Two cases of Dieulafoy’s lesion in unusual sites: A rare case reports of gastrointestinal bleeding

Abstract

Introduction: Dieulafoy's lesion (DL) is a vascular malformation that can lead to massive gastrointestinal bleeding. It's usually found in the stomach. However, DL's occurrence in atypical sites such as the small bowel and colon is exceptionally rare, posing significant management challenges.

Case presentation: In this report, we present two cases of DL occurring in uncommon sites, each managed with distinct approaches. Case 1 is a 50-year-old man admitted to the emergency department due to massive GI bleeding and hemodynamic instability. The diagnosis of DL was established through computed tomography angiography and confirmed by histopathological examination after emergency surgery.

Case 2 involves a 68-year-old woman presented with melena due to a colonic DL. This case was successfully managed through an endoscopic hemostasis approach.

Discussion: Dieulafoy's lesions (DL) were first identified as a large submucosal artery lacking typical gastric ulcer characteristics in three of Paul Georges Dieulafoy's patients. This lesion is responsible for approximately 1–2 % of all cases of gastrointestinal bleeding. Endoscopy is the preferred method for diagnosing and managing DL lesions, especially in cases of active bleeding that is accessible. However, if endoscopic treatment or angiographic embolization fails, a surgical approach may be needed.

Conclusion: DL presents a diagnostic challenge due to its rarity and is not usually included in the differential diagnosis of gastrointestinal bleeding, particularly when occurring in unusual sites. Endoscopy is the preferred method to identify DL and a possible therapeutic approach in active bleeding. However, if endoscopy hemostasis fails, angiographic embolization or surgical intervention may be required.

Highlights

• •Dieulafoy's lesion (DL) is a rare vascular malformation that can cause potentially life-threatening bleeding.
• •DLs located in unusual sites such as the small intestine and colon present a challenge for diagnosis and management.
• •Endoscopic methods of hemostasis are the first choice for the management of DLs.
• •In cases of unsuccessful endoscopic hemostasis, surgery or selective arterial embolization may be required.

1. Introduction

Dieulafoy's lesion (DL) is a dilated and tortuous submucosal vessel that erodes the overlying mucosa without inflammatory or ulcerative mucosa [1]. The artery does not normally branch to the mucosa and its diameter of 1 to 3 mm, a caliber that is 10 times larger than a normal mucous capillary [2]. This arterial malformation is a grain-of-sand-sized lesion that appears as a mucosal defect with an artery protruding from its base. The small size and caliber of DL make their endoscopic diagnosis very difficult and can present a significant diagnostic challenge. Patients with DL typically present with comorbidities such as cardiovascular disease, high blood pressure, diabetes, chronic kidney disease, or alcohol consumption [3]. The use of non-steroidal anti-inflammatory drugs (NSAIDs) has also been associated with DL bleeding as it may cause mucosal atrophy and ischemic injury [1]. Although most DLs are usually found in the stomach or duodenum, only a few cases (2 %) are located in the small bowel or colon [4]. In These cases of DLs in atypical sites, diagnosing them poses a significant challenge and requires a multidisciplinary approach.

This work was reported in line with the SCARE guidelines [5].

2. Case Presentation 1

A 50-year-old man presented to the emergency department with a sudden onset of bloody stools. He had no medical history and didn't report hematemesis, abdominal pain, or recent use of NSAIDs. At admission, his pulse rate and blood pressure were within normal range, and his abdominal examination was normal. Rectal examination revealed the presence of hematochezia. Initial blood analysis showed a hemoglobin level of 11.1 g/dl and normal renal function. Upper gastrointestinal endoscopy was performed immediately, which did not reveal any sign of bleeding. Following this, a colonoscopy was performed, which revealed fresh blood in the ileum. However, the source of the bleeding could not be identified. The patient's hemodynamic condition rapidly worsened, marked by a drop in blood pressure to 100 mmHg/70 mmHg, and tachycardia. Blood tests revealed a decrease in hemoglobin level to 8.1 g/dl, a platelet count of 202/μl, an international normalized ratio (INR) of 1,29, prothrombin time (PT) of 63 %, and normal renal function. Consequently, the patient received intravenous fluid therapy and a transfusion of 2 units of packed red blood cells, followed by an emergency computed tomography angiography, which revealed active contrast extravasation in the middle section of the small bowel, without any visible mass (Fig. 3). Given the unavailability of arterial embolization and enteroscopy, an emergency laparotomy was performed.

Fig. 3.

CT angiography showing extravasation of contrast in the middle part of the small bowel.

During the surgical exploration, no intraperitoneal effusion or blood was observed in the peritoneal cavity. Palpation of the small bowel, guided by a CT scan, identified a round intraluminal formation in the mid-section, pinpointing the source of bleeding. Subsequently, a 2.5 cm segmental resection, including the lesion, followed by a manual end-to-end anastomosis. (Fig. 4). Upon macroscopic examination of the specimen, a polypoid lesion covered with a blood clot was noted. The lesion measured 1 cm by 1 cm by 1.2 cm, with secure margins of 0.5 cm and 1 cm on either side. Subsequent histopathological analysis confirmed the diagnosis of Dieulafoy's lesion (Fig. 1, Fig. 2). Following the surgery, the patient had no further bleeding and was discharged from the hospital after a five-day stay, with a hemoglobin level of 10.5 g/dl.

Fig. 4.

Surgical specimen showing a blood clot in the small bowel.

Fig. 1.

Illustra presents a sequence of cross-sections of the small bowel resection in the first case that identified a blood clot and the vessel protruding into the submucosa.

Fig. 2.

Pathology specimen showing Dieulafoy's lesion in the small bowel.

3. Case presentation 2

A 68-year-old woman with a medical history notable for arterial hypertension, diabetes, and heart failure, was under treatment with insulin, diuretics, and anticoagulants rivaroxaban as a necessary daily medication. She was admitted to our department for seven days of GI bleeding with black stool, abdominal fullness, and weakness. She had no prior history of GI bleeding, abdominal pain, or recent use of NSAIDs.

On physical examination, her blood pressure was normal (120/07 mmHg), and rectal examination revealed melenas. No abnormalities were noted during the remainder of the clinical examination. Blood analysis revealed a hemoglobin level of 8.5 g/dl, chronic renal failure with a creatinine level of 1.6 mg/dl and a glomerular filtration rate of 51 ml/min, an international normalized ratio (INR) of 1.2, prothrombin time (PT) of 71 %, which required intravenous fluid therapy and transfusion of 2 units of packed red blood cells. The patient's anticoagulation therapy was temporarily interrupted, and she underwent upper GI endoscopy, which showed no abnormalities. A colonoscopy was then performed, which showed fresh blood along the colonic mucosa, and visibility of a pulsating vessel with active oozing at the right colonic angle, consistent with a DL, that had been managed by argon plasma coagulation to stop bleeding. (Fig. 5). The patient's condition remained stable, and she was closely monitored during her hospital stay. After four days, she was discharged with no recurrence of GI bleeding and showed improvement in her hemoglobin levels during the follow-up period.

Fig. 5.

Dieulafoy's lesion in the right colonic angle, characterized by punctiform oozing bleeding, was efficiently achieved through endoscopic hemostasis using APC. (A, B, and C: Pulsatile bleeding from a small vessel surrounded by normal mucosa. D and E: Using APC to control the bleeding).

4. Discussion

Dieulafoy's lesions (DLs) were first identified as ‘exulceratio simplex’, a large submucosal artery lacking typical gastric ulcer characteristics in three of Paul Georges Dieulafoy's patients. It was until 1898 following the publication of Paul Georges Dieulafoy's work that the name Dieulafoy's lesion was designated [6]. DLs are more common in men than women with a sex ratio of 2:1 [7]. DLs usually affect the stomach in 72 % of cases and the duodenum in 15 % of cases. However, they can also occur in other sites, such as the esophagus (8 %), colon (2 %), rectum (2 %), and jejunum (2 %). The majority of cases are diagnosed at an advanced age, between 60 and 70 years [8] [9] [10].

Patients affected by DLs also have comorbidities like cardiopulmonary, renal disease, diabetes, or hypertension, as observed in our second case. In addition, the use of NSAIDs and anticoagulants has also been linked to DL bleeding [1]. The factors that led to Dieulafoy's rupture remain unclear, it consists of an aberrant submucous artery that erodes the overlying mucosa and does not normally branch to the mucosa, maintaining a diameter of 1 to 3 mm (a caliber 10 times larger than a normal mucous capillary). This aberrant artery can protrude through a small mucosal defect, and become susceptible to even minor mechanical trauma, such as the passage of a food bolus in the stomach or solid stool in the colon. Eventually, it can erode into the lumen to cause severe acute GI bleeding [11]. These lesions are typically located in the lesser curvature of the stomach or duodenum and they represent only 2 % of all gastrointestinal bleedings identified in the colon and 1 % in the jejunum. Gastrointestinal bleeding is a common symptom of these lesions and can vary from occasional to massive bleeding depending on its location [4].

The pathophysiology of these vascular abnormalities leading to mucosal ischemia and subsequent erosion remains incompletely understood. Some authors suggest that spontaneous bleeding underscores the potential role of oxidative and ischemic stress, triggered by various factors, such as the use of NSAIDs, alcohol consumption, chronic gastritis, and prior surgeries, all of which may contribute to vessel rupture. Congenital predisposition may also add to the pathogenesis. Another theory suggests arterial thrombosis as the cause of ischemia and bleeding. Despite its uncertain pathogenesis, it appears that vascular lesions are more common in older individuals or those with more underlying diseases [12].

The diagnosis of DL is challenging due to its rarity and the small size of the lesion, making it susceptible to being mistaken for other vascular abnormalities such as aneurysms or telangiectasia. Furthermore, it can occur in unusual sites within the digestive tract, such as the small bowel or colon, which makes it even more challenging to identify [10,13].

Endoscopy is the method of choice to identify DLs and a possible therapeutic approach during active bleeding. It may be necessary to repeat the endoscopic procedure when bleeding is minimal and recurrent. The presence of an isolated protruding vessel surrounded by normal mucosa without any ulcer or associated mass is a classic endoscopic finding [1,7,10]. However, upper GI endoscopy could fail to identify the source of bleeding. A colonoscopy is mandatory to identify a special location other than the gastric or duodenum in this condition. Otherwise, the diagnostic yield of this modality to diagnose DL is still unknown [12]. Enteroscopy with a single or double balloon is the best method to detect distal DL, with a diagnostic yield of 96 % to 98 % [10,14]. However, this procedure is not always feasible depending on the patient's hemodynamic status and the center's expertise. In our first case, when endoscopy failed to find the bleeding source (upper GI endoscopy was normal and on colonoscopy, there was blood in the ileum without finding the source of bleeding and the patient's hemodynamic state rapidly deteriorated, a computed tomography (CT) angiography was performed revealed active contrast extravasation in the small bowel's middle part with no visible mass (Fig. 3). Mesenteric angiography can be useful in cases of unidentified causes of bleeding with selective arterial embolization. This approach should be used when endoscopy fails to find the origin of bleeding, the lesion is not accessible by endoscopy and hemodynamic instability is evident [7,10], as in our first case, but is not available. In this situation, it's crucial the use CT angiography to identify the source of bleeding, it's a quick and simple technique that can detect bleeding rates as low as 0,5 ml/min [10,15]. In the first case, the patient was in his fifth decade of life and his CT angiography showed contrast extravasation in the jejunum. Surgery was performed to control the patient's GI bleeding.

The second case of DL in the right colon was diagnosed by colonoscopy and managed using endoscopic hemostasis techniques.

Management of DLs varies depending on their location, the patient's hemodynamic status, and the expertise of the medical team. Hemostatic therapy is crucial due to the severity of bleeding associated with DLs, the risk of acute recurrence if left untreated, and the significant mortality risk. Although several effective therapeutic approaches are available, including endoscopic hemostasis, angiographic embolization, and surgery, there are no consensus recommendations on treatment. However, endoscopic hemostasis remains the preferred treatment option, it is a safe and effective technique in controlling bleeding in more than 90 % of accessible lesions with a recurrence rate of less than 10 % rate, which has gradually decreased the mortality rate to 8 %. This endoscopic technique could be combined with injection therapy such as epinephrine or sclerosant agents and mechanical therapy like banding and haemoclipping to avoid rebleeding if it is used alone. Additionally, it is crucial to mark a tattoo at the site of DL (e.g., using Indian ink) to serve as a reference point in the event of recurrent bleeding. Endoscopic hemoclipping with or without epinephrine in comparison to band ligation with or without epinephrine, achieved a higher primary hemostasis rate (91 % vs. 79 %) and a lower rebleeding rate (9 % vs. 21 %), respectively. [14,16] [17]. Thermal endoscopic therapy is used to achieve hemostasis, it has been classified into two categories: contact non-contact approaches. Contact thermocoagulation includes bipolar and heater probes. While these methods are effective, there is a risk of transmural injury. Argon plasma coagulation (APC) is a non-contact thermocoagulation, which has been reported to be effective and safer compared to contact methods. Therefore, APC is considered a safe technique since it mainly induces superficial tissue injury, with a success rate of this hemostasis method was similar to endoscopic mechanical therapy [9,16,17].

Mechanical endoscopic methods such as hemoclipping and band ligation have a slight therapeutic superiority over other techniques. Both methods have similar efficacy in achieving hemostasis in DL bleeding since the hemoclipping technique is more feasible and was used in the majority of patients with accessible DLs. Combining haemoclipping with epinephrine injection further enhances its efficacy. The success of this approach depends on the location of the lesion, the position, and the expertise of the endoscopic team [9,17].

In our second case, we successfully applied APC as an endoscopic method to stop bleeding, with no immediate or long-term complications. However, in this case, Dieulafoy's lesion is located in the right colon behind a fold with an acute angulation, making the use of the mechanical method relatively difficult. The rate of recurrent bleeding following attempted endoscopic hemostasis varies between 9 % and 40 %, with a higher rate observed when endoscopic monotherapy is performed. In case of recurrence, repeated endoscopy is the preferred therapeutic approach. Various hemostatic therapies are effective in the management of DL bleeding, including arterial embolization and surgery. Surgery is considered to be the last resort of treatment. Unfortunately, 3 to 16 % of DLs will require surgery when endoscopic or angiographic treatment fails. This was the case for our first patient, who had a segmental resection of the small bowel to control the bleeding [1,7,9,10] (Fig. 4).

The mortality rate associated with acute bleeding from a Dieulafoy's lesion is lower compared to that from ulcerative disease. Previously, the mortality rate was up to 80 %. However, with the advent of therapeutic endoscopy, the diagnosis of DLs has increased, resulting in a significant decrease in the mortality rate to 9 % [12] [17].

5. Conclusion

DLs should be considered in the differential diagnosis when endoscopy fails to identify the bleeding source. Diagnosing this rare entity poses challenges, especially when located in unusual sites like the small bowel and colon. Its potential for causing massive GI bleeding with associated mortality underscores the importance of early and prompt recognition. Endoscopic treatment employing various techniques is preferred for diagnosing and managing DL when lesions are accessible, as seen in our second case. We adopted a multidisciplinary approach requiring the intervention of endoscopists, radiologists, surgeons, and pathologists to locate, diagnose, and treat DL efficiently. Unfortunately, due to the severity of the bleeding, surgery was the only option available for our first patient.

Patient consent

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

This report is about a clinical case that does not require approval from the ethics committee. Data were anonymously registered in the database of our department.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contribution

All the authors contributed to the management of the patients.

Abdelkrim Zazour wrote the manuscript.

Chifaa Belkhayat was involved in the data collection.

Amal Bennani contributed to the anatomopathological diagnosis.

Mohamed Bouziane participated in the surgical management of the patient.

Ghizlane Kharrasse and Zahi Ismaili revised and approved the final version of the manuscript.

Guarantor

Abdelkrim Zazour, Hepato-gastroenterology Department, Mohammed VI University

Hospital, Faculty of Medicine and Pharmacy of Oujda, Laboratory of Digestive Disease

Research, University of Mohammed First. Oujda, Morocco

Email: zazourabdelkrim1987@gmail.com/abdelkrim.zazour@gmail.com

Declaration of competing interest

The authors declare no conflicts of interest.