scholarly journals Fulminant Progressive Atypical Acute Zonal Occult Outer Retinitis

2021 ◽  
Vol 62 (8) ◽  
pp. 1148-1154
Author(s):  
Sung Joon Kim ◽  
Hyeshin Jeon ◽  
Ik Soo Byon ◽  
Ji Eun Lee ◽  
Sung Who Park

Purpose: To report a case of unilateral progressive atypical acute zonal outer retinitis in a healthy young female.Case summary: A 32-year-old healthy woman presented with visual disturbance in the left eye for 1 week. Best corrected visual acuity was 0.4 in the left eye. No abnormal findings were detected on brain magnetic resonance imaging and a laboratory work-up. Zonal retinal opacity around the optic nerve disc was noticed in a fundus photo which was consistent with the outer retinal signal defect on optical coherence tomography and a defect in the central visual field from perimetry. Multiple evanescent white dot syndrome was suspected based on fluorescein angiography and indocyanine green angiography. Prednisolone was prescribed. Visual acuity was reduced to hand-motion after 10 days with extended zonal retinal opacity. No virus was detected by multiplex polymerase chain reaction of the aqueous humor. The lesion did not improve despite high-dose intravenous steroid and antiviral treatment.Conclusions: Visual function was lost with the progression of outer retinitis. This case differed from previous cases as the outer retinitis progressed rapidly from the posterior pole to the peripheral retina. It was named fulminant progressive atypical acute zonal outer retinitis.

2021 ◽  
Vol 62 (12) ◽  
pp. 1679-1684
Author(s):  
Jae Pyeong Jeon ◽  
Donghun Lee ◽  
Jong Won Moon

Purpose: We report a case of idiopathic chiasmal optic neuritis in a pediatric patient.Case summary: A 13-year-old boy with no history of systemic disease was referred to our ophthalmology clinic because of visual disturbance in both eyes of 5 days in duration. The best-corrected visual acuity was 0.08 in the right eye and finger counting at 30 cm in the left eye; mild blurring of the disc margins (both eyes) was evident on fundus examination, as were temporal hemianopsia in the right eye and diffuse field loss in the left eye. Brain magnetic resonance imaging revealed focal nodular enhancement in the optic chiasm. Blood and cerebral fluid analysis yielded no evidence of infection or autoimmune disease. Therefore, we diagnosed isolated idiopathic chiasmal optic neuritis and commenced a systemic steroid. After 5 days, the visual acuity began to improve, and the field defect was almost eliminated (except for a small central scotoma) at 1 month. He has remained stable to the time of writing (4 months after treatment) and his visual acuity has normalized.Conclusions: Isolated idiopathic chiasmal optic neuritis developed in a pediatric patient and the visual function improved after steroid treatment. Although there was no evidence of systemic demyelinating disease, regular observation is scheduled given the possibility of late-onset disease.


2021 ◽  
Vol 20 (3) ◽  
pp. 129-133
Author(s):  
Tae Yeon Kim ◽  
So Hee Kim ◽  
Youna Choi ◽  
Seung Kwon Choi ◽  
Jae Jung Lee ◽  
...  

Purpose: To report a case of acute stromal keratitis after vitrectomy for the treatment of myopic tractional maculopathy in a lupus patient.Case summary: A lupus patient who were treated with low dose steroid complained of decreased visual acuity due to myopic tractional maculopathy with fovea detachment. No evidence of intraocular inflammation was observed. The best-corrected visual acuity (BCVA) was 20/40. We performed the vitrectomy and removal of epiretinal membrane and internal limiting membrane. BCVA of 20/80 at day 1 decreased to hand motion at day 5. Severe corneal edema and superficial punctate keratitis that were observed did not improve three days after the anti-herpetic treatment. Based on this, we diagnosed her as immune-mediated acute stromal keratitis in lupus, and treated with oral methylprednisolone of 20 mg. The corneal edema improved rapidly following high dose systemic steroid. Myopic fovea detachment also improved. Improvement of BCVA was limited to 20/80 at six months, compared to the baseline one.Conclusions: We observed the development of acute stromal keratitis after vitrectomy for the myopic tractional maculopathy in the patient with lupus. High dose systemic steroid improved this corneal complication. Increased dose of systemic steroid should be considered before and after vitrectomy in lupus patients.


2021 ◽  
pp. 247412642110189
Author(s):  
Austen N. Knapp ◽  
Jawad I. Arshad ◽  
Daniel F. Martin ◽  
Rula Hajj-Ali ◽  
Kimberly Baynes ◽  
...  

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis–like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.


2021 ◽  
pp. 1-11
Author(s):  
Visish M. Srinivasan ◽  
Phiroz E. Tarapore ◽  
Stefan W. Koester ◽  
Joshua S. Catapano ◽  
Caleb Rutledge ◽  
...  

OBJECTIVE Rare arteriovenous malformations (AVMs) of the optic apparatus account for < 1% of all AVMs. The authors conducted a systematic review of the literature for cases of optic apparatus AVMs and present 4 cases from their institution. The literature is summarized to describe preoperative characteristics, surgical technique, and treatment outcomes for these lesions. METHODS A comprehensive search of the English-language literature was performed in accordance with established Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify all published cases of AVM in the optic apparatus in the PubMed, Web of Science, and Cochrane databases. The authors also searched their prospective institutional database of vascular malformations for such cases. Data regarding the clinical and radiological presentation, visual acuity, visual fields, extent of resection, and postoperative outcomes were gathered. RESULTS Nine patients in the literature and 4 patients in the authors’ single-surgeon series who fit the inclusion criteria were identified. The median age at presentation was 29 years (range 8–39 years). Among these patients, 11 presented with visual disturbance, 9 with headache, and 1 with multiple prior subarachnoid hemorrhages; the AVM in 1 case was found incidentally. Four patients described prior symptoms of headache or visual disturbance consistent with sentinel events. Visual acuity was decreased from baseline in 10 patients, and 11 patients had visual field defects on formal visual field testing. The most common visual field defect was temporal hemianopia, found in one or both eyes in 7 patients. The optic chiasm was affected in 10 patients, the hypothalamus in 2 patients, the optic nerve (unilaterally) in 8 patients, and the optic tract in 2 patients. Six patients underwent gross-total resection; 6 patients underwent subtotal resection; and 1 patient underwent craniotomy, but no resection was attempted. Postoperatively, 9 of the patients had improved visual function, 1 had no change, and 3 had worse visual acuity. Eight patients demonstrated improved visual fields, 1 had no change, and 4 had narrowed fields. CONCLUSIONS AVMs of the optic apparatus are rare lesions. Although they reside in a highly eloquent region, surgical outcomes are generally good; the majority of patients will see improvement in their visual function postoperatively. Microsurgical technique is critical to the successful removal of these lesions, and preservation of function sometimes requires subtotal resection of the lesion.


2015 ◽  
Vol 6 (3) ◽  
pp. 385-389 ◽  
Author(s):  
Bettina Hohberger ◽  
Michael Rudolph ◽  
Antonio Bergua

Purpose: Choroidal neovascularization (CNV) associated with punctate inner choroidopathy (PIC) is a rare clinical entity, yet still a challenge for medical treatment. A case of a young myopic woman developing CNV secondary to unilateral PIC is presented. Clinical morphology, diagnostic procedure and follow-up are reported. Case Report: A 29-year-old woman presented with multiple yellowish dots at the posterior pole. No other signs of inflammation could be seen. Angiography with fluorescein yielded hyperfluorescent signals in the affected areas with a diffuse leak, and SD-OCT showed a slightly elevated retinal pigment epithelial layer, consistent with the diagnosis of PIC. Additionally a classic CNV was observed. Results: Anti-inflammatory therapy with local prednisolone acetate eye drops in combination with intravitreal injection of anti-vascular endothelial growth factor (VEGF, bevacizumab) yielded an increased best-corrected visual acuity. As CNV reappeared, systemic medication with prednisone and azathioprine in combination with two further intravitreal injections of anti-VEGF stabilized CNV and increased visual acuity again. Conclusion: Combined therapy of immunosuppression with intravitreal anti-VEGF injections can be considered as therapeutic strategy in the management of recurrent CNV associated with PIC.


2014 ◽  
Vol 32 (9) ◽  
pp. 949-959 ◽  
Author(s):  
Deepa Bhojwani ◽  
Noah D. Sabin ◽  
Deqing Pei ◽  
Jun J. Yang ◽  
Raja B. Khan ◽  
...  

Purpose Methotrexate (MTX) can cause significant clinical neurotoxicity and asymptomatic leukoencephalopathy. We sought to identify clinical, pharmacokinetic, and genetic risk factors for these MTX-related toxicities during childhood acute lymphoblastic leukemia (ALL) therapy and provide data on safety of intrathecal and high-dose MTX rechallenge in patients with neurotoxicity. Patients and Methods Prospective brain magnetic resonance imaging was performed at four time points for 369 children with ALL treated in a contemporary study that included five courses of high-dose MTX and 13 to 25 doses of triple intrathecal therapy. Logistic regression modeling was used to evaluate clinical and pharmacokinetic factors, and a genome-wide association study (GWAS) was performed to identify germline polymorphisms for their association with neurotoxicities. Results Fourteen patients (3.8%) developed MTX-related clinical neurotoxicity. Of 13 patients rechallenged with intrathecal and/or high-dose MTX, 12 did not experience recurrence of neurotoxicity. Leukoencephalopathy was found in 73 (20.6%) of 355 asymptomatic patients and in all symptomatic patients and persisted in 74% of asymptomatic and 58% of symptomatic patients at the end of therapy. A high 42-hour plasma MTX to leucovorin ratio (measure of MTX exposure) was associated with increased risk of leukoencephalopathy in multivariable analysis (P = .038). GWAS revealed polymorphisms in genes enriched for neurodevelopmental pathways with plausible mechanistic roles in neurotoxicity. Conclusion MTX-related clinical neurotoxicity is transient, and most patients can receive subsequent MTX without recurrence of acute or subacute symptoms. All symptomatic patients and one in five asymptomatic patients develop leukoencephalopathy that can persist until the end of therapy. Polymorphisms in genes related to neurogenesis may contribute to susceptibility to MTX-related neurotoxicity.


Author(s):  
Daniel A. Brinton ◽  
Charles P. Wilkinson

The differential diagnosis of rhegmatogenous retinal detachment includes secondary (nonrhegmatogenous) retinal detachment and other entities that may simulate a retinal detachment. Nonrhegmatogenous detachments are categorized as exudative (serous) and tractional detachments. Conditions that may be mistaken for retinal detachment include retinoschisis, choroidal detachment or tumors, and vitreous membranes. Sometimes benign findings in the peripheral retina are mistaken for retinal breaks. The most prominent feature of the fundus is the optic nerve head or disc, the only place in the human body that affords a direct view of a tract of the central nervous system. The foveola, the functional center of the fundus, is located in the center of the fovea, which has a diameter of about 5°. The macula is centered on the fovea and has a diameter of about 17°. The multiple branches of the central retinal artery are readily identifi ed by their bright red color and relatively narrow caliber. The multiple tributaries of the central retinal vein are recognized by their dark red color and relatively wider caliber. In a darkly pigmented fundus, the choroidal vessels in the posterior pole can be obscured from view, but in an eye with minimal pigment, they are readily visible. The venous tributaries of the choroid that make up the vortex veins are usually easily seen. The most prominent features of the choroidal venous system are the vortex ampullae, of which there are usually four (but sometimes more). They are located approximately in the 1-, 5-, 7-, and 11-o’clock meridians, just posterior to the equator. The horizontal meridians are usually identifiable by their radially oriented, long posterior ciliary nerves, and infrequently the long posterior ciliary artery can be seen adjacent to the nerve. The nerve is relatively broad and has a yellow color, and the artery is identifiable by its red color. The artery is usually inferior to the nerve temporally, and superior to it nasally (Figure 5–1).


2020 ◽  
pp. 112067212090466
Author(s):  
Ahmad Al-Moujahed ◽  
Natalia F Callaway ◽  
Daniel Vail ◽  
Cassie A Ludwig ◽  
Marco H Ji ◽  
...  

Background: Optic disc pits frequently lead to visual deterioration due to macular retinoschisis or serous retinal detachment. Here, we report a case of optic disc pit–associated macular retinoschisis due to intraretinal fluid accumulation that resolved with improvement in visual acuity after treatment with topical dorzolamide. Case description: A 56-year-old otherwise healthy female with no ocular history presented with 2 weeks of slowly worsening blurry vision in her right eye. Visual acuity was 20/30 in the right eye. Posterior segment examination revealed posterior vitreous detachment, an optic disc pit at 9 o’clock, macular edema and foveoschisis with fluid extending from the optic nerve, and a normal peripheral retina. Optical coherence tomography imaging of the macula showed central subfield thickness of 526 µm. The patient preferred no surgical intervention, so topical dorzolamide 2% three times daily was initiated. Over the next 2 years, the central subfield thickness steadily declined from 526 to 262 µm, and her vision improved to 20/20 with improvement in the macular retinoschisis. Conclusion: Our report presents a case of resolution of optic disc pit–associated macular retinoschisis due to intraretinal fluid accumulation with possible role for dorzolamide as a potential treatment option.


1985 ◽  
Vol 3 (12) ◽  
pp. 1683-1689 ◽  
Author(s):  
G Wilding ◽  
R Caruso ◽  
T S Lawrence ◽  
Y Ostchega ◽  
E J Ballintine ◽  
...  

Because of increasing complaints of visual dysfunction, 13 patients with refractory or recently diagnosed ovarian carcinoma were evaluated for possible cisplatin-induced ophthalmologic toxicity. All patients had received high-dose cisplatin (200 mg/m2 in five divided daily doses) over two to four cycles. Eight patients (62%) developed symptoms of blurred vision and three (23%) also developed altered color perception. Retinal toxicity in the form of cone dysfunction was documented by electroretinography and color vision testing in 11 patients. Three patients were studied prospectively. Two patients who developed cone dysfunction had normal ophthalmologic exams before the initiation of chemotherapy or after one cycle of cisplatin, suggesting a causal relationship between cisplatin therapy and subsequent retinal abnormalities. Though visual acuity improved off therapy, color vision abnormalities persisted as long as 16 months beyond therapy.


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