Unusual Presentation of Presumed Arteritic Ischemic Optic Neuropathy in Waxy Pale Disc of Retinitis Pigmentosa.

This case report describes an unusual presentation of arteritic anterior ischemic optic neuropathy (AAION) in a 68-year-old patient with retinitis pigmentosa (RP) secondary to Usher syndrome. The authors report a patient with RP who presented with rapid unilateral vision loss. A diagnosis of AAION was made by fluorescein angiography and temporal artery biopsy despite the lack of typical optic nerve features of anterior ischemic neuropathy, which were likely masked due to the waxy pale disc associated with RP. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:350-352.].

Combined bilateral ophthalmic artery occlusion & central retinal vein occlusion from presumed giant cell arteritis.

PURPOSE: To report on a severe case of presumed giant cell arteritis (GCA) presenting with partial and complete ophthalmic artery occlusion along with bilateral central retinal vein occlusions (CRVO). OBSERVATIONS: A 73-year-old female presented with bilateral complete vision loss of sudden onset. The patient also experienced a mild frontal headache prior to onset of vision loss. Fundus examination revealed bilateral central retinal artery occlusion (CRAO) and CRVO. Subsequent fluorescein angiography indicated partial right ophthalmic artery occlusion and complete left ophthalmic artery occlusion. Acute phase reactants were elevated. The patient was clinically diagnosed with GCA and intravenous (IV) steroids were initiated. Four days later, a temporal artery biopsy (TAB) was performed and resulted as negative for granulomatous inflammation. The patient did not regain vision and remained with no light perception (NLP) in both eyes. CONCLUSIONS: and Importance: This case highlights the discrepancy between clinical diagnosis and pathologic tissue diagnosis in a patient that presented with such extensive ocular vasculitic disease. Such extensive bilateral disease has not been reported. In addition, there are few studies regarding the effect of pulse-dosed IV steroids on TAB results. This case report suggests that the gradual histologic changes that occur over one or two weeks while on oral steroids may occur over three to four days while on high dose IV steroids, necessitating early biopsy.

Serpin treatment suppresses inflammatory vascular lesions in temporal artery implants (TAI) from patients with giant cell arteritis.

Giant cell arteritis (GCA) and Takayasu's disease are inflammatory vasculitic syndromes (IVS) causing sudden blindness and widespread arterial obstruction and aneurysm formation. Glucocorticoids and aspirin are mainstays of treatment, predominantly targeting T cells. Serp-1, a Myxomavirus-derived serpin, blocks macrophage and T cells in a wide range of animal models. Serp-1 also reduced markers of myocardial injury in a Phase IIa clinical trial for unstable coronary disease. In recent work, we detected improved survival and decreased arterial inflammation in a mouse Herpesvirus model of IVS. Here we examine Serp-1 treatment of human temporal artery (TA) biopsies from patients with suspected TA GCA arteritis after implant (TAI) into the aorta of immunodeficient SCID (severe combined immunodeficiency) mice. TAI positive for arteritis (GCApos) had significantly increased inflammation and plaque when compared to negative TAI (GCAneg). Serp-1 significantly reduced intimal inflammation and CD11b+ cell infiltrates in TAI, with reduced splenocyte Th1, Th17, and Treg. Splenocytes from mice with GCApos grafts had increased gene expression for interleukin-1 beta (IL-1ß), IL-17, and CD25 and decreased Factor II. Serp-1 decreased IL-1ß expression. In conclusion, GCApos TAI xenografts in mice provide a viable disease model and have increased intimal inflammation as expected and Serp-1 significantly reduces vascular inflammatory lesions with reduced IL-1ß.

Return of visual function after bilateral visual loss following flow diversion embolization of a giant ophthalmic aneurysm due to both reduction in mass effect and reduction in aneurysm pulsation.

Large and giant paraclinoid aneurysms are challenging to treat by either surgical or endovascular means. Visual dysfunction secondary to optic nerve compression and its relationship with aneurysm size, pulsation and thrombosis is poorly understood. We present a patient with a giant paraclinoid aneurysm resulting in bilateral visual loss that worsened following placement of a Pipeline Embolization Device and adjunctive coiling. Visual worsening occurred in conjunction with aneurysm thrombosis, increase in maximal aneurysm diameter and new adjacent edema. Her visual function spontaneously improved in a delayed fashion to better than pre-procedure, in conjunction with reduced aneurysmal mass effect, size and pulsation artifact on MRI. This report documents detailed ophthalmologic and MRI evidence for the role of thrombosis, aneurysm mass effect and aneurysm pulsation as causative etiologies for both cranial nerve dysfunction and delayed resolution following flow diversion treatment of large cerebral aneurysms.

Return of visual function after bilateral visual loss following flow diversion embolization of a giant ophthalmic aneurysm due to both reduction in mass effect and reduction in aneurysm pulsation.

Large and giant paraclinoid aneurysms are challenging to treat by either surgical or endovascular means. Visual dysfunction secondary to optic nerve compression and its relationship with aneurysm size, pulsation and thrombosis is poorly understood. We present a patient with a giant paraclinoid aneurysm resulting in bilateral visual loss that worsened following placement of a Pipeline Embolization Device and adjunctive coiling. Visual worsening occurred in conjunction with aneurysm thrombosis, increase in maximal aneurysm diameter and new adjacent edema. Her visual function spontaneously improved in a delayed fashion to better than pre-procedure, in conjunction with reduced aneurysmal mass effect, size and pulsation artifact on MRI. This report documents detailed ophthalmologic and MRI evidence for the role of thrombosis, aneurysm mass effect and aneurysm pulsation as causative etiologies for both cranial nerve dysfunction and delayed resolution following flow diversion treatment of large cerebral aneurysms.

Fulminant chorioretinitis and papillitis secondary to coxsackievirus B presenting as acute posterior multifocal placoid pigment epitheliopathy with positive response to intravenous immunoglobulin.

PURPOSE: To describe a case of fulminant bilateral papillitis and chorioretinitis in the setting of positive coxsackievirus B titers, which convalesced months after the patient's presentation. The patient presented with an acute posterior multifocal placoid pigment epitheliopathy-like clinical picture and her visual acuity and visual field improved moderately after initiation of treatment with intravenous immunoglobulin. METHODS: Case report of a white female with bilateral blurred vision is presented in this study. RESULTS: A previously healthy 59-year-old white woman presented with a 3-day history of bilateral blurred vision. Initial visual acuity was hand motion in the right eye and count fingers in the left eye. Extensive workup and imaging was done and she was empirically started on intravenous antibiotics followed 24 hours later by intravenous steroids. The patient was found to have high titers of coxsackievirus B and sustained modest visual acuity and perimetry improvement in one eye after intravenous immunoglobulin infusion. CONCLUSION: A case of fulminant bilateral chorioretinitis and papillitis that resulted in bilateral vision loss despite high-dose steroids within 24 hours of initial loss in vision is presented in this study. The patient was found to have high titers of coxsackievirus B3, B4,B5 after presentation and her condition improved after treatment with intravenous immunoglobulin, suggesting a plausible immune component.

Orbital aspergillosis: voriconazole - the new standard treatment?

BACKGROUND/AIM: To describe a case of invasive orbital aspergillosis and evaluate treatments and outcomes. METHODS: A case report and review of orbital aspergillosis treatment with voriconazole in the English language literature. CONCLUSION: Amphotericin B with debridement is the current standard of care for orbital aspergillosis; however, its prognosis is unfavorable. When compared to amphotericin B, voriconazole demonstrates a survival benefit, has less systemic toxicity, and is better tolerated by patients. While a prospective trial comparing amphotericin B to voriconazole in orbital aspergillosis is not feasible, there is evidence to support the use of voriconazole as primary therapy.

Fatal posterior ischemic optic neuropathy.

OBJECTIVE: We would like to describe a case report of posterior ischemic optic neuropathy (PION) misdiagnosed as arteritic PION with presumed GCA which leads to missing the correct diagnosis with fatal outcome. METHODS: The clinical course of a single patient will be described including presentation, testing,diagnosis and treatment. RESULTS: An 82-year-old male patient presented with headache and temporal tenderness and mildly elevated ESR and was treated with steroids for presumed diagnosis of GCA. He developed progressive visual loss to NLP in the left eye while on steroids. MRI Brain and orbit showed enhanced mass at orbital apex misdiagnosed as incidental meningioma. Endoscopic biopsy was done and the pathology exam showed aspergillosis. The patient later died after he developed aspergillosis fungal meningitis with subsequent multiple brain infarcts despite appropriate antifungal treatment. CONCLUSION: Over-diagnosis of Giant cell arteritis and unwise use of steroids in elderly without appropriate work-ups can lead to unfavorable outcomes including a delay in diagnosis and an exacerbation of occult fungal infection that would manifest weeks later with a fatal outcome.