Bilateral aneurysm of pulmonary arteries associated with Right Ventricular Thrombus.

Clinical History

A 14 year-old boy presented with a productive cough aggravated by two episodes of hemoptysis of average abundance without dyspnoea or chest pain.
Clinical examination found buccal aphthous.

Imaging Findings

A chest radiograph was first performed and showed bilateral central hilar opacities (Fig.1).
We performed a chest CT, which showed bilateral pulmonary-artery aneurysms with a large, crescent-shaped and non-enchancing filling defect, strongly suggestive of partial thrombosis (Fig 2).
It is associated with a right ventricular thrombus (Fig 3) and thrombophlebitis of the left lower limb extending to the external iliac vein.
Our patient had a history of recurrent oral ulcerations and initially, the hypothesis of Behçet Disease was suggested.
Since we did not observe genital or ocular lesions and the pathergy test was negative, the diagnosis of Hughes-Stovin Syndrome was made.
The patient was treated with anticoagulants, corticotherapy and cyclophosphamide with a good clinical evolution. The long-term follow-up showed a decrease in size of the hilar opacities on chest radiographs (figure4).

Discussion

Hughes-Stovin Syndrome (HSS) is a very uncommon disease (fewer than 40 cases have been reported in the literature) characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms [1, 2].
The disease bears the name of two British physicians, John Patterson Hughes and Peter George Ingle Stovin who first described it in 1959 [3].
It usually affects young males and is frequently lethal through the rupture of the pulmonary aretry aneurysm(PAA) into a bronchus leading to a massive hemoptysis [4].
Typically it has three phases: in the first phase, thrombophlebitis occurs, followed by the development of PAA and the third stage, characterized by aneurysmal rupture [5].
As pulmonary embolism rarely induces aneurysms, Hughes and Stovin supposed that congenital or degenerative changes of the bronchial arteries would engender poor nutrition of the pulmonary arteries, producing a type of embolus different from that seen in normal pulmonary arteries [3, 6].
Some authors have postulated that HSS is an incomplete form of Behçet’s disease (BD) [7]. Findings unique to BD are eye lesions, recurrent genital aphthous, skin lesions, iritis, arthralgia and a positive pathergy test [2] and this helps in distinguishing the two entities.
Histopathologically, PAA in HSS patients is characterized by vessel wall destruction and perivascular infiltration [7, 8], indistinguishable from that seen in BD patients.
Symptoms include cough, dyspnoea, hemoptysis, headache, intermittent fever, papilloedema and peripheral thrombophlebitis [9].
Chest roentgograms depict PAA as hilar enlargements or lobulated opacities [10].
Traditionally, angiography has been regarded as a gold standard for the diagnosis of pulmonary artery aneurysms [11]. However, it carries the risk of aneurysm rupture [6].
Multi-detector CT angiography (ACT) gives more precise visualization of large systemic arteries [2].
Magnetic resonance angiography is limited for finding small aneurysms [2, 10, 12].
PAA are multiple and bilateral in 50% of cases [4]. Hepatic and iliac artery aneurysms have also been described [4, 6].
The majority of patients develop peripheral venous thrombosis.
Intracardiac thromboses have also been described [3, 4, 9].
Anticoagulation should be used cautiously in case of PAA with systemic thrombosis; preferably after the immunosuppressive therapy [4].
Therapeutic options include immunosuppressive treatment, surgical resection, and embolization [4].
Colchicine may be successful in a single HSS patient [13]. Our patient had a good response to colchicine with a decrease in size of the Hilar masses.
Immunosuppressive treatment made of glucocorticoids and cyclophosphamide has been used as a first line option in the treatment of HSS, but its effectiveness is not fully concluded [14, 15].
Total pneumectomy or lobectomy is indicated in case of massive bleeding of a ruptured aneurysm. Surgery is not possible in the case of multiple bilateral aneurysms [16]. Embolization is not the best treatment (the main pathological involvement is in the surrounding structures of the aneurysm) [4].

Differential Diagnosis List

Final Diagnosis

Hughes-Stovin Syndrome

URL:	https://www.eurorad.org/case/11935
DOI:	10.1594/EURORAD/CASE.11935
ISSN:	1563-4086