Underuse of screening in Osler –Weber–Rendu syndrome

<span class="paragraphSection">According to the view expressed by the authors the management of Hereditary Haemorrhagic Telangiectasia (HHT) should include prevention of complications of vascular lesions.<a href="#hcw221-B1" class="reflinks"><sup>1</sup></a> Accordingly, the opportunity should have been taken to screen the patient for pulmonary arteriovenous malformations (PAVM), the latter a feature in 15 –50% of patients with HHT.<a href="#hcw221-B2" class="reflinks"><sup>2</sup></a> With transthoracic contrast echocardiography as the imaging modality, the International Guidelines recommend screening all HHT patients for PAVM (level of evidence III, strength of evidence: 96% agreement), on the premise that screening will detect PAVM before the development of life-threatening or debilitating complications.<a href="#hcw221-B2" class="reflinks"><sup>2</sup></a> The latter include stroke (in 33% of HHT patients) and cerebral abscess in 23%.<a href="#hcw221-B3" class="reflinks"><sup>3</sup></a> Embolization is the mainstream therapy for PAVM, one of its aims being the prevention of strokes and cerebral abscesses attributable to PAVM-related right to left shunting. According to the 2015 update of the Cochrane systematic review of the literature, embolotherapy appears to reduce mortality and morbidity compared with no treatment.<a href="#hcw221-B3" class="reflinks"><sup...
Source: QJM - Category: Internal Medicine Source Type: research