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Angioectasias on the major and accessory duodenal papillae; a unique cause of recurrent bleed in a patient with von Willebrand's disease
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  1. Dimitrios E Sigounas1,
  2. Diana E Yung1,
  3. John N Plevris1,2,
  4. Anastasios Koulaouzidis1
  1. 1Centre of Liver and Digestive Disorders, Royal Infirmary of Edinburgh, Edinburgh, UK
  2. 2Medical School, The University of Edinburgh, Edinburgh, UK
  1. Correspondence to Dr Anastasios Koulaouzidis, akoulaouzidis{at}hotmail.com

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Description

An 83-year-old woman with von Willebrand's disease (vWD) presented with multiple episodes of gastrointestinal bleeding over the past few years, leading to symptomatic anaemia. She underwent bidirectional negative endoscopies and MR cholangiopancreatography (MRCP). Small bowel capsule endoscopy (SBCE) revealed intraluminal blood clots and a P01 lesion on the ampulla of Vater (AoV) (figure 1A, B). Further upper digestive endoscopy with a side-viewing endoscope identified active bleeding from both the major and accessory papillae (figure 2A–D). Argon plasma coagulation (APC) was applied for haemostasis. Repeat SBCE documented active bleeding from the previously seen P0 lesion on the AoV (figure 3). Repeat APC on both papillae was performed with improvement of anaemia, although it was still ongoing. The patient remained asymptomatic thereafter.

Figure 1

(A and B) P0 lesion at the ampulla of Vater (AoV), first seen on initial small bowel capsule endoscopy.

Figure 2

(A–D) Active bleeding from both major and accessory papillae, as seen via side-viewing endoscope.

Figure 3

Active bleeding from the same lesion as in figure 1, documented on repeat small bowel capsule endoscopy.

Bleeding from the AoV is a rare SBCE finding. However, an association between vWD and small bowel angioectasias has been suggested; in an international survey regarding the complications of vWD, the prevalence of angiodysplasias in type 2 vWD was 2%.2 This is a rare report of bleeding angiodysplasias on the major and accessory papillae in a patient with vWD. This ‘rarity’ might be due to difficulty assessing the papilla using forward-viewing gastroscopes as well as the inherent inability of SBCE to visualise the AoV in the majority of cases.3 It is difficult to explain why such lesions were located on the major and accessory papillae. In the context of vWD, depletion of vWD factor from terminal vessels in the sphincter areas where shear flow is high is a plausible explanation.

Learning points

▸ Red spots are usually characterised as P0 and considered of little clinical significance.

▸ However, such lesions may become more significant in the context of a patient with bleeding diathesis: in our case, it was the cause of recurrent anaemia.

References

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Footnotes

  • Contributors DES and AK collected the images and wrote up the case. DEY performed further editing and prepared the case for submission. JNP supervised the process as a whole.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.