RESUMO
Osler-Weber-Rendu disease (OWRD), also known as hereditary haemorrhagic telangiectasia (HHT), is an autosomal dominant genetic disorder characterised by arteriovenous malformations in several organs. Ischemic cholangitis is a rare life-threatening complication of OWRD, with only a few documented cases in the literature. A liver transplant is the main curative treatment. In this paper, we report a case of a 33-year-old woman with a history of recurrent epistaxis, admitted with abdominal pain and fever, physical examination found multiple cutaneous and mucosal telangiectasias and the biological workup showed cholestasis, abdominal imaging identified arterio-venous shunts and multiple cystic hepatic lesions, one of them seemed to communicate with an intrahepatic biliary duct, finally the diagnosis of ischemic cholangitis due to OWRD was retained and antibiotic treatment has been initiated. We review the various therapeutic options available to improve the management of this fatal complication.
RESUMO
OBJECTIVE: Dieulafoy's lesions (DLs) are a rare but potentially life-threatening source of gastrointestinal (GI) haemorrhage. They are responsible for roughly 1%-6.5% of all cases of acute non-variceal GI bleeding.Here, we describe retrospectively the clinical and endoscopic features, review the short-term and long-term outcomes of endoscopic management of bleeding DLs and we identify rate and risk factors, of recurrence and mortality in our endoscopic unit. DESIGN: Data were collected from patients presenting with GI haemorrhagic secondary to DLs between January 2018 and August 2023. Patients' medical records as well as endoscopic databases were retrospectively reviewed. Demographic data, risk factors, bleeding site, outcomes of endoscopy techniques, recurrence and mortality rate were taken into account. RESULTS: Among 1170 cases of GI bleeding, we identified only seven cases involving DLs. Median age was 74 years, with a male-to-female ratio of 2.5. 75% of patients had significant comorbidities, mainly cardiovascular diseases. Only anticoagulant and antiplatelet agents were significantly associated with DLs. All patients were presented with GI bleeding as their initial symptom. The initial endoscopy led to a diagnosis in 85% of the cases. Initial haemostasis was obtained in all patients treated endoscopically. Nevertheless, the study revealed early recurrence in two out of three patients treated solely with epinephrine injection or argon plasma coagulation. In contrast, one of three patients who received combined therapy, experienced late recurrence (average follow-up of 1 year). Pathological diagnosis was necessary in one case. One patient (14%) died of haemorrhagic shock. Average length of hospital stay was 3 days. CONCLUSION: Although rare, DLs may be responsible for active, recurrent and unexplained GI bleeding. Thanks to the emergence of endoscopic therapies, the recurrence rate has decreased and the prognosis has highly improved. Therefore, the endoscopic approach remains the first choice to manage bleeding DLs.
