Orbital Bruit in Churg–Strauss Orbitopathy; A Novel Sign

CASE REPORT

A 60-year-old male presented with pain in the left eye, diplopia and unilateral non-pulsatile axial proptosis (Figure 1). His left best corrected visual acuity (BCVA) was 6/24 with a left relative afferent pupillary defect. There was periorbital soft tissue swelling and exophthalmometry revealed 4 mm of left proptosis. Ocular motility in the left eye was globally limited. There were prominent episcleral blood vessels. On orbital auscultation there was a deep, rumbling, continuous and non-pulsatile bruit. Prior medical history included adult-onset asthma. The clinical appearance suggested a carotid cavernous sinus fistula (CCF). He underwent enhanced magnetic resonance imaging, and subsequently computed tomography (CT) and CT angiography. Imaging failed to show signs consistent with CCF, but confirmed nasal and sinus mucosal thickening (Figure 2). There was progression to bilateral orbital involvement within a couple of weeks, and he developed conjunctival nodules, which were biopsied.

FIGURE 1  Patient with CSS, with bilateral asymmetric periocular edema and proptosis (top left). Subconjunctival nodules on the superior bulbar conjunctival surface (bottom left). Reduced periorbital soft tissue swelling and proptosis following treatment with systemic corticosteroids (top right). Histology of biopsied conjunctival nodules shows the presence of a prominent number of eosinophils with necrotising eosinophilic granulomata, in keeping with CSS (bottom right).

FIGURE 2  Coronal MRI showing mucosal thickening within the paranasal sinuses (left). Axial MRI showing bilateral orbital proptosis, more severe on the left (centre). CTA showing normal cavernous sinuses in the arterial phase (right).

There was a peripheral eosinophilia of 3.04 × 10⁹/L, which in conjunction with asthma and sinusitis, raised the possibility of Churg–Strauss syndrome (CSS). Biopsy of the subconjunctival nodules confirmed prominent eosinophils, with necrotising granulomatous inflammation, in keeping with CSS (Figure 1). The orbitopathy responded rapidly to oral prednisolone, his vision returning to BCVA 6/6, and the orbital bruit resolved.

COMMENT

Orbital involvement in CSS is rare, and this is only the third presentation of diffuse bilateral orbital inflammation.^1–4 Thyroid orbitopathy has been reported to have a pulsatile bruit, due to being a hyperdynamic state.⁵ Arteriovenous fistulas, CCF, intracranial haemangiomas and severe anaemia are causes of continuous orbital bruits that were excluded. The orbital bruit is a novel sign in CSS orbitopathy, which may be the result of turbulent flow within arterial blood vessels narrowed by vasculitis. This is the first time orbital bruit has been reported in CSS associated orbitopathy.

ACKNOWLEDGEMENTS

This case has been presented as a poster at BOPSS Annual Meeting, Edinburgh 17–18 June 2010 and ESOPRS Annual Meeting, Munich, 9–11 September 2010.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.