Rare, Life Threatening, Treatment Dilemma: A Case Report of Hughes Stovin Syndrome

Case Report

Austin J Clin Case Rep. 2020; 7(1): 1165.

Rare, Life Threatening, Treatment Dilemma: A Case Report of Hughes Stovin Syndrome

N Assoufi*, A Maaroufi, N Elomri, J Smaali, A Charef, F Mekouar, M Jira and J Fatihi

Department of Internal Medicine, Mohamed V Military Hospital, Faculty of Medical Sciences, University Mohammed V Rabat, Morocco

*Corresponding author: Naoufal Assoufi, Department of Internal Medicine, Mohamed V Military Hospital, Faculty of Medical Sciences, University Mohammed V Rabat, Morocco

Received: June 06, 2020; Accepted: August 03, 2020; Published: August 10, 2020

Abstract

Introduction: Hughes-Stovin Syndrome (HSS) is a rare clinical entity, which is characterized by peripheral venous thrombosis and multiple pulmonary artery aneurysms with potentially life-threatening complications.

Materials and Methods: We report a case of a 35 years old Moroccan military male with history of pulmonary tuberculosis treated 02 years ago and bipolar ulcer in his oral cavity and genital region that was presented to our service for mild abundance hemoptysis. Chest X ray showed opacity in the right lung field. Computed Tomography (CT) scan revealed bilateral multiple pulmonary artery aneurysms. Cardiac MRI was performed and showed the presence of a thrombosis of the right ventricle.

Hughes-Stovin syndrome was diagnosed, and high-dose of steroids (methyl prednisolone) and immunosuppressant (cyclophosphamide) were administered. The hemoptysis was controlled with stabilization of the pulmonary aneurysms.

Conclusion: Our case shows a particular localization of thrombosis and the problem of treatment which keep to be a very difficult and hard decision especially when there is both a specific deep thrombosis and high risk pulmonary aneurysm.

Keywords: Hughes Stovin Syndrome; Ventricular thrombosis; Pulmonary aneurysm

Abbreviations

HSS: Hughes Stovin Syndrome; CT: Computed Tomography; MRI: Magnetic Resonance Imaging

Background

Hughes and Stovin had reported in 1959 four cases of deep vein thrombosis and multiple pulmonary artery aneurysms [1]. Since then this association caries the name of HSS (Hughes stovin syndrome). It is a rare and life threatening clinical situation which affects almost men with only two women described in the literature [2].

The main cause of mortality in HSS is related the rupture of the pulmonary artery aneurysm which leads to a fatal hemoptysis [3-5]. This unpleasing and complicated situation for the patricians makes the use of anticoagulant a very hard decision [1,6].

Case Report

A young Moroccan military male of 35 years old with medical history of pulmonary tuberculosis treated 03 years ago has been admitted to our department for an isolated mild hemoptysis. He had a one year history of recurrent oral and genital ulceration. His physical examination was within normal limits). Contrast-enhanced CT scans of his chest demonstrated a bilateral pulmonary artery aneurysm (Figure 1). To asses extension of the disease a transthoracic echocardiography showed a mobile right ventricular mass (Figure 2). A cardiac MRI was subsequently done, which showed a 3.8 cm 1.6 cm right ventricular mass attached to his inter-ventricular septum (Figure 3). An ophthalmologic examination of our patient showed no evidence of iritis or retinal vasculitis.

He was thus diagnosed with Hughes-Stovin syndrome, which is a variant of Behcet's disease.

A multidisciplinary discussion between the internists, thoracic surgeons and cardiovascular surgeons finally decided that anticoagulation therapy as well as any surgical or interventional therapy must be primarily avoided to initially. The patient underwent combined pulse therapy with methylprednisolone (1 g for three days) and cyclophosphamide (1 g per monthly) with 1 mg once a day of colchicine for initial management and stabilization of the aneurysms. No more hemoptysis has been recurred and the patient was discharged to be seen one month later for another pulse of cyclophosphamide.