Duodenal and gastric Dieulafoy's lesions in a patient with type 2A von Willebrand's disease

doi:10.1016/S0016-5107(04)02636-7

Gastrointestinal Endoscopy

Volume 61, Issue 2, February 2005, Pages 322-325

https://doi.org/10.1016/S0016-5107(04)02636-7 Get rights and content

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Case report

A 65-year-old man with VWD-2A was hospitalized because of severe anemia and melena. The Hb level was 5.3 g/dL (normal: 12-16 g/dL); hematocrit, 17.5% (37%-47%); and red blood cell count, 2.15 × 10⁶/μL) (4.0-5.4 × 10⁶/μL). The patient was one of 7 affected members of a family with VWD-2A. A type 2A abnormality was confirmed by gene screening, which revealed a mutation within the A2 domain that produced a V1607D substitution.⁸ The medical history included a partial gastric resection with

Discussion

GI bleeding is a clinically significant problem in patients with congenital bleeding disorders. Episodes of GI bleeding are frequent in patients with VWD, occurring in about 14% of affected individuals vs. 1% of the general population.¹⁰ Angiodysplasia has been found in all segments of the GI tract in patients with VWD.10, 11 Angiodysplasia is the pathology description for acquired tortuous dilatations of submucosal veins and capillaries in the GI tract; it is responsible for 6% of episodes of

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Life-threatening haemorrage from a gastric Dieulafoy lesion in a child with Haemophilia

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Cited by (5)

Dieulafoy's lesion in the cecum: A rare case report presentation
2021, International Journal of Surgery Case Reports
Citation Excerpt :
The second hypothesis is based on a degenerative background caused by oxidative and ischemic stress of diverse or even multifactorial causes, such as chronic gastritis, previous surgeries, and alcohol consumption or non-steroidal anti-inflammatory drug usage [2,4,10–12]. Despite its uncertain pathogenesis, it is known that these vascular lesions are more frequent in the elderly or individuals with more underlying diseases [2,4,9,11,13]. Dieulafoy's lesions affect males twice as often as females and can occur at any age [2,5,14].
Dieulafoy's lesion is a rare entity, normally present in the stomach and more rarely in the colon, and it is responsible for 1% to 5% of acute gastrointestinal bleeding cases. Its true incidence may be underrated, since most cases are asymptomatic and difficult to diagnose despite endoscopic advances. We present a clinical case of acute gastrointestinal bleeding due to Dieulafoy's lesion in the cecum.
An 85-year-old woman presented with a clinical condition of haematochezia associated with anaemia and haemodynamic instability, needing blood transfusion. Colonoscopy demonstrated a Dieulafoy's lesion in the cecum with active bleeding, and haemostasis was performed successfully with localized adrenaline injection and haemostatic clip placement. Hospitalization occurred without further complications.
The diagnosis of Dieulafoy's lesion is difficult because it is a rare condition and thus, usually not included in the differential diagnosis of gastrointestinal bleeding. Its endoscopic diagnostic and therapeutic approach is the standard method in the event of an acute gastrointestinal bleeding episode, with greater efficiency with the combined use of haemostatic techniques. Surgery is necessary in less than 5% of cases when bleeding is not effectively controlled by endoscopic or angiographic techniques.
It is essential to be aware of this lesion as a possible cause of gastrointestinal bleeding and differentiate it from other causes. Advances in endoscopy have increased the rate of diagnosis of these lesions and reduced their associated mortality.
Hemostasis of Dieulafoy's lesions by argon plasma coagulation (with video){A figure is presented}
2007, Gastrointestinal Endoscopy
Citation Excerpt :
After the exclusion of 4 patients in whom hemostasis was successfully performed with endoclips alone, 23 DLs treated with APC in 22 patients were considered for analysis. Two DLs were observed within a 4-month period in 1 patient with von Willebrand's disease.12 DL was detected at the first episode of bleeding in 18 patients (82%), whereas 2 to 4 acute bleeding episodes recurred before the diagnosis in the other 4 patients.
A Dieulafoy's lesion is a submucosal artery that may erode the epithelium and lead to severe hemorrhages. The safety and efficacy of argon plasma coagulation (APC) for the hemostasis of these lesions has not been studied.
To evaluate efficacy of APC alone in the hemostasis of Dieulafoy's lesions.
A retrospective analysis of hemostasis by chart review, with long-term follow-up by outpatient visit or phone interview.
An academic hospital with 24-hour endoscopic service availability.
All patients with acute bleeding from a Dieulafoy's lesion treated with APC.
Hemostasis was attempted with 2.3-mm APC probes, with settings varying from 40 W to 60 W, according to lesion location.
Initial hemostasis, recurrent bleeding, and 30-day mortality rates.
Twenty-three Dieulafoy's lesions were treated with APC, which represented 85% of all such lesions observed. Severe comorbidities and abnormal coagulation were present in 39% and 22%, respectively. Dieulafoy's lesions were located in the upper-GI tract in 20 patients (87%). Active bleeding was found in 20 patients (87%), a nonbleeding visible vessel was found in 2 patients (9%), and a minute mucosal defect below an adherent clot was found in 1 (4%). Initial hemostasis was achieved in all patients, without complications. An injection of an average volume of 3 mL of 1:10,000 epinephrine solution preceded APC in 3 cases for the identification of the bleeding lesion. Recurrent bleeding occurred in a patient after 48 hours; no bleeding-related deaths were observed during a median follow-up of 29 months.
Retrospective study.
Dieulafoy's lesions can be successfully managed by APC alone.
Vascular abnormalities in patients with von Willebrand disease: A scoping review
2021, Journal of Thrombosis and Haemostasis
Dieulafoy's lesion: Current trends in diagnosis and management
2010, Annals of the Royal College of Surgeons of England
Management of inherited von Willebrand disease in 2006
2006, Seminars in Thrombosis and Hemostasis

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Case ReportDuodenal and gastric Dieulafoy's lesions in a patient with type 2A von Willebrand's disease

Section snippets

Case report

Discussion

First page preview

J Am Coll Surg

Am J Gastroenterol

Gastrointest Endosc

Am J Gastroenterol

Gastrointest Endosc

Gastrointest Endosc

J Vasc Interv Radiol

Hum Pathol

Endoscopic injection treatment of gastrointestinal bleeding in hemophiliacs

World J Surg

Vascular disease of the small intestine

The correlation of endoscopically identified vascular lesions to their pathological diagnosis

Gastrointest Endosc

Life-threatening haemorrage from a gastric Dieulafoy lesion in a child with Haemophilia

Haemophilia

Case Report
Duodenal and gastric Dieulafoy's lesions in a patient with type 2A von Willebrand's disease