Acute idiopathic blind spot enlargement syndrome (AIBSES) with retinal vasculitis

PURPOSE: To report the clinical and anatomical features of an unusual case of acute idiopathic blind spot enlargement syndrome (AIBSES) with retinal vasculitis. OBSERVATIONS: A 39-year-old patient, who was a known case of chronic headache with nonspecific visual symptoms for nine years, developed scotomas in her right eye. She was initially diagnosed with AIBSES which had been stable, but later worsened with symptoms of subsequent blind spot enlargement and photopsia on the left eye a year later. Increase in the size of the blind spot over the left eye and stability of the blind spot enlargement over the right eye was documented on Humphrey visual field (HVF) testing. Due to the rapid onset and severity of symptoms, an inflammatory etiology was entertained and this prompted referral to our clinic. At initial presentation, fluorescein angiogram (FA) revealed moderate diffuse vasculitis and disc leakage in the left eye, which existed alongside the enlarged blind spot. Corticosteroid treatment was suggested and initiated. The patient was found to have a reduction in the size of her blind spot and a decrease in severity of retinal vasculitis a month later. Unfortunately, the patient was then lost to follow up and had stopped steroid treatment of her own accord. After nine months without treatment, the patient's blind spot increased to a larger size than her initial presentation, as documented on HVF, with recurrence of vasculitis in the left eye. CONCLUSION AND IMPORTANCE: This is an unusual case of AIBSES which presented with vasculitis and rapid progression and has responded to steroids. Though monocular AIBSES has been shown to later affect the contralateral eye, concurrent vasculitis with AIBSES has not previously been reported. Furthermore, the response to treatment with reduction in blind spot enlargement is unusual for AIBSES. These findings stress the need for regular monitoring in cases of AIBSES.