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Title of Journal: radiol med

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Abbravation: La radiologia medica

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Springer Milan

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DOI

10.1002/jez.1402570216

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1826-6983

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Diagnostic imaging in the study of visceral involv

Authors: M Memeo A Scardapane R De Blasi C Sabbà A Carella G Angelelli
Publish Date: 2008/05/13
Volume: 113, Issue: 4, Pages: 547-566
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Abstract

Hereditary haemorrhagic telangiectasia HHT also known as RenduOslerWeber disease is an autosomaldominant vascular disease characterised by mucocutaneous or visceral angiodysplastic lesions Its diagnosis is exclusively based on clinical criteria The brain lungs and liver in growing order of prevalence are the most frequently involved organs Diagnostic imaging based on ultrasound US computed tomography CT magnetic resonance imaging MRI and digital subtraction angiography DSA has a fundamental role in detecting visceral involvement in HHT patients and is therefore crucial for the prognostic assessment and therapeutic approach Arteriovenous shunts are the most common cerebrovascular malformations CVMs MRI and CT angiography are the methods of choice for diagnosing cerebral involvement and it is debated whether MRI could be considered as a screening examination on account of its noninvasiveness Pulmonary arteriovenous malformations diffuse telangiectases or highflow lowpressure shunts between pulmonary arteries and veins can be studied with contrastenhanced US but multidetector CT seems to provide the most comprehensive evaluation of their angioarchitecture whereas angiography has a predominant role in treatment Liver involvement is frequent and characterised by the presence of intrahepatic shunts disseminated intraparenchymal telangiectases and other vascular lesions US is useful for detecting hepatic lesions but should be completed by more accurate imaging methods such as multidetector CT and MRILa telangiectasia emorragica ereditaria HHT o malattia di RenduOslerWeber è un disordine vascolare ereditario caratterizzato dalla comparsa di lesioni angiodisplasiche mucocutanee e viscerali la cui diagnosi si basa esclusivamente su criteri clinici L’encefalo i polmoni ed il fegato sono in ordine crescente di prevalenza le sedi viscerali più frequentemente coinvolte La diagnostica per immagini mediante gli ultrasuoni US la tomografia computerizzata TC la risonanza magnetica RM e la angiografia DSA ha un ruolo cruciale nella diagnosi del coinvolgimento viscerale nei pazienti affetti da HHT ed è pertanto fondamentale nella valutazione prognostica e nella impostazione terapeutica Tra le malformazioni vascolari cerebrali gli shunt arterovenosi costituiscono l’alterazione più frequente La RM e la angioTC sono le metodiche di scelta nella diagnosi dell’interessamento cerebrale ed è dibattuto se per la scarsa invasività la RM sia da proporre come metodica di screening Le malformazioni arterovenose polmonari telangiectasie diffuse o fistole tra arterie e vene polmonari possono essere ricercate con US con MdC ma la TC multidetettore appare l’indagine più completa per la valutazione della loro angioarchitettura mentre l’angiografia mantiene un ruolo prevalentemente terapeutico L’interessamento epatico è frequente e caratterizzato dalla comparsa di fistole arterovenose telangiectasie intraparenchimali ed altre alterazioni vascolari Gli US sono utili nella ricerca delle lesioni epatiche ma devono essere integrati da indagini più accurate quali la TC multidetettore e la RM


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