Chest imaging
Case TypeClinical Cases
AuthorsSonia Casella, Andrea Molinari, Federico Pistoia, Sara Sanguinetti, Cristina Gauglio
Patient56 years, male
A 56-year-old female patient with past history of hypertension presented to emergency following a minor trauma with right-sided chest pain at the midclavicular line. On physical examination she was afebrile, eupnoic (12 breaths/minute) with bilateral vesicular murmur and saturation rate of 97%.
She underwent chest X-ray that showed no traumatic bone lesions; however, a single rounded nodule at the apex of the upper right lobe was seen. CT was then performed. Two additional nodules were seen: one in the right middle lobe and one in the left lingular paracardiac region (maximum diameter 36x25 mm). After contrast injection there was rapid opacification and wash out of all three lesions, simultaneously with the main pulmonary artery and right ventricle. All had irregular shape and sharp edges and each of them was fed by one artery and drained by one vein.
Pulmonary arteriovenous malformations (PAVMs), also known as arteriovenous fistulae, are abnormal connections between the pulmonary arterial and venous circulation.
Their prevalence is 38/100000 individuals with a predilection for females. Up to 70% of cases of PAVMs are associated with Rendu-Osler-Weber disease (hereditary haemorragic telangiectasia-HHT), but in some patients they are incidental findings; they can be multiple in 35% and bilateral in 10% of cases [1, 2].
Symptoms relate to PAVM’s size: lesions with diameter > 2 cm are more likely symptomatic. The most common symptoms are cyanosis, dyspnoea and haemoptysis, and the major complications are rupture and paradoxical embolisation. Dyspnoea and cyanosis are the expression of a right to left shunt between pulmonary veins and arteries that causes left-heart overload. Pregnant women with PAVMs are at increased risk of haemorrhage [3].
PAVMs can be simple, with just one feeding artery and one draining vein, or complex (less common), which have more than one feeding artery.
Diagnosis is performed by contrast-enhanced CT, and treatment is based on embolisation [4].
Our patient had no cardiac symptoms and didn’t show any clinical signs of Rendu-Osler-Weber disease (according to Curacao criteria: recurrent epistaxis; teleangiectasias at typical sites: oral cavity, nose, fingers; visceral lesions: GI, CNS and lung; and family history of a HHT-affected first-degree relative. Diagnosis is based on presence of at least 3 of these 4 criteria) [5].
In order to evaluate the risk of future complications, the patient was addressed to the Pulmonology department and she is currently followed-up.
[1] Shovlin, Jackson J (2016) Pulmonary Vascolar Abnormalities in Murray & Nadel’s Textbook of Respiratory Medicine. 1081-1085
[2] Webb R, Higgins C (2016) Congenital lung abnormalities and vascular malformations in: Thoracic Imaging. Pulmonary and Cardiotvascular Radiology. 283-288
[3] Di Crescenzo V, Napolitano F, Vatrella et al. (2015) Pulmonary arterovenous malformation causing hemothorax in a pregnant woman without Osler- Weber-Rendu syndrome. Open Med 10:549-554 (PMID: 28352753)
[4] Grosso M, Groppo Marchisio F, Testa F et al. (2008) Malformazioni artero-venose polmonari: Trattamento percutaneo con conservazione del parenchima nelle fistole ad alto flusso. Radiol Medica 113:395-413
[5] Sadick H, Sadick M, Götte K (2006) Hereditary hemorrhagic telangiectasia: an update on clinical manifestations and diagnostic measures. Wien Klin Wochenschr 118:72-80 (PMID: 16703249)
URL: | https://www.eurorad.org/case/15697 |
DOI: | 10.1594/EURORAD/CASE.15697 |
ISSN: | 1563-4086 |
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