Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.

Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and anecdotal evidence has suggested a therapeutic role for monoclonal antibodies (Rennebohm et al. 2008, Lee and Amezcua 2009) [3,4]. We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab.

Long-term outcome of gold eyelid weights in patients with facial nerve palsy.

OBJECTIVE: To assess the safety and efficacy of upper eyelid gold weight implants in managing paralytic lagophthalmos and to compare two surgical techniques for their insertion. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: One hundred four patients had a gold weight implanted for paralytic lagophthalmos between 1982 and 1996 at the Royal Prince Alfred Hospital in Sydney. Each patient had more than 2 years of follow-up of lid load function. MAIN OUTCOME MEASURES: The effectiveness of gold weights in reanimating eyelid closure, mean duration of gold weight retention, reasons for removal, and complications resulting from a gold weight; these outcomes were also compared using two surgical techniques for gold weight insertion. RESULTS: One hundred three patients maintained corneal integrity. At the time of assessment, 46 patients had had their lid loads removed from their eyelids, and 58 lid loads remained in situ. Of the lid loads that were removed, 78% were because the facial nerve had recovered. The remaining 22% were removed because of cosmetic dissatisfaction (7 patients), the lid load becoming too superficial (6 patients), migration (3 patients), partial extrusion (1 patient), and ptosis resulting from too heavy a weight (1 patient). CONCLUSION: Gold weights are well tolerated and effective in managing paralytic lagophthalmos. An open surgical technique with direct suture fixation of the gold weight to the tarsal plate produced fewer complications than inserting the lid load into a prefashioned tissue pocket in the preseptal space through a small lateral skin incision.

Susac syndrome: microangiopathy of the retina, cochlea and brain.

BACKGROUND: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. METHODS: Analysis of two male and two female cases of Susac syndrome recognized in Australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.

Long-term results using scleral-fixated posterior chamber intraocular lenses.

Transscleral ciliary sulcus fixation of a posterior chamber intraocular lens (IOL) is a technique for managing complicated cataract surgery, secondary IOL implantation, and IOL exchange. We performed a retrospective review of 32 patients who had a minimum of six months and a mean of 13.3 months follow-up after transscleral ciliary sulcus fixation of a posterior chamber IOL. In 30 patients, vision was maintained or improved following surgery. Complications were not uncommon; they were visually significant in three patients and necessitated a reoperation for late subluxation of the IOL in one other patient. Although the long-term visual results are encouraging, the postoperative complication rate of scleral-fixated posterior chamber IOLs is between that of posterior chamber IOLs inserted when the posterior capsule is intact and that of closed-loop anterior chamber IOLs.