Dieulafoy's Lesion
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Dieulafoy's lesion (or Dieulofoy lesion) is a medical condition characterized by a large tortuous
arteriole An arteriole is a small-diameter blood vessel in the microcirculation that extends and branches out from an artery and leads to capillaries. Arterioles have muscular walls (usually only one to two layers of smooth muscle cells) and are the primar ...
most commonly in the
stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...
wall (
submucosa The submucosa (or tela submucosa) is a thin layer of tissue (biology), tissue in various organ (anatomy), organs of the gastrointestinal tract, gastrointestinal, respiratory tract, respiratory, and genitourinary system, genitourinary tracts. It i ...
l) that erodes and bleeds. It can present in any part of the
gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organ (biology), organs of the digestive syste ...
. It can cause gastric
hemorrhage Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vag ...
but is relatively uncommon. It is thought to cause less than 5% of all
gastrointestinal bleed Gastrointestinal bleeding (GI bleed), also called gastrointestinal hemorrhage (GIB), is all forms of bleeding in the gastrointestinal tract, from the mouth to the rectum. When there is significant blood loss over a short time, symptoms may incl ...
s in adults. It was named after French surgeon Paul Georges Dieulafoy, who described this condition in his paper "Exulceratio simplex: Leçons 1-3" in 1898. It is also called "caliber-persistent artery" or "aneurysm" of gastric vessels. However, unlike most other
aneurysm An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus (s ...
s, these are thought to be developmental malformations rather than degenerative changes.


Signs and symptoms

Dieulafoy's lesion often do not cause symptoms (asymptomatic). When present, symptoms usually relate to painless bleeding, with vomiting blood (
hematemesis Hematemesis is the vomiting of blood. It is always an important sign. It can be confused with hemoptysis (coughing up blood) or epistaxis (nosebleed), which are more common. The source is generally the upper gastrointestinal tract, typically abo ...
) and/or black stools (
melena Melena or melaena refers to the dark black, tarry feces that are associated with upper gastrointestinal bleeding. The black color and characteristic strong odor are caused by hemoglobin in the blood being altered by digestive enzymes and intest ...
). Less often, Dieulafoy's lesions may cause rectal bleeding (
hematochezia Haematochezia is the passage of fresh blood through the anus path, usually in or with stools (contrast with melena). The term is from Greek αἷμα ("blood") and χέζειν ("to defaecate"). Hematochezia is commonly associated with lower gastro ...
), or rarely,
iron deficiency anemia Iron-deficiency anemia is anemia caused by a lack of iron. Anemia is defined as a decrease in the number of red blood cells or the amount of hemoglobin in the blood. When onset is slow, symptoms are often vague such as feeling tired, weak, shor ...
. Usually, there are no gastrointestinal symptoms that precede the bleeding (abdominal pain, nausea, etc.). Though exceptionally rare, cases of Dieulafoy lesions occurring in the gallbladder can cause upper abdominal pain, which is usually right upper quadrant or upper middle (
epigastric In anatomy, the epigastrium (or epigastric region) is the upper central region of the abdomen. It is located between the costal margins and the subcostal plane. Pain may be referred to the epigastrium from damage to structures derived from the f ...
). Though gallbladder Dieulafoy lesions usually occur with anemia (83%), they generally do not cause overt bleeding (hematochezia, hematemesis, melena, etc.).


Cause

In contrast to peptic ulcer disease, a history of
alcohol use disorder Alcoholism is, broadly, any drinking of alcohol that results in significant mental or physical health problems. Because there is disagreement on the definition of the word ''alcoholism'', it is not a recognized diagnostic entity. Predomin ...
or
NSAID Non-steroidal anti-inflammatory drugs (NSAID) are members of a therapeutic drug class which reduces pain, decreases inflammation, decreases fever, and prevents blood clots. Side effects depend on the specific drug, its dose and duration of ...
use is usually absent in Dieulafoy's lesion.


Pathophysiology

Dieulafoy lesions are characterized by a single abnormally large blood vessel (
arteriole An arteriole is a small-diameter blood vessel in the microcirculation that extends and branches out from an artery and leads to capillaries. Arterioles have muscular walls (usually only one to two layers of smooth muscle cells) and are the primar ...
) beneath the gastrointestinal mucosa (
submucosa The submucosa (or tela submucosa) is a thin layer of tissue (biology), tissue in various organ (anatomy), organs of the gastrointestinal tract, gastrointestinal, respiratory tract, respiratory, and genitourinary system, genitourinary tracts. It i ...
) that bleeds, in the absence of any ulcer, erosion, or other abnormality in the mucosa. The size of these blood vessels varies from 1–5 mm (more than 10 times the normal diameter of mucosal capillaries). Pulsation from the enlarged vessels leads to focal pressure that causes thinning of the mucosa at that location, leading to exposure of the vessel and subsequent hemorrhage. Approximately 75% of Dieulafoy's lesions occur in the upper part of the stomach within 6 cm of the
gastroesophageal junction The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...
, most commonly in the
lesser curvature The curvatures of the stomach refer to the greater and lesser curvatures. The greater curvature of the stomach is four or five times as long as the lesser curvature. Greater curvature The greater curvature of the stomach forms the lower left ...
. However, Dieulafoy's lesions may occur in any part of the
gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organ (biology), organs of the digestive syste ...
. Extragastric lesions have historically been thought to be uncommon but have been identified more frequently in recent years, likely due to increased awareness of the condition. The
duodenum The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine m ...
is the most common location (14%) followed by the colon (5%), surgical anastamoses (5%), the
jejunum The jejunum is the second part of the small intestine in humans and most higher vertebrates, including mammals, reptiles, and birds. Its lining is specialised for the absorption by enterocytes of small nutrient molecules which have been previous ...
(1%) and the
esophagus The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...
(1%). Dieulafoy's lesions have been reported in the gallbladder. The pathology in these extragastric locations is essentially the same as that of the more common gastric lesion.


Diagnosis

A Dieulafoy's lesion is difficult to diagnose, because of the intermittent pattern of bleeding. Dieulafoy's lesion are typically diagnosed during endoscopic evaluation, usually during
upper endoscopy Esophagogastroduodenoscopy (EGD) or oesophagogastroduodenoscopy (OGD), also called by various other names, is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract down to the duodenum. It is considered ...
, which may show an isolated protruding blood vessel. Lesions affecting the colon or end of the small bowel (
terminal ileum The ileum () is the final section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear and the terms posterior intestine or distal intestine ma ...
) may be diagnosed during
colonoscopy Colonoscopy () or coloscopy () is the endoscopic examination of the large bowel and the distal part of the small bowel with a CCD camera or a fiber optic camera on a flexible tube passed through the anus. It can provide a visual diagnosis (''e. ...
. Dieulafoy's lesions are not easily recognized and therefore multiple evaluations with endoscopy may be necessary. Once identified during endoscopy, the mucosa near a Dieulafoy's lesion may be injected with ink. Tattooing the area can aid in identifying the location of the Dieulafoy's lesion in the event of rebleeding.
Endoscopic ultrasound Endoscopic ultrasound (EUS) or echo-endoscopy is a medical procedure in which endoscopy (insertion of a probe into a hollow organ) is combined with ultrasound to obtain images of the internal organs in the chest, abdomen and colon. It can be used ...
has been used both to facilitate identification of Dieulafoy lesions and confirm the treatment success.
Angiography Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is performe ...
may be helpful with diagnosis, though this only identifies bleeding that actively occurs during the time of that test. Mesenteric angiography may be particularly helpful for Dieulafoy lesions in the colon or rectum, where the evaluation may be limited by the presence of blood or poor
bowel preparation Bowel cleansing or bowel preparation is a medical technique to cleanse bowel to prepare colonoscopy or colorectal surgery. Cleansing can be performed either by the oral ingestion of medication or by enema. Polyethylene glycol and citrates are used ...
.


Treatment

In most cases, Dieulafoy lesions are treated with endoscopic interventions. Endoscopic techniques used in the treatment include
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands and ...
injection followed by bipolar or monopolar
electrocoagulation Electrocoagulation (EC) is a technique used for wastewater treatment, wash water treatment, industrially processed water, and medical treatment. Electrocoagulation has become a rapidly growing area of wastewater treatment due to its ability to remo ...
, injection
sclerotherapy Sclerotherapy (the word reflects the Greek ''skleros'', meaning ''hard'') is a procedure used to treat blood vessel malformations (vascular malformations) and also malformations of the lymphatic system. A medicine is injected into the vessels, wh ...
, heater probe,
laser photocoagulation Laser coagulation or laser photocoagulation surgery is used to treat a number of eye diseases and has become widely used in recent decades. During the procedure, a laser is used to finely cauterize ocular blood vessels to attempt to bring about var ...
, hemoclipping or banding. In cases of refractory bleeding,
interventional radiology Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs bo ...
may be consulted for an angiogram with subselective embolization.


Prognosis

The
mortality rate Mortality rate, or death rate, is a measure of the number of deaths (in general, or due to a specific cause) in a particular population, scaled to the size of that population, per unit of time. Mortality rate is typically expressed in units of de ...
for Dieulafoy's was much higher before the era of endoscopy, where open surgery was the only treatment option. Mortality has decreased from 80% to 8% as a result of endoscopic therapies. Long term control of bleeding (hemostasis) is achieved in 85 - 90 percent of cases.


Epidemiology

Dieulafoy's lesions account for roughly 1.5 percent of gastrointestinal hemorrhage. These lesions are twice as common in men, and often occur in older individuals (over 50 years of age) with multiple comorbidities, including hypertension, cardiovascular disease, chronic kidney disease, and diabetes. Dieulafoy's lesions present in individuals with an average age of 52 years.


History

Dieulafoy's lesion was first described in 1884 by M.T. Gallard. The lesion was named after French surgeon Paul Georges Dieulafoy, who described the condition in his paper "Exulceratio simplex: Leçons 1-3" in 1898.G. Dieulafoy. Exulceratio simplex: Leçons 1-3. In: G. Dieulafoy, editor: Clinique medicale de l'Hotel Dieu de Paris. Paris, Masson et Cie: 1898:1-38. Dieulafoy believed (incorrectly) the bleeding from this lesion was due to erosions of the mucosa in the stomach.


References


External links

{{Digestive system diseases Stomach disorders