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 used first for removing the large clot from the papilla pushing it distally into the duodenum.
Now we are using Fujinon high flow 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 Text
The 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. High flow water jet flushing is useful in optimal visualization of the bleeding point. Prophylactic pancreatic stents prevent post-procedure pancreatitis in these cases too. Combination of prophylactic pancreatic stenting with thermal coagulation with coagulation forceps might be suggested as a rescue method in severe post-papillotomy bleeding.
Conflict of interest
All 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 the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955;142(4):709–23.
[2] Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology 2008;135(5): 1469–92.
[3] Morrow EH, Norton JA. Surgical management of Zollinger–Ellison syndrome; state of the art. Surg Clin North Am 2009;89(5): 1091–103.
[4] Ellison EC. Zollinger–Ellison syndrome: a personal perspective. Am Surg 2008;74(7):563–71. [review].
Background
Materials
Scripted voiceover
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