Continuing Education Activity

Dieulafoy lesion is a developmental vascular malformation of the gastrointestinal tract (GIT). It is an enlarged submucosal blood vessel that bleeds in the absence of any abnormality, such as ulcers or erosions. The signs and symptoms are related to blood loss, either due to intermittent or massive gastrointestinal hemorrhage. To avoid the complications associated with this condition, it must be promptly diagnosed and treated. This activity reviews the pathophysiology, evaluation, and treatment of Dieulafoy lesions and highlights the healthcare team's role in evaluating and treating patients with this condition.

Objectives:

• Describe the pathophysiology of Dieulafoy lesions.
• Summarize the epidemiology of Dieulafoy lesions.
• Describe the typical imaging findings associated with Dieulafoy lesions.
• Outline the typical presentation of a patient with Dieulafoy lesions.

Introduction

Dieulafoy lesion is a developmental vascular malformation of the gastrointestinal tract (GIT) first described by a French Surgeon, Paul Georges Dieulafoy, in 1898 as “exulceratio simplex.” It is a rare cause of life-threatening gastrointestinal bleeding that constitutes about 1% to 2% of all the causes of gastrointestinal bleeding and 6.5% of all causes of upper gastrointestinal non-variceal bleeding.[1][2] 

The Dieulafoy lesion is composed of a normal blood vessel with an abnormally wide diameter of 1 to 3 mm, protruding into the mucosa from the submucosa. The protrusion results in a small wall defect in GIT with fibrinoid necrosis at the base of the lesion; 70% of these lesions are found in the stomach, usually along the lesser curvature.

Etiology

A Dieulafoy lesion is an enlarged submucosal blood vessel that bleeds in the absence of any abnormality such as ulcers or erosions. The majority of the lesions arise in the stomach at the lesser curvature, within 6 cm of the gastroesophageal junction, as this region receives its arterial blood supply directly from the branches of the left gastric artery.[3] 

Less commonly, extra-gastric lesions may also arise in the duodenum, colon, jejunum, esophagus, or surgical anastomosis site.[4] Bleeding from a Dieulafoy lesion is most commonly associated with comorbid conditions like cardiovascular disease, chronic kidney disease, hypertension, peptic ulcer disease, diabetes mellitus, and chronic use of certain drugs(nonsteroidal anti-inflammatory drugs(NSAIDs) and anticoagulants).[5]

Epidemiology

Dieulafoy lesion accounts for 6.5% of the causes of upper gastrointestinal hemorrhage.[2] In adults, they are found twice as frequently in men as in women (2 to 1).[6] In the pediatric population, they occur in an equal distribution among the male and female populations.[7] The lesions reportedly present in all age groups; however, they are most common in the elderly in their fifth decade of life with multiple comorbidities such as cardiovascular disease, hypertension, chronic kidney disease, and diabetes mellitus.[5]

Pathophysiology

There are several possible theories about the mechanism that results in the development of Dieulafoy lesions and their complications. The three main theories include the following:

• Pulsations of an abnormally large artery may disrupt the mucosal surface leading to exposure of the artery to the bowel contents resulting in mechanical and chemical erosion and bleeding[8]
• Wear and tear of the gastric mucosa may lead to arterial thrombus formation resulting in necrosis and bleeding.
• Age-related gastric mucosal atrophy

Several factors appear to trigger the rupture of the tortuous vessel, including nonsteroidal anti-inflammatory drugs (NSAIDs), tobacco, alcohol, and peptic ulcer disease (PUD); however, there is no proven association yet.

Histopathology

A biopsy is not recommended for the diagnosis of Dieulafoy lesions due to an increased risk of bleeding. Histological examination of the lesion in the past revealed a normal artery with the presence of occasional amyloid deposits.[9] 

Macroscopically, the lesion resembles a pseudopolyp. Lesions that are actively bleeding have a wide arterial size of 1 to 2 mm, with arterial spurting and mucosal erosions measuring up to 5 mm. Microscopically, the artery has an elastic lamina and impinges on the muscularis mucosae. Dieulafoy lesion can be distinguished from gastric ulcers on histology by a lack of subintimal fibrosis and mucosal inflammation.[10]

History and Physical

Dieulafoy lesions are often asymptomatic unless the mucosa overlying the vessel erodes, resulting in bleeding. The signs and symptoms are related to blood loss, either due to intermittent or massive gastrointestinal hemorrhage. Melena is the most common presentation, followed by hematemesis, hematochezia, iron deficiency anemia (IDA), hemoptysis, or hemodynamic instability. In rare circumstances, Dieulafoy lesion of the gall bladder may present with upper abdominal pain in the absence of overt bleeding.[11] 

Evaluation

Direct endoscopic visualization of the Dieulafoy lesion is the gold standard method for diagnosis. Most patients are diagnosed after a single endoscopy; however, poor visualization of the GIT due to food particles or blood may require multiple endoscopies. The endoscopic visual diagnostic criteria that are necessary for a diagnosis of Dieulafoy lesion is as following:

• Normal mucosa around the small defected mucosal lesions which has active pulsatile bleeding smaller than 3 mm
• The visualization of a protruding vessel from a slight defect or normal mucosa
• The observation of a fresh clot attached to a defect of normal mucosa[12]

If lesions are not detected by endoscopy, angiography and capsule endoscopy can be used to confirm the diagnosis. On an angiogram, there is an extravasation of contrast into the GIT from the eroded artery. The presence of tortuous vessels in the arterial phase with no early venous return also indicates Dieulafoy lesions. The bleeding source is visualizable using technetium-99m red blood cell scanning or computerized tomography (CT) angiography.[13] 

Treatment / Management

The first-line treatment modality for Dieulafoy lesion is endoscopy.[14] In the past, surgical treatment with gastrostomy or gastrectomy was performed; however, it has been primarily replaced by endoscopy since 1986. The three commonly performed endoscopic treatment procedures include:

• Thermal, heat probe, or argon plasma coagulation
• Regional injection-epinephrine (EPI) or norepinephrine(NOR) injection and sclerotherapy
• Mechanical banding and hemoclips[6][15]

Mechanical hemostasis comprising of banding and hemoclips is the safest and most effective treatment.[16] A study by Alis et al. revealed that endoscopic band ligation (EBL) was associated with a lower risk of recurrent bleeding and shorter hospital stay compared with sclerotherapy.[15] Hemoclipping has proven effective in the treatment of Dieulafoy lesions with a success rate of 95%.[17] Combination therapy has better outcomes when compared with monotherapy for Dieulafoy lesions. During endoscopic treatment, the bleeding site is often tattooed with India ink for ease of future identification in a recurrent bleed event.[18] If endoscopic treatment fails, surgical treatment is performed, including wide wedge resection or partial /wedge gastrectomy.[19] 

Differential Diagnosis

Dieulafoy lesion has a vast number of differential diagnoses based on the patients’ age, comorbidities, and overall health. Angiodysplasia is an important differential due to similar presenting symptoms. It can be differentiated on angiography, by the presence of arteriovenous shunting and vascular ectasia, and by histological examination of the lesion, which reveals the presence of abnormal submucosal vessels. Other similar conditions should be differentiated from Dieulafoy lesion based on presenting signs and symptoms, endoscopy, and angiography. These include diverticulosis, telangiectasias, vascular neoplasms, mucosal spider nevi, and connective tissue disorders.

Prognosis

The mortality and morbidity of Dieulafoy lesions have significantly reduced to 8.6% since the development of endoscopy.[8] Hemostasis is successfully achievable in 80 to 85% of the cases treated with endoscopy.

Complications

Due to the subtle nature of the disease, it is often missed as a diagnosis initially and may result in delayed treatment resulting in adverse complications. The most common complication is massive spontaneous gastrointestinal hemorrhage that may be fatal if left untreated. Intermittent blood loss may lead to iron deficiency anemia (IDA) and, in severe cases, hemodynamic instability. In some cases, it may lead to the worsening of pre-existing co-morbidities.

Deterrence and Patient Education

Patients need to minimize the risk factors for Dieulafoy lesion by avoiding the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and alcohol. Regular follow up with a gastroenterologist is necessary to monitor the condition and prevent a recurrence.

Enhancing Healthcare Team Outcomes

Most cases of Dieulafoy lesions go undiagnosed due to lack of awareness, acute onset of signs and symptoms, intermittent bleeding pattern, and difficulty in visualization on endoscopy. On endoscopy, Dieulafoy lesion may be difficult to diagnose due to its small size or the presence of normal-appearing surrounding mucosa.[9] 

Excessive blood in the GIT may also prevent visualization and further delay diagnosis. For these reasons, physicians and endoscopists should consider this condition as a differential diagnosis for intermittent or massive GIT bleeding. Radiologists should also be aware of the subtle nature of this condition that may be missed in a patient with gastrointestinal bleeding. Once diagnosed, endoscopy should be the preferred treatment since it has replaced invasive surgery due to better health care outcomes. During the evaluation of the lesion, it is essential to determine the focal point of the bleeding for the management of future episodes.