Zollinger-Ellison Syndrome.
Zollinger-Ellison Syndrome.

Video Endoscopic Sequence 1 of 38.

Gastric Ulcer with Gastrocolic fistula due to a Zollinger-Ellison Syndrome.

A 64 year-old man that that presented a history of weight loss of 26 pounds with several upper gastrointestinal hemorrhages. The patient had two previous upper endoscopies elsewhere that apparently report as chronic ulcer of duodenal bulb and another one in a national public hospital.
This image was thought to be a Gastric Carcinoma, the biopsies taken by endoscopy did not show malign tissue. The patient had surgery due to obstructive symptoms and in suspicion of malignancy but the surgical finding and pathological finding did not reveal cancer.
At surgery, the ulcer was found to be penetrating into Transverse colon with fistula. A Billroth II gastrectomy was performed with partial resection of transverse colon. At that time we did not know that the patient had ZE syndrome.

later studies revealed:
GASTRINA 3.460.00 PICO G/ML. normal= 0.01-100.
The cat scan displayed thickening of the head of the
pancreas as it gives the suspicion us of being the
gastrinoma that was confirm with nuclear study with
radionuclide octreotide scanning (also known as
somatostatin receptor scintigraphy or 111In pentetreotide
SPECT).

One month after the sub-total gastrectomy, patient suffer multiple times of peptic stenosis of the cardias, refractory to esophageal dilation, later in spite of that underwent a distal esophagectomy, refractory esophageal stenosis reappeared, also refractory to multiple esophageal dilations, surgeons decide to perform a total gastrectomy keeping the tumor in the head of the pancreas.

6 years later the patient has been stable but the gastrinoma in the head of the pancreas has grown enough with non-symptoms and no metastases, the gastrina has been elevated to 32.250.

As a peculiar information is that the patient has his second wife pregnant in April 2008.

For more endoscopic details download the video clips by clicking on the endoscopic images, wait to be downloaded complete then press Alt and Enter; thus you can observe the video in full screen.

All endoscopic images shown in this Atlas contain
video clips.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 2 of 38.

Gastro-Colonic fistula due to Zollinger Ellison Syndrome.

Colonoscopy performed previously.

Retrospectively we knew that this enlarged image of the transverse colon was part of the gastrocolonica fistula.

Due to the medical history of the patient. We performed a colonoscopy found in the image and the video displayed above. Immediately after the colonoscopy, an upper gastroscopy was performed.

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 3 of 38.

The images and videos of this sequence display a big ulcer with a blood clot and obstructing of the antrum. This image at the beginning was diagnosed as gastric carcinoma.
The Zollinger-Ellison syndrome (ZES), characterized by severe peptic ulcer disease, gastric acid hypersecretion, and non-beta islet cell tumors of the pancreas, was first described in 1955. It is now known that the potent gastric acid secretagogue proposed originally by Zollinger and Ellison is the heptadecapeptide gastrin. Although most gastrinomas occur sporadically, about 20%.

The syndrome was first reported in 1955 by Zollinger and Ellison. The true incidence is unknown, but it has been estimated that it accounts for 0.1 to 1% of peptic ulcers. It may occur at any age, but the initial manifestations are most common between ages 20 and 50.

The tumors are cancerous in 50 percent of the cases. They secrete a hormone called gastrin that causes the stomach to produce too much acid, which in turn causes stomach and duodenal ulcers (peptic ulcers). The ulcers caused by ZES are less responsive to treatment than ordinary peptic ulcers. What causes people with ZES to develop tumors is unknown, but approximately 25 percent of ZES cases are associated with a genetic disorder called multiple endocrine adenomatosis.(M.E.A.) Type I. This syndrome includes hyperplasia and/or tumors of the parathyroids, pancreatic islets and pituitary. In addition, thyroid nodules, carcinoid tumors and hyperplasia of the adrenal cortex have often been described in family members. The diagnosis of Zollinger-Ellison syndrome is made on the demonstration of high serum gastrin levels. Fasting gastrin levels in normals and in patients with ordinary duodenal ulcer average approximately 60 pg/ml. Patients with gastrinoma almost always have levels greater than 150 pg/ml and not uncommonly greater than 1,000 pg/ml. The diagnosis is also suspected on the clinical history, marked acid hypersecretion, prominence of mucosal folds in the stomach, duodenum and sometimes jejunum or SR. It may be confirmed by several provocative tests involving measurement of serum gastrin levels in response to calcium infusion, secretin injection or a standard test meal.

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 4 of 38.

Gastro-Colonic Fistula.


The images and the videos belong to the same patient of the case described above with enormous ulcer that was penetrating to the pancreas and fistula to the transverse colon.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 5 of 38.

A close up to the ulcer.

Gastric Ulcer with Gastrocolic fistula due to Zollinger-Ellison syndrome. 


Zollinger Ellison Syndrome

Video Endoscopic Sequence 6 of 38.

Due to unusual colonoscopic image. We published several images and videos of this fistula.

Some videos clips display, bloody secretion.

 

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 7 of 38.

The antrum is obstructed, the histopathologic report did not find any malignancy.

But an experienced endoscopist can think that these images can belong to a gastric neoplasia.

 

More details download the video clip.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 8 of 38.

Images and videos on a case of complicated ulcer with fistula to the transverse colon.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 9 of 38.

Images and video clips of Zollinger-Ellison Syndrome.

Gastroesophageal Reflux Disease with the Zollinger-Ellison Syndrome. Peptic stenosis of the cardias in a patient that underwent a subtotal gastrectomy due to gastrocolic fistula and Zollinger-Ellison syndrome with gastric outlet obstruction. The endoscopic image of the endoscopy that we performed was of a gastric carcinoma but no malignancy was found with the pathologist.

Approximately one month after the surgery, the patient started to develop a dysphagia to solids and liquids. And subsequently developed esophageal stricture We suspected the Zollinger Ellizon syndrome due to the aberrant clinical course. Multiple repeat esophageal dilation were performed but the stenosis was refractory in spite of to have used proton pump inhibitors in high doses.

Pubmed: The gastrinoma triangle: operative implications.





Zollinger Ellison Syndrome

Video Endoscopic Sequence 10 of 38.

The image displays a narrowing in the gastroesophagic junction.

Patients with MEN1 with Gastrinomas have an Increased Risk of Severe Esophageal Disease Including Stricture, and the Premalignant Condition, Barrett's Esophagus.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 11 of 38.

Some bands of tissue are observed, multiple biopsies
were obtained. 


Zollinger Ellison Syndrome

Video Endoscopic Sequence 12 of 38.

Esophageal Dilatation.

A hydrostatic dilating balloon straddled across the stricture.
Under direct observation, the stricture is dilated.

Esophageal dilatation is a procedure for opening a blocked orconstricted section of the esophagus. (The esophagus is a longtube, known colloquially as the "gullet," through which foodpasses on its way to the stomach.) The procedure is normallyperformed by passing bougies, or dilators, through the constrictedsection of the esophagus, in order to break the constricting tissuesor stretch the narrowed section.

 

 

Video Endoscopic Sequence 13 of 38.

The stenosis was overcome, the anastomosis of subtotal
gastrectomy is appreciated, see the video clip. 

 

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 14 of 38.

A long segment of the jejunum is observed in the video clip.

 



Zollinger Ellison Syndrome

Video Endoscopic Sequence 15 of 33.

The patient needed several endoscopic dilations that was
performed in several days.
Therefore the surgeon decided to perform a surgery,
cutting the distal segment of the esophagus
(distal esophagectomy resection) but in spite of
the surgery the evolution was not satisfactory and multiple
dilations of the esophagus were carried out.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 16 of 38.

Balloon dilation of esophageal stricture under direct
endoscopic visualization.

The Balloon dilatation is one from the therapeutic procedures ofstenosis of the esophagus and the balloon dilatation of thetherapeutic procedures of the choice in stenosis of the esophagus.

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 17 of 38.

The image and the video displays the dilator has overcome
the Acid Peptic Stricture due to Zollinger Ellison syndrome.

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 18 of 38.

Status post esophageal dilatation.

This image displays a long segment with necrosis and
rest of silk of the previous surgery of the distal segment
of the esophagus.



Zollinger Ellison Syndrome

Video Endoscopic Sequence 19 of 38.

Extensive ulceration of the distal esophagus.

Another image and video of this status post esophageal
dilation. Necrosis and rest of silk threads is observed.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 20 of 38.

A self-expanding 10 cm. Z-Stent.

Due to the aggressiveness of this disease and the
repetition of the stenosis, we decided to place an
esophageal stent.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 21 of 38.

Three days after the placement of the stent an endoscopy
was carried out.

Finally, Patient undergone a total gastrectomy keeping the
tumor of the head of the pancreas where was the exact site
of the gastrinoma, patient gain weight after six year remain
stable.



Zollinger Ellison Syndrome

Video Endoscopic Sequence 22 of 38.

Computerized Tomography Scan.

Six years after the total gastrectomy the patient has been
stable with no symptoms, the cat scan displays
a large mass in the head of the pancreas with no
metastases the gastrina level has been elevated to 32.250. 

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 23 of 38.

Gastrinomas are an integral part of the Zollinger-Ellison
syndrome (ZES). In fact, ZES is also known as gastrinoma.
This syndrome consists of ulcer disease in the upper
gastrointestinal tract, marked increases in the secretion of
gastric acid in the stomach, and tumors of the islet cells in
the pancreas. The tumors produce large amounts of
gastrin that are responsible for the characteristics of
Zollinger-Ellison syndrome, namely severe ulcer disease.
Although usually located within the pancreas, they may
occur in other organs.



Zollinger Ellison Syndrome

Video Endoscopic Sequence 24 of 38.

Surgically, gastrinomas are often difficult to locate, even
with careful inspection. They may be missed in at least
10–20% of patients with ZES. Gastrinomas are sometimes
found only because they have metastasized and produced
symptoms related to the spread of malignancy. Such
metastasis may be the most reliable indication of whether
the gastrinoma is malignant or benign,

Approximately half of all gastrinomas do not show up on
imaging studies. Therefore, exploratory surgery is often
recommended to try to locate and remove the tumors.


Zollinger Ellison Syndrome

Video Endoscopic Sequence 25 of 38.

Diagnostic imaging techniques help locate the gastrinomas.
The most sophisticated is radionuclide octreotide scanning
(also known as somatostatin receptor scintigraphy or 111In
pentetreotide SPECT.





Zollinger Ellison Syndrome

Video Endoscopic Sequence 26 of 38.

The radiological detection of endocrine tumors of the
pancreas poses a difficult challenge to the radiologist
because of the small size of most of these tumors and
frequently requires the combined use of different imaging
modalities. However, important progress in the detection of
these tumors by noninvasive means has been achieved
bythe introduction of CT and fast MRI and with the
combined use of ultrasound, CT and MRI a sensitivity of
more than 90% can nowadays be achieved.

Zollinger Ellison Syndrome

Video Endoscopic Sequence 27 of 38.

Over 90% of gastrinomas are found within an anatomic triangle referred to as the gastrinoma triangle. The 3 points that define this region are: (1) the confluence of the cystic and common bile duct, (2) the junction of the second and third portions of the duodenum, and (3) the junction of the neck and body of the pancreas.

Recent evidence suggests that gastrinomas occur at least as frequently in the duodenal wall as in the head of the pancreas within this triangle. Gastrinomas vary in size, ranging from 0.1 cm to more than 20 cm in diameter. In at least 50% of cases, these tumors are multiple. Less commonly, gastrinomas may be found in the hilum of the spleen, in the stomach, liver, or parapancreatic and mesenteric lymph nodes. A small number of ovarian tumors have been shown to be gastrinomas. Although up to two thirds of gastrinomas are malignant, it is difficult to determine on histologic appearance alone. Even when malignant, these tumors are slow growing. However, a small number of patients with gastrinomas have tumors that grow and metastasize rapidly.
Patients with gastrinomas limited to lymph nodes tend to survive for long periods, often over 20 years, without tumor progression. The presence of liver metastases reduces life expectancy to about 8 years.






Zollinger Ellison Syndrome

Video Endoscopic Sequence 28 of 38.

Clinical Features of ZES

The mean age at presentation is 45-50 years, and men are
affected more often than women. Because of the rarity of
the disease, the average interval between onset of
symptoms and diagnosis is about 6 years. Most
gastrinomas are malignant; therefore, a high index of
suspicion remains key to proper and prompt management
of the disease. Management is aimed at cure. ZES may
present in one of several ways:


 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 29 of 38.

Clinical Features of ZES

Peptic ulcer disease: This disease is present in 90%-95%
of patients with gastrinomas. Patients who are Helicobacter
pylori infection-negative and have no history of
nonsteroidal anti -inflammatory drug use may have ZES.
Peptic ulcers associated with ZES tend to be more
persistent and less responsive to therapy than those not
associated with ZES. Ulcers occurring in the second, third,
or fourth portions of the duodenum or the jejunum should
alert one to the possibility of ZES, although a single ulcer in
the duodenal bulb is the most common presentation.
Gastroesophageal reflux disease complicated by
ulcerations and strictures of the esophagus also tends to be
more prevalent and more severe in patients with ZES.





Zollinger Ellison Syndrome

Video Endoscopic Sequence 30 of 38.

Clinical Features of ZES

Diarrhea: This symptom may precede ulcer symptoms and is seen in over 30% of patients with gastrinoma Diarrhea results not only from gastric acid hypersecretion and subsequent activation of pepsinogens by the acid (which causes mucosal injury of the small intestine), but also from acid inactivation of pancreatic enzymes and the acid damage to enterocytes.

Steatorrhea: This defect occurs in part because inactivation ofpancreatic lipase by intraluminal acid in the upper small intestineand the low pH environment render some primary bile acidsinsoluble, and thereby reduce the formation of micelles(which are necessary for fatty acid and monoglyceride absorption). In addition, patients often have blunted villi and, in rare cases,totally flat mucosa with resultant malabsorption.




Zollinger Ellison Syndrome

Video Endoscopic Sequence 31 of 38.

Clinical Features of ZES

Less frequently, ZES may present as:

Vitamin B12 malabsorption: This deficiency may developbecause low intraluminal intestinal pH interferes with intrinsicfactor-facilitated active absorption of vitamin B12 by the distalileum. 

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 32 of 38.

More images of the Computerized Tomography Scan.


 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 33 of 38.

More images of the Computerized Tomography Scan.

 

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 34 of 38.

8 years after:

The tumor has metastasized to the liver

 

 

 

 



Zollinger Ellison Syndrome

Video Endoscopic Sequence 35 of 38.

To download the video clips, press on the images of this Magnetic resonance imaging (MRI)


 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 36 of 38.

To download the video clips, press on the images of this Magnetic resonance imaging (MRI)
.

 

Zollinger Ellison Syndrome

Video Endoscopic Sequence 37 of 38.

To download the video clips, press on the images of this Magnetic resonance imaging (MRI).


Zollinger Ellison Syndrome

Video Endoscopic Sequence 38 of 38.

To download the video clips, press on the images of this Magnetic resonance imaging (MRI).

 


2000 - 2016 gastrointestinalatlas.com
San Salvador, El Salvador | Contact