Sir,

Visual loss in Adamantiades-Behçet disease (ABD) usually results from chronic relapsing panuveitis. We report that ABD can be acutely blinding.

Case report

A 43-year-old male immigrant from Kosovo presented with acute, painless visual loss in the right eye, perceived on awakening. BCVA was hand movements in the right eye and 20/20 in the left eye, associated with a right afferent pupillary defect. Intraocular pressure was 10 mm Hg in both eyes. Few cells were found in the anterior chamber of both eyes and the vitreous of the right eye. Ophthalmoscopy showed a right retinal oedema with relative cilioretinal sparing, mild papillary hyperaemia and venous congestion. Multiple dot–blot haemorrhages were not present initially but developed within a few days (Figure 1). No clinical signs of previous intraocular inflammation could be seen in either eye.

Figure 1
figure 1

Fundus photography and fluorescein angiography obtained 3 days after presentation. Note grossly impaired perfusion, retinal whitening and relative cilioretinal sparing.

Full size image

The medical history of this patient revealed few episodic, ill-defined external eye inflammations, recurrent arthralgias, genital and oral ulcerations and diarrhoea. Echocardiography and carotid ultrasound showed no evidence of a source of embolism. Neither systemic hypertension nor diabetes was present.

A clinical diagnosis of ABD was established. Treatment with systemic corticosteroids (prednisolone 100 mg/d) was started and later replaced by interferon-α2a (3 × 9 MioIE/week). Optic atrophy developed within several weeks and final VA was 20/400 but no further ocular inflammatory episode was noted during a 3-year follow-up.

Comment

In our patient, acute CRAO was the first ocular event that led to clinical evaluation and diagnosis of ABD. Venous congestion and increasing retinal haemorrhages were indicative of co-existing obstruction of the central retinal vein and speak against an embolic event. In ABD, occlusive vasculitis and perivasculitis have been described histopathologically. We postulate that a focal arteritis in the anterior optic nerve and an involvement of the central vein through direct inflammation or secondary compression has occurred in this case.

To our knowledge this is the first report of CRAO without additional signs of previous intraocular inflammation in ocular ABD, although involvement of the optic nerve head in ABD is well described.1 CRAO has been reported only once as a complication of advanced ocular ABD.2

Our case highlights that ABD contributes to the differential diagnosis of acute CRAO in a young patient.