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The Baader-Meinhof Phenomenon of Dieulafoy’s Lesion

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Abstract

Despite modern investigative innovations in the cutting edge field of gastroenterology, we are reminded of our contemporary limitations when we encounter the ever evasive Dieulafoy's lesion (DL). Ever since it has been initially described in 1884, its rare but frustrating presence creates a calamitous situation. Even more so when it presents atypically, much like it did in our patient. This review of DL delves into the history, epidemiology, characteristics, the most current and innovative diagnostic measures available, as well as treatment and prevention of recurrence of these obscure gastrointestinal (GI) bleeding sources.
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Received 04/26/2019
Review began 04/30/2019
Review ended 04/30/2019
Published 05/03/2019
© Copyright 2019
Kolli et al. This is an open access article
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The Baader-Meinhof Phenomenon of Dieulafoy’s
Lesion
Sindhura Kolli , Khoi Paul Dang-Ho , Amit Mori , Krishna Gurram
1. Internal Medicine, The Brooklyn Hospital Center, Affiliate of the Mount Sinai Hospital, Brooklyn, USA 2.
Gastroenterology, The Brooklyn Hospital Center, Affiliate of the Mount Sinai Hospital, Brooklyn, USA
Corresponding author: Sindhura Kolli, sindhura.kolli@gmail.com
Abstract
Despite modern investigative innovations in the cutting edge field of gastroenterology, we are reminded of
our contemporary limitations when we encounter the ever evasive Dieulafoy’s lesion (DL). Ever since it has
been initially described in 1884, its rare but frustrating presence creates a calamitous situation. Even more
so when it presents atypically, much like it did in our patient. This review of DL delves into the history,
epidemiology, characteristics, the most current and innovative diagnostic measures available, as well as
treatment and prevention of recurrence of these obscure gastrointestinal (GI) bleeding sources.
Categories: Internal Medicine, Medical Education, Gastroenterology
Keywords: occult gi bleeding, dieulafoy's lesion, dieulafoy
Introduction And Background
There is a cognitive bias when one has a new experience or learned something new and suddenly one begins
to encounter it more frequently, even if its been there all along. This phenomenon, known as Baader-
Meinhof, becomes most crucial in the dissemination of academic literature about rare entities such as
Dieulafoy’s lesion (DL). By parlaying information about DLs to investigators or endoscopists, it forces them
to not only intelligibly discern these esoteric submucosal defects that can readily go unnoticed, but also
actively search for it with every withdrawal of the scope to determine the etiology behind obscure
gastrointestinal (GI) bleeding. This review will serve to explain the history, epidemiology, characteristics,
the most current and innovative diagnostic measures available, as well as treatment and prevention of
recurrence of DLs.
An 87-year-old female patient with a history of gastroesophageal reflux disease (GERD), gastritis,
diverticulosis and chronic non-steroidal anti-inflammatory drugs (NSAID) use presented with melena,
diffuse abdominal pain and non-bloody, non-bilious emesis. In the setting of her acute anemia with
hemoglobin (Hb) of 5.9 g/dL and tachycardia, an esophagoduodenoscopy (EGD) demonstrated a single
medium-sized DL in the second portion of the duodenum next to the major papilla oozing blood that was
controlled with a bipolar diathermy (Figures 1-2). Daily proton pump inhibitors (PPIs), discontinuation of
NSAIDs, and appropriate outpatient follow-up were recommended.
1 1 2 2
Open Access Review
Article DOI: 10.7759/cureus.4595
How to cite this article
Kolli S, Dang-Ho K, Mori A, et al. (May 03, 2019) The Baader-Meinhof Phenomenon of Dieulafoy’s Lesion. Cureus 11(5): e4595. DOI
10.7759/cureus.4595
FIGURE 1: Bleeding periampullary Dieulafoy's lesion
FIGURE 2: Primary hemostasis of Dieulafoy's lesion through
electrocoagulation
Review
DLs are dilated submucosal veins that are exposed to the surface and endoscopically visualized as bleeding
points without surrounding erosions or ulcerations. It accounts for 1% to 5.8% of cases of acute nonvariceal
upper GI bleeding [1-2] (Poster: Kolli S, Mori A, Gurram K. A Rare Dieulafoy Lesion in the Periampullary
Duodenum. American College of Gastroenterology Conference; Oct 2018). Despite being initially described
2019 Kolli et al. Cureus 11(5): e4595. DOI 10.7759/cureus.4595 2 of 4
by Dr. M.T. Gallard in 1884, the nomenclature for this lesion is attributed to Georges Dieulafoy in 1898 for
detailing seven patients with this pathology. He theorized that they were the initial stages of a common
gastric ulcer interrupted by erosive bleeding and are now widely known as DLs [3-5].
Histologically, an alternative nomenclature for DL is a “caliber-persistent artery,” due to the submucosal
artery failing to narrow to rival the normal capillary microvasculature of the mucosa otherwise known as
ramification. Instead, it maintains a diameter 10 times the size compared to normal mucosal arteries,
leading to profuse bleeding when irritated or under pressure during normal peristalsis [4].
These lesions can affect any age group but present more commonly in the older population, ranging from the
fifth to the seventh decade of life [1,6-8]. The lesion is more prominent in males than females with a ratio of
2:1 [1,6-7]. Comorbidities are present in up to 90% of patients [7]. The most common include cardiovascular
disease, hypertension, diabetes mellitus, liver disease, and renal failure [6-7]. Patients with DL might also
present with a history of NSAIDs or anticoagulant use which can promote bleeding [6]. No causal link,
however, has been established between DLs and the use of NSAIDs, alcohol use, tobacco use, presence of
peptic ulcer disease or Helicobacter pylori (H.pylori) infection [4,6,9].
Around 70% of DLs are located in the stomach. Within the gastric region, most of the lesions (75%) can be
found in the proximal stomach, particularly 6 cms from the gastroesophageal junction [1,6]. Lesions can also
occur further along the GI tract including the duodenum, jejunum, ileum, cecum, appendix, colon, and anal
canal which can present as lower GI bleeding. DLs can also present in extra-gastrointestinal regions such as
the bronchus, leading to hemoptysis [6-7]. A high proportion of DLs were found at intestinal anastomoses,
predominantly Billroth II anastomosis after gastrectomy [1,4].
Patients with DLs are typically asymptomatic but then can present acutely with massive GI hemorrhage [1,6-
8]. Bleeding is typically reported as melena (44%), hematemesis (30%), a combination of both (18%),
hematochezia (6%), or as iron deficiency anemia (1%) [1,5-6]. Patients can present with signs of
hemodynamic instability such as tachycardia, hypotension, and orthostasis, along with acute prerenal
azotemia [6]. Hemoglobin levels typically range between 8.4 to 9.2 g/dL [6-7]. Recurrent bleeding < 72 hours
after initial presentation can occur if left untreated following the initial endoscopy, encouraging prompt
diagnosis [6]. Other GI symptoms, especially abdominal pain, are uncommon in patients with DL, and if
present, usually indicates an alternate diagnosis such as peptic ulcer disease or complications from bleeding
such as mesenteric ischemia secondary to hemorrhagic shock [6].
Diagnosis remains challenging as urgent EGD is indicated, but without an ulcer base to guide identification,
diagnosis on an initial endoscopy can be as low as 49%; 33% of those affected require a repeat endoscopy
and 18% require an exploratory laparotomy [9]. False negatives can be attributed to a small bleeding point,
intermittent bouts of bleeding, lack of anatomical identifiers, or excessive bleeding concealing the lesion
[4,9]. Proper diagnosis can involve multiple endoscopies, the use of push enteroscopy which is an extension
of an EGD, side-viewer endoscope, or an exploratory laparotomy which provide diagnostic and therapeutic
utility. Diagnostic modalities without the advantage of immediate intervention include wireless capsule
endoscopy, angiography, or technetium-99m labelled bleeding scans when searching in anatomically
inaccessible locations [1,4,6,9] (Poster: Kolli S, Oct 2018). Agreed upon criteria for accurate diagnosis of a DL
include the following: 1) active arterial spurting or micro-pulsatile streaming of blood from a minute (<3
ram) mucosal defect or through normal surrounding mucosa; 2) visualization of a protruding vessel, with or
without active bleeding, within a minute mucosal defect or through normal surrounding mucosa; or 3) fresh,
densely adherent clot with a narrow point of attachment to a minute mucosal defect or to normal-appearing
mucosa [1,4,6,10].
Treatment is a two-pronged approach since the recurrence rate of a DL can range from 9% to as high as 40%.
Primary hemostasis can be achieved with electrocoagulation, which provides a quick and inexpensive
solution with an almost 80% hemostatic success rate [11]. Equivocal methods also include sclerotherapy with
ethanol or norepinephrine, thermocoagulation, argon plasma coagulation or hemostatic clips [1,4,6,11]
(Poster: Kolli S, Oct 2018). Secondary hemostasis can be accomplished through repeat endoscopy,
angiography, or rarely, surgical wedge resection. Tattooing the site of original therapeutic intervention
during the initial endoscopy steers the secondary hemostatic measures [11]. With proper diagnosis and
treatment, the rate of mortality has decreased from 30% in the 1970s to currently 8%; however, the difficulty
lies in vigilant detection, especially in anomalous locations, as was the case in our patient [6] (Poster: Kolli
S, Oct 2018).
Conclusions
DLs incur healthcare costs from numerous tests and hospital visits and increases aggravation due to its
difficulty in diagnosis and delay in prompt treatment. Numerous available modalities, such as push
enteroscopy as an extension of an EGD and side-viewer endoscope, aid in diagnosing DLs in anatomically
difficult locations. However, the ability to recognize them lies in the endoscopist's scope of discernation. We
hope this review coupled with the Baader-Meinhof phenomenon increases the perception of this rarely
occurring pathology.
2019 Kolli et al. Cureus 11(5): e4595. DOI 10.7759/cureus.4595 3 of 4
Additional Information
Disclosures
Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the
following: Payment/services info: All authors have declared that no financial support was received from
any organization for the submitted work. Financial relationships: All authors have declared that they have
no financial relationships at present or within the previous three years with any organizations that might
have an interest in the submitted work. Other relationships: All authors have declared that there are no
other relationships or activities that could appear to have influenced the submitted work.
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... Despite advances in endoscopic techniques, the diagnosis of DLs remains difficult [1,3]. This is likely due to the varying presentations and intermittent bleeding of DLs, in combination with the infrequency of the condition and a lack of clinician familiarity [1,4]. This case explores the challenges of diagnosing a duodenal DL in the context of a concurrent gastric ulcer. ...
... Endoscopy is considered the gold standard diagnostic technique, allowing for immediate treatment [1,5,7]. While endoscopy has significantly improved survival for DLs, diagnosis remains a challenge with one study reporting only 49% of DLs were identified on initial endoscopy [2][3][4]7]. Factors which contribute to this include the intermittent nature of bleeding, small lesion size with an absence of abnormal surrounding mucosa, and a lack of clinical experience with the condition [1,4,7,8]. ...
... While endoscopy has significantly improved survival for DLs, diagnosis remains a challenge with one study reporting only 49% of DLs were identified on initial endoscopy [2][3][4]7]. Factors which contribute to this include the intermittent nature of bleeding, small lesion size with an absence of abnormal surrounding mucosa, and a lack of clinical experience with the condition [1,4,7,8]. Additionally, it may further be obscured by pooling of blood or gastric contents [4,7]. Duodenal angulations, folds and diverticulum may also further conceal DLs in the duodenum [7,8]. ...
A diagnostic dilemma: a case report of concomitant duodenal Dieulafoy lesion and gastric ulcer
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Dieulafoy lesions (DL) are an uncommon cause of gastrointestinal bleeding which is often difficult to diagnose due to the rarity of the condition and varying clinical presentations. This case describes an unusual presentation of upper gastrointestinal bleeding in an 85-year-old female with findings on two separate gastroscopies of both a gastric ulcer and duodenal DL. The pathophysiology of DL remains poorly understood and despite shared risk factors, these two pathologies are rarely reported concurrently. The presence of a concomitant gastric ulcer further complicated the diagnosis and treatment of the duodenal DL in this case. This highlights the importance of clinician awareness of this pathology and its presentation and the need for early repeat endoscopy.
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... When working up the cause of a problem, oftentimes the rarer occurring potential etiologies are discounted with statements invoking the Baader-Meinhof phenomenon. These phrases include the statement, "When you hear the beating of hoofs, don't think of zebras" or "Keep it simple, stupid", or the KISS principle [2][3][4][5][6][7][8]. The Baader-Meinhof phenomenon, also known as the frequency bias, states that rarely occurring events are rare. ...
... The Baader-Meinhof phenomenon, also known as the frequency bias, states that rarely occurring events are rare. Secondarily, the logical heuristic posits that it is the knowledge that such rare occurrences do exist, that causes the interlocutor to become more likely to search out the event or see it occur more frequently [2][3][4][5][6][7][8]. The original researchers of the Baader-Meinhof phenomenon would state that this false increase in the frequency of observation is then blamed on the simple fact that the interlocutor is now aware [2][3][4][5][6][7][8]. ...
... Secondarily, the logical heuristic posits that it is the knowledge that such rare occurrences do exist, that causes the interlocutor to become more likely to search out the event or see it occur more frequently [2][3][4][5][6][7][8]. The original researchers of the Baader-Meinhof phenomenon would state that this false increase in the frequency of observation is then blamed on the simple fact that the interlocutor is now aware [2][3][4][5][6][7][8]. Due to the need for proper and quick diagnosis of a problem in the development of novel devices, preventing damage or destruction of property, or in the treatment of patients, the Baader-Meinhof logical heuristic is often mis-utilized to minimize the chances of rarer phenomena from being considered [2][3][4][5][6][7][8]. ...
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Often when discussing what is considered a rarer occurring event, individuals reference the Baader-Meinhof phenomenon as insurance against fallacious thinking. Also known as the frequency bias, this logical heuristic states that rare occurring events are rare and the knowledge of the existence of rare occurrences makes the interlocutor more likely to search out the event or see it occur more frequently. These false increases in observation frequency can logically be blamed, at least in part, on the interlocutor being made aware of the event existence. This Baader-Meinhof logical heuristic is often mis-utilized in the sciences to minimize the chances of rarer phenomena from being considered within a logical framework for the work up of a problem. This article presents a new logical heuristic, the "Mongoose Phenomenon" as a counter argument and presents it in the context of the fields of medicine, the hard sciences, engineering, and philosophy. It is the intention of the authors that this logical heuristic be utilized to improve the thought process of scientists, clinicians, and others to ensure the best thought process for the work up and creation of a solution for problems.
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... Dieulafoy's lesions are believed to be congenital, but they have been reported to occur more frequently with advancing age [2] . Dieulafoy's lesions account for 1%-5.8% of acute gastrointestinal bleeding [3,4] , but they often present with urgent and massive bleeding usually leading to shock, and even death. They also have a high rate of recurrence. ...
... Dieulafoy's lesions have been reported throughout the digestive tract with 8% in the esophagus, 71% in the stomach, 15% in the duodenum, 1% in the intestine, 2% in the colon, 2% in the rectum and 1% in gastric anastomosis [3] . Santos reported a case of a Dieulafoy's lesion located in the gallbladder in 2020 [1] . ...
... Due to urgent, massive, arterial bleeding, it is often difficult to stop the hemorrhage using common conservative therapies. Treatment by endoscopy has become the preferred and most effective way of managing a Dieulafoy's lesion, with a reported success rate of over 90% [3] . Commonly used endoscopic hemostatic procedures include electrocoagulation, thermocoagulation, local epinephrine injection, sclerotherapy, banding, and hemoclips [7,14] . ...
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Background: Dieulafoy's lesion is a rare vascular abnormality characterized by a small abnormally dilated artery that runs a tortuous course in the submucosa. There is usually no ulcer present in Dieulafoy's lesions and the overlying mucosa is most often normal. Bleeding caused by a Dieulafoy's lesion is usually urgent, massive, life-threatening and prone to recurrence. Dieulafoy's lesions have been reported throughout the digestive tract although the majority of them have been found in the upper digestive tract especially the stomach and duodenum. However, a Dieulafoy's lesion occurring inside a duodenal diverticulum is very rare. Case summary: A 74-year-old Asian male with epigastric pain, hematemesis and melena was admitted to our clinic. Before admission, the patient had vomited 500 mL of dark red blood, and passed 200 g of black tarry stool. Conservative management was first undertaken as the patient had not been fasting. However, hemorrhage recurred and the patient went into shock. Urgent endoscopy was performed and a diverticulum of 1.8 cm × 1.2 cm × 0.8 cm was found on the anterior wall of the descending duodenum. The diverticulum was covered with a blood clot. After the clot was removed, an artery stump was observed in the diverticulum with a diameter of 2-3 mm. Two titanium hemostatic clips were inserted to clamp the vessel stump. The patient was discharged 7 d post-endoscopy and followed for 6 mo with no recurrence. Conclusion: This case was diagnosed with a Dieulafoy's lesion inside a duodenal diverticulum which has rarely been reported. Hematemesis was stopped by clamping the vessel stump with titanium clips. No complications occurred.
View
... However, this case demonstrates the need to observe the capsule's real-time display, if available, in patients under investigation for OGB. Dieulafoy's lesion, as presented here, is a submucosal vascular lesion identified endoscopically as a bleeding point not associated with erosions or ulcers, making its endoscopic diagnosis challenging [4]. ...
View
Case report: Endoscopic Hemoclip treatment for massive hematemesis caused by a Dieulafoy’s lesion on duodenal papilla caused acute pancreatitis
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Background: Dieulafoy's lesion is an uncommon cause of hemorrhage of the digestive tract. It often presents with urgent and massive bleeding usually leading to shock, even death. Dieulafoy's lesions have been reported throughout the digestive tract but which occurred on duodenal papilla were particularly rare and presented challenges in the choice of hemostasis. Case presentation: A 66-year-old man with melena for 2 days was admitted. Gastrointestinal endoscopy revealed blood clots covering the duodenal papilla with oozing blood. During the procedure of trying to place a plastic stent into the duodenal papilla first, the hemorrhage began to present pulsating bleeding. The patient went into shock. With consent, two titanium clips were inserted to clamp the bleeding site to stop the bleeding. The patient complained of epigastric pain 14 h after the endoscopy. An abdominal CT scan showed signs of acute pancreatitis. Endoscopy was performed to remove the titanium clips and showed a vessel stump on the duodenal papilla. The patient was discharged from the hospital on the 14th day and followed for 6 months with no recurrence. Conclusion: This case was diagnosed with a Dieulafoy's lesion on the duodenal papilla, which has rarely been reported. Hematemesis was stopped by clamping the vessel stump with titanium clips but caused acute pancreatitis. Reviewing the treatment, electrocoagulation might be a better choice, and life support treatment, including central vena catheterization and an adequate supply of blood products, should be prepared in advance to provide extra time for the stent placement or vascular intervention treatment.
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Duodenal Dieulafoy lesion: a rare pathogeny of gastrointestinal bleeding in children
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Background: Dieulafoy lesion is a calibre persistent submucosal artery associated with a minuter mucosal defect. Dieulafoy lesion has been reported to account for 1-5.8% of acute nonvariceal upper gastrointestinal bleeding in adults, but it is rarely reported in children. Case: Here we report a case of duodenal Dieulafoy lesion in a 13-year-old boy. After endoscopy and laparotomy, he still had no definite diagnosis and effective treatment. The duodenal Dieulafoy lesion was finally identified by selective angiography and was effectively treated by intravascular embolization. Conclusions: For unexplained upper gastrointestinal bleeding, the possibility of duodenal Dieulafoy lesion should be considered. A combination of multiple diagnosis and treatment methods can improve the success rate of diagnosis and treatment when a single test or treatment method cannot provide definitive diagnosis or effective treatment.
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Current views on the etiology, pathogenesis, diagnosis and treatment of Dieulafoy syndrome (review of literature)
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The article is devoted to Dieulafoy syndrome, a rare pathology that manifests itself by intense bleeding from a defect of abnormally dilated vessels of the submucosal layer of the stomach. The article discusses the main methods of diagnosis, treatment and prevention of Dieulafoy syndrome, lists the synonyms of «Dieulafoy syndrome». A brief historical reference is given. The definition of the concept of «Dieulafoy syndrome» is given. The age and gender prevalence of this syndrome is analyzed. Variants of localization of the lesion in this syndrome are described. The main causes and risk factors for the occurrence of Dieulafoy syndrome are highlighted. The most common clinical manifestations are characterized. The advantages and disadvantages of various methods of diagnosis of Dieulafoy syndrome are described. Special attention is paid to the diagnosis and criteria for endoscopic verification of this syndrome. Various methods of treatment have been investigated: conservative, endoscopic and operative. The indications and contraindications to each of the treatment methods are described, as well as their advantages and disadvantages. Possible combinations of various methods of endoscopic hemostasis are listed. The question of choosing the optimal combination of different methods of endoscopic hemostasis is highlighted. Risk factors for recurrence of bleeding have been determined. The frequency of recurrence of bleeding in Dieulafoy syndrome was analyzed. A number of unresolved issues related to the diagnosis and treatment of this syndrome have been identified, such as issues of effective prevention and prediction of recurrent bleeding.
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