BMJ Case Reports | 2021
Successful management of severe gastrointestinal bleeding from jejunal angiodysplasia in a patient with Bernard-Soulier syndrome
Abstract
© BMJ Publishing Group Limited 2021. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A 57yearold woman presented to the emergency department with melena, easy fatiguability and dizziness for 2 days. She did not have haematemesis, abdominal pain, ascites or jaundice. Her routine master health checkup done 4 years previously revealed a diagnosis of BernardSoulier syndrome (BSS). She did not have any bleeding manifestation so far. Her personal history and family history were not contributory. On examination, she was drowsy, pale, tachycardic and hypotensive. Her abdomen examination was unremarkable, and digital rectal examination revealed melenic stool staining. Her blood investigations showed severe anaemia (haemoglobin: 54 g/L) and thrombocytopenia (platelet count: 65×10/L), with giant platelets on a peripheral smear. She received two units of packed red blood cells. Her initial upper endoscopy, colonoscopy and CT abdominal angiogram did not reveal any abnormality. Video capsule endoscopy showed multiple brisk bleeding spots in the proximal jejunum (figure 1A–D). She received octreotide infusion initially. However, there was no response for over 72 hours, and her haemoglobin value did not improve. Hence, we decided to proceed with diagnostic laparoscopy and intraoperative enteroscopy. It revealed multiple ectatic cherry red spots in proximal jejunum, with active diffuse pinpoint ooze. She underwent resection of 50 cm of proximal jejunum in the same sitting (figure 2A). Histopathological examination of the resected bowel confirmed diagnosis of angiodysplasia (AD) (figure 2B). She had an uneventful postoperative period and got discharged in a stable condition. She did not have further bleeding episodes and is doing well for the past 2 years. AD is the most frequently reported cause for bleeding from the small bowel. It presents as chronic anaemia due to occult gastrointestinal (GI) blood loss or an overt GI bleed like melena or haematochezia. The presence of a concomitant systemic condition like chronic kidney disease, aortic stenosis or haemostatic disorder increases the risk of bleeding from ADs. BSS presenting with GI bleeding from ADs is rarely encountered. 4 The association between BSS and AD is not well established, and whether such ADs are more prevalent in BSS or incidentally diagnosed because of bleeding is not known. Video capsule endoscopy is the firstline modality for small bowel evaluation and has a higher diagnostic yield if performed early. Other modalities include CT angiography, push enteroscopy and deviceassisted enteroscopy. Intraoperative enteroscopy should be reserved as a final option when other diagnostic modalities fail or are not available. The various treatment modalities for intestinal AD include pharmacological treatment, endoscopy, radiological intervention and surgery. Somatostatin analogues and thalidomide have shown promising results in some patients. Endoscopic treatment includes argon plasma coagulation, sclerotherapy Figure 1 Video capsule endoscopy images showing (A) normal duodenal mucosa, (B) proximal jejunum showing active ooze of blood, (C) distal jejunum showing altered blood and (D) terminal ileum showing blackcoloured stool staining of the mucosa. Figure 2 (A) Photograph of the resected segment of the proximal jejunum. (B) Photomicrograph showing the biopsy of the resected segment with normal mucosa, thickened walls of the submucosal blood vessels, veins exhibiting arterialisation of walls and thinwalled capillaries (H&E: 10×).