Multimodal Imaging in Idiopathic Neuroretinitis with Localized Choroidal Insufficiency: A Case Report

Neuroretinitis is a rare clinical entity, characterized by optic nerve edema and star-shape hard exudate around fovea. The clinical features include acute unilateral visual loss, dyschromatopsia, relative afferent pupillary defect and visual field abnormalities. Increased vascular permeability of the optic disc is the main pathophysiology. As it is a not fully known clinical entity, diagnosis is challenging. In this case, we use multimodal imaging to reveal pathophysiology and anatomical change of early mild neuroretinitis. Case presentation: A 28-year-old healthy woman presented to the clinic with mild blurred vision in her left eye. After complete ophthalmic examination, outer retinal thickening of the temporal peripapillary area and optic disc edema were observed. Two days after diagnosis, the retinal edema and visual symptoms were aggravated. A hard exudate, maybe a part of macular star, was observed. Multimodal imaging including optical coherence tomography (OCT), swept-source OCT angiography (SS-OCTA), fluorescein angiography, and indocyanine green angiography visualized choroidal thinning and insufficient circulation beneath the outer retinal edema. Following steroid pulse therapy, the retinal edema and blurred vision were completely resolved. Conclusions: Multimodal imaging suggested that unilateral optic disc edema and early macular star help the diagnosis of neuroretinitis. In SS-OCTA, we found focal choroidal insufficiency. The focal insufficient choroidal circulation might be a contribution factor for idiopathic neuroretinitis. Multimodal imaging including SS-OCTA may be a valuable tool for detecting and monitoring disease progression.

REDUKSI ABLASIO MAKULA SEROSA PADA NEURORETINITIS DENGAN PEMBERIAN ASETAZOLAMID

Neuroretinitis merupakan suatu sindrom klinis ditandai dengan penurunan visus unilateral akut dengan makulopati eksudatif yang terdiri dari eksudat hard tersusun dalam gambaran bintang di sekitar fovea. Pada artikel ini, penulis melaporkan suatu kasus neuroretinitis dengan ablasio makula serosa yang memperlihatkan outcome yang baik setelah pemberian terapi asetazolamid. Seorang pasien perempuan, umur 18 tahun, datang dengan keluhan utama berupa penglihatan mata kiri kabur. Visus mata kiri saat presentasi awal yaitu 1/300 dan pada funduskopi hanya didapatkan edema papil nervus optik disertai perdarahan peripapil. Tidak didapatkan perbaikan gejala klinis setelah pemberian metilprednisolon intravena selama 3 hari. Pada follow up hari keempat, muncul gambaran macular star yang khas untuk neuroretinitis, dan pada pemeriksaan optical coherence tomography (OCT) didapatkan cairan subretina pada makula sehingga diberikan tambahan terapi asetazolamid. Setelah pemberian asetazolamid selama 2 minggu didapatkan perbaikan visus menjadi 5/60 dengan visus akhir pada follow up bulan ketiga menjadi 6/12. Meskipun neuroretinitis bersifat self-limited tetapi pada kasus ini pemberian asetazolamid mempercepat perbaikan visus, dan reduksi ablasio makula serosa.

Atypical unilateral exudative retinal detachment in a child affected by tuberous sclerosis

Introduction: Tuberous sclerosis complex (TSC) is a rare hereditary phakomatosis. The clinical features can include benign growths in the central nervous system, tumors of various visceral organs, and retinal or optic disc astrocytic hamartomas in the nerve fiber layer. Here we present the case of a child with known TSC developing Coats-like manifestations. Case description: A 22-month-old girl with known TSC and retinal hamartoma followed since birth presented for the development of exotropia and leukocoria in the left eye. Fundus examination of the left eye showed blurred optic disc, macular star, and yellow retinal exudation in the temporal area. In addition, the left eye showed marked retinal vascular tortuosity and telangiectasias. The patient underwent brain and orbit magnetic resonance imaging, revealing heterotopic gray matter nodulations along ependyma of both lateral ventricles, with partial calcification, and a posterior flattening of the left eye. Conclusion: This report shows a rare case of Coats-like disease in a child with tuberous sclerosis. In case of presence of Coats’ manifestations associated with atypical retinal or systemic findings, genetic diseases should be considered in the differential diagnosis.

Bartonella hanselae retinitis patient evaluated with multimodal retinal exams

Background Cat scratch disease is a systemic infectious illness caused by the bacterium Bartonella henselae. The most common ophthalmological involvement due to infection by Bartonella is Parinaud oculoglandular syndrome, whereas the most common posterior segment findings are neuroretinitis and subsequent late macular star. Moreover, other findings, such as retinal or subretinal lesions, intermediate uveitis and angiomatous lesions, may be present. Case presentation A 37-year-old female patient with retinal findings and serological confirmation of Bartonella infection was evaluated via multimodal retinal exams. The patient received treatment with doxycycline 100 mg twice daily for 2 weeks. One month after treatment, complete improvement of her visual scotoma symptoms was confirmed. A swept-source optical coherence tomography exam also showed decreases in the size and intraretinal extension of the lesion. Improvement of light perception at the affected area was confirmed by microperimetry. Conclusions Bartonella henselae infection, particularly retinitis, can present a variable spectrum of clinical and ophthalmological findings. Multimodal retinal exams can clearly identify lesion characteristics, thus providing important information for diagnosis and the evaluation of lesion improvement after antibiotic treatment.

Ocular Burkholderia pseudomallei, a rare variant in presentation – a case series

Burkholderia pseudomallei is Gram-negative anaerobe causing melioidosis. We report a case series including three cases of rare ocular presentations in patients with positive melioidosis serology in the state of Pahang, Malaysia. The first case involved a 32-year-old male with a sudden onset of painless decrease in vision in the left eye, with a history of swimming in a river. Eye examination revealed a choroidal abscess with inferior retinal detachment. The second case was that of a 14 year-old-male patient with painless reduction of vision in the left eye and a history of parotid gland swelling. Eye fundus examination showed optic disc swelling with macular star. The third case, a 10-year-old male, presented with left eye optic disc granuloma and subtotal exudative retinal detachment with vasculitis. The three cases were successfully treated with the antibiotic ceftazidime. Ocular melioidosis should always be taken into consideration in any patient with suspected infectious uveitis. A high index of suspicion is required to initiate early and prompt treatment.

Leptospira, the ocular predator

Neuroretinitis is an inflammatory optic neuropathy with a classic funduscopic appearance of optic disc swelling and hard exudates on the macula in a star formation. It can be a manifestation of systemic, infectious or autoimmune disease. Here, we report three patients who presented with sudden onset of painless blurring of vision. Ophthalmic evaluation revealed a characteristic picture of neuroretinitis. Detailed study of the cases revealed leptospirosis being the aetiology of the neuroretinitis. Leptospirosis is a zoonotic infection caused by spirochetes Leptospira, which presents with both ocular and systemic manifestations. Neuroretinitis has been reported in the few cases of leptospirosis. We present three cases of leptospirosis with bilateral and unilateral neuroretinitis presenting with sudden loss of vision, optic disc oedema, and macular star. Leptospirosis was confirmed by serological test and the disease responded optimally to specific therapy. Although funduscopic examination showed marked inflammatory changes in the retina and optic nerve head, the recovery following medical treatment was remarkable.

Bartonella neuroretinitis (cat-scratch disease)

We report a patient with cat-scratch disease presenting with meningitis and neuroretinitis. This condition, caused by Bartonella henselae, has a worldwide distribution and is among the most common infective causes of neuroretinitis. Bartonella neuroretinitis is a rare but under-recognised mimic of optic neuritis; it should be suspected in a patient with an infective prodrome whose fundus shows optic disc oedema and a macular star. A low-positive initial serological test for Bartonella henselae does not exclude cat-scratch disease if there is high clinical suspicion, and repeat testing is recommended to look for titre rise.

Bilateral Idiopathic Neuroretinitis

BACKGROUND: Neuroretinitis is a clinical entity characterized by an acute loss of vision associated with disc edema and a star pattern of exudates in the macula. It can be divided into two, those with a specific infectious agent or idiopathic. Most infectious cases are due to cat-scratch disease caused by Bartonella species and other infectious agents. Case with a clear infectious is categorized as idiopathic. Most patients with idiopathic neuroretinitis recover excellent visual acuity with or without intervention. Although the presentation is most often unilateral, bilateral cases of neuroretinitis have been reported. CASE REPORT: A 20-year-old woman with a 2-weeks history of sudden progressive visual loss both eyes (visual acuity: RE 3/60; LE 20/200). The optic disc was edema and the hard exudate on the macular area. Two weeks follow-up, funduscopic shows a macular star and the optic disc’s edema was reduced. Perimetric test shows general depressed in both eyes. The blood tests and the brain computed tomography scan were normal. She received methyl prednisolone 48 mg for 2 weeks. Eight months follow-up, VA: RE 20/50, LE 20/40 with the pinhole 20/15 in both eyes. The funduscopic still showed edema and slight paling. There was an improvement in central visual acuity after steroid treatment but leave residual abnormal funduscopic. CONCLUSION: Neuroretinitis is generally self-limited. The visual acuity recovers excellent with the steroid, but the abnormal disc in this case related to a vaso-occlusive mechanism of prelamin arterioles with subsequent disc infraction.