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Video Endoscopic Sequence 1 of 21.
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 clip
by clicking on the endoscopic image. Wait to be
downloaded complete then press Alt and Enter for full
screen for windows media and Ctrl and 3 for Real player.
All endoscopic images shown in this Atlas contain
video clips. We recommend seeing the video clips in full
screen mode.
Medline.
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Video Endoscopic Sequence 2 of 21.
Gastro-Colonic fistula due to Zollinger Ellison Syndrome.
Colonoscopy performed previously.
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.
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Video Endoscopic Sequence 3 of 21.
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.
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Video Endoscopic Sequence 4 of 21.
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.
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Video Endoscopic Sequence 5 of 21.
A close up of the ulcer.
Gastric Ulcer with Gastrocolic fistula due to Zollinger-
Ellison syndrome.
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Video Endoscopic Sequence 6 of 21.
Due to unusual colonoscopic image. We publish several
images and videos of this fistula.
Some videos display bloody secretion.
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Video Endoscopic Sequence 7 of 21.
The antrum is obstructed, the histopathologic report
did not find any malignancy.
More details download the video clip.
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Video Endoscopic Sequence 8 of 21.
Images and videos on a case of complicated ulcer with
fistula to the transverse colon.
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Video Endoscopic Sequence 9 of 21.
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.
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Video Endoscopic Sequence 10 of 21.
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.
Medline.
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Video Endoscopic Sequence 11 of 21.
Some bands of tissue are observed, multiple biopsies
were obtained.
Medline.
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Video Endoscopic Sequence 12 of 21.
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 or
constricted section of the esophagus. (The esophagus is a long
tube, known colloquially as the "gullet," through which food
passes on its way to the stomach.) The procedure is normally
performed by passing bougies, or dilators, through the constricted
section of the esophagus, in order to break the constricting tissues
or stretch the narrowed section.
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Video Endoscopic Sequence 13 of 21.
The stenosis was overcome, the anastomosis of subtotal
gastrectomy is appreciated, see the video clip.
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Video Endoscopic Sequence 14 of 21.
A long segment of the jejunum is observed in the video clip.
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Video Endoscopic Sequence 15 of 21.
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 but in spite of
the surgery the evolution was not satisfactory and multiple
dilations of the esophagus were carried out.
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Video Endoscopic Sequence 16 of 21.
Balloon dilation of esophageal stricture under direct
endoscopic visualization.
The Balloon dilatation is one from the therapeutic procedures of
stenosis of the esophagus and the balloon dilatation of the
therapeutic procedures of the choice in stenosis of the esophagus.
Esophageal Dilation, Medline.
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Video Endoscopic Sequence 17 of 21.
The image and the video displays the dilator has overcome
the Acid Peptic Stricture due to Zollinger Ellison syndrome.
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Video Endoscopic Sequence 18 of 21.
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.
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Video Endoscopic Sequence 19 of 21.
Extensive ulceration of the distal esophagus.
Another image and video of this status post esophageal
dilation. Necrosis and rest of silk threads is observed.
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Video Endoscopic Sequence 20 of 21.
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.
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Video Endoscopic Sequence 21 of 21.
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.
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Computerized Tomography Scan.
Six year 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.
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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.
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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.
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Diagnostic imaging techniques help locate the gastrinomas.
The most sophisticated is radionuclide octreotide scanning
(also known as somatostatin receptor scintigraphy or 111In
pentetreotide SPECT.
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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.
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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.
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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:
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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.
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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 of
pancreatic lipase by intraluminal acid in the upper small intestine
and the low pH environment render some primary bile acids
insoluble, 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.
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Clinical Features of ZES
Less frequently, ZES may present as:
Vitamin B12 malabsorption: This deficiency may develop
because low intraluminal intestinal pH interferes with intrinsic
factor-facilitated active absorption of vitamin B12 by the distal
ileum.
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