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Rupture of a pulmonary arteriovenous malformation: a diagnosis not to be missed
  1. Mostafa El Hajjam1,2,
  2. Alexis Lacout3,
  3. Pierre Yves Marcy4,
  4. Sephen Binsse1,2,
  5. Pascal Lacombe1,2
  1. 1 Pluridisciplinary HHT team, Ambroise Paré Hospital, Boulogne Billancourt, France
  2. 2 Groupement des Hôpitaux Ile-de-France Ouest, Assistance Publique—Hôpitaux de Paris, Université de Versailles Saint Quentin en Yvelines, Boulogne Billancourt, France
  3. 3 Centre d'Imagerie Médicale, Aurillac, France
  4. 4 Polyclinique Les Fleurs, Service d'Imagerie Médicale, Ollioules, France
  1. Correspondence to Dr Alexis Lacout, Centre d'imagerie Médicale, 47, Boulevard du Pont Rouge, Aurillac 15000, France; lacout.alexis{at}wanadoo.fr

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Introduction

Also known as Rendu-Osler-Weber syndrome, hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder that leads to abnormal blood vessel formation in the skin, mucous membranes and often in organs such as lungs, liver and brain.1 At clinical inspection, patients frequently present with skin, lip and/or tongue telangiectasias. Common clinical symptoms include frequent epistaxis and less commonly acute and chronic digestive tract bleeding.1 About 15%–35% of patients with HHT will present with pulmonary arteriovenous malformations (PAVMs).2 As the spontaneous complication rate can reach up to 50%, PAVMs patients require screening on CT scan examination.

PAVMs complications include spontaneous rupture and subsequent life-threatening haemoptysis/haemothorax, a right-to-left shunting leading to paradoxical embolism causing stroke or cerebral abscess or pulmonary hypertension.2 ,3 Treatment is based on antibiotic prophylaxy and percutaneous transcatheter embolisation of the diagnosed PAVM that are screened on imaging means.2 ,3

Case description

A 57-year-old female patient was referred to our institution soon after having an air …

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