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Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia
  1. Maximilian Ackermann1,
  2. Steven J Mentzer2,
  3. Wilfried Roth3,
  4. Urban Geisthoff4,
  5. Erich Stoelben5
  1. 1Institute of Functional and Clinical Anatomy, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany
  2. 2Division of Thoracic Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
  3. 3Institute of Pathology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany
  4. 4Department of Otorhinolaryngology, Essen University Hospital, Essen, Germany
  5. 5Department of Thoracic Surgery, Cologne-Merheim Hospital, Kliniken der Stadt Köln gGmbH, Witten-Herdecke University Hospital, Cologne, Germany
  1. Correspondence to Dr Maximilian Ackermann, Institute of Functional and Clinical Anatomy, University Medical Center of the Johannes Gutenberg University Mainz, Johann-Joachim-Becher-Weg 13, Mainz 55128, Germany; maximilian.ackermann{at}uni-mainz.de

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A 24-year-old Caucasian man was admitted with a known hereditary haemorrhagic telangiectasia (HHT) and heterozygous mutation of factor V Leiden following episodes of cerebral infarctions in occipital lobes, cerebellum and brainstem. In his case history, the patient underwent several interventional embolisation of arteriovenous (AV) malformations in the middle and lower lobes (figure 1). However, those were not completely successful as the malformations were diffuse. We performed video-assisted thoracoscopic surgery with a resection of the middle lobe and a wedge resection of segment 10.

Figure 1

CT scans depict the pulmonary arteriovenous malformations after re-embolisation in the middle lobe.

HHT (Osler-Weber-Rendu disease) is an autosomal-dominant disease determined by multiple dilated vessels including mucocutaneous telangiectasias and vascular malformations of visceral organs resulting in recurrent epistaxis, gastrointestinal bleedings, paradoxical emboli, cerebral infarctions and abscesses.1 In about 55% of patients with HHT type 1 (HHT-1) and about 10% of patients with HHT type 2 (HHT-2), large pulmonary arteriovenous malformations (PAVMs) are …

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