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Persistent fever with chills and an endocardial mass in a child: an unusual presentation of Hughes–Stovin syndrome

Published online by Cambridge University Press:  11 November 2016

Marianna Fabi ,
Francesca Lami  and
Maurizio Zompatori
Marianna Fabi*
Affiliation:
Pediatric Cardiology and Adult Congenital Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Francesca Lami
Affiliation:
Pediatric Cardiology and Adult Congenital Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Maurizio Zompatori
Affiliation:
Radiology Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
*
Correspondence to: M. Fabi, MD, Pediatric Cardiology and Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy. Tel: +39 333 835 1572; Fax: +39 051 636 3116; E-mail: [email protected]
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Abstract

A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierre’s syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes–Stovin syndrome was diagnosed. Hughes–Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case. The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.

Keywords

Hughes–Stovin syndromepulmonary aneurysmschildhoodendocardial vegetationspersistent fever
Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 3 , March 2017 , pp. 605 - 608
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Copyright
© Cambridge University Press 2016 

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