Dieulafoy's lesion () is a medical condition characterized by a large tortuous
artery
An artery () is a blood vessel in humans and most other animals that takes oxygenated blood away from the heart in the systemic circulation to one or more parts of the body. Exceptions that carry deoxygenated blood are the pulmonary arteries in ...
most commonly in the
stomach
The stomach is a muscular, hollow organ in the upper gastrointestinal tract of Human, humans and many other animals, including several invertebrates. The Ancient Greek name for the stomach is ''gaster'' which is used as ''gastric'' in medical t ...
wall (
submucosa
The submucosa (or tela submucosa) is a thin layer of tissue in various organs of the gastrointestinal, respiratory, and genitourinary tracts. It is the layer of dense irregular connective tissue that supports the mucosa (mucous membrane) an ...
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 Digestion, digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascula ...
.
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, ...
but is relatively uncommon. It is thought to cause less than 5% of all
gastrointestinal bleeds 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 :wikt:bulge, 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 b ...
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) and/or black stools (
melena).
Less often, Dieulafoy's lesions may cause rectal bleeding (
hematochezia), 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, sh ...
. 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).
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 the continued drinking of alcohol despite it causing problems. Some definitions require evidence of dependence and withdrawal. Problematic use of alcohol has been mentioned in the earliest historical records. The World Hea ...
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 capillary, capillaries.
Arterioles have vascular smooth muscle, muscular walls (usually only one to two layers of smoo ...
) beneath the gastrointestinal mucosa (
submucosa
The submucosa (or tela submucosa) is a thin layer of tissue in various organs of the gastrointestinal, respiratory, and genitourinary tracts. It is the layer of dense irregular connective tissue that supports the mucosa (mucous membrane) an ...
) 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, most commonly in the
lesser curvature. 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 Digestion, digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascula ...
.
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 vertebrates, including mammals, reptiles, and birds. In mammals, it may be the principal site for iron absorption.
The duodenum precedes the jejunum and ileum and is the shortest p ...
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 specialized for the absorption by enterocytes of small nutrient molecules which have been pr ...
(1%) and the
esophagus
The esophagus (American English), oesophagus (British English), or œsophagus (Œ, archaic spelling) (American and British English spelling differences#ae and oe, see spelling difference) all ; : ((o)e)(œ)sophagi or ((o)e)(œ)sophaguses), c ...
(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, 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 a medical procedure involving the Endoscopy, endoscopic examination of the large bowel (colon) and the distal portion of the small bowel. This examination is performed using either a Charge-coupled device, CCD ...
. 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 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 perfo ...
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
Colonoscopy () or coloscopy () is a medical procedure involving the Endoscopy, endoscopic examination of the large bowel (colon) and the distal portion of the small bowel. This examination is performed using either a Charge-coupled device, CCD ...
.
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 a ...
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 medication is injected into the vessels ...
,
heater probe,
laser photocoagulation,
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 Fluoroscopy, x-ray fluoroscopy, CT scan, computed tomography, magnetic resonance imaging, or ultraso ...
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 Statistical population, population, scaled to the size of that population, per unit of time. Mortality rate is typically ...
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