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RESEARCH PRODUCT
Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia
Erich StoelbenWilfried RothUrban W. GeisthoffMaximilian AckermannSteven J. Mentzersubject
Pulmonary and Respiratory MedicineMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyMucocutaneous zoneMedizinArticleResection03 medical and health sciencesYoung Adult0302 clinical medicineMicroscopy Electron Transmissionhemic and lymphatic diseasesotorhinolaryngologic diseasesmedicineFactor V LeidenHumans030223 otorhinolaryngologyLungHeterozygous mutationHereditary haemorrhagic telangiectasiabusiness.industryMiddle LobeMicrovascular architecturemedicine.diseaseSurgery030228 respiratory systemMicrovesselsTelangiectasia Hereditary HemorrhagicRadiologybusinessTomography X-Ray ComputedWedge resection (lung)description
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 …
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2017-01-02 |