Human Immunodeficiency Virus (HIV) Associated Retinopathy

The most common retinal manifestation of human immunodeficiency virus (HIV) is a retinal microvasculopathy, also known as noninfectious acquired immunodeficiency syndrome (AIDS) retinopathy. This microvasculopathy is characterized by diabetic retinopathy-like findings including cotton-wool spots and retinal hemorrhages or microaneurysms in the posterior pole and/or periphery. Opportunistic infections such as cytomegalovirus (CMV) retinitis, acute retinal necrosis (ARN), and progressive outer retinal necrosis (PORN) are more visually devastating viral retinal infections associated with HIV and require emergent systemic and/or local antiviral therapy. Very rarely HIV can cause a primary retinal infection.

Cotton-wool spots due to noninfectious HIV retinopathy.

Courtesy of Dr. Jay Pepose

This is an AIDS patient with retinal infiltrates associated with HIV infection. Note the small multifocal white peripheral retinal infiltrates in each eye with staining on the fluorescein angiogram (FA).

Courtesy of Dr. Robin Vora and Dr. Emmett Cunningham

Cytomegalovirus Retinitis

Cytomegalovirus (CMV) retinitis is the most common ocular opportunistic infection in AIDS and may also occur in other immunocompromised patients, especially in those with CD4 counts under 50/mm ³ . In the era of highly active antiretroviral therapy, the immune status of AIDS patients has dramatically improved and opportunistic retina infections such as CMV retinitis are more rarely encountered. Symptoms will vary, as peripheral disease (zone 3) may go unnoticed by patients. Visual changes will be perceived by patients if the infection involves the posterior pole (zone 1). Mild intraocular inflammation in the anterior chamber and vitreous may be present. The classic retinal findings of CMV retinitis include a hemorrhagic retinitis with a sectoral or perivascular distribution.

A “frosted branch angiitis” in areas with and without retinitis may be appreciated. Retinal venous occlusive disease and optic disc neovascularization may complicate the course. Patients should be closely monitored for the eventual development of rhegmatogenous retinal detachment requiring surgical intervention to prevent severe vision loss and blindness.

These are patients with CMV retinitis. A hemorrhagic retinitis is noted superiorly in the color montage with patch areas of “cheesy” necrosis associated with retinal hemorrhages in a perivascular distribution (arrows). Note the widespread “frosted branch angiitis” pattern throughout the fundus (top) and in the peripheral color photograph. The histopathology shows inflammatory cells along the walls of a retinal vessel. The FA shows active staining of the inflamed retinal vessels in a frosted branch angiitis pattern.

This is a patient with bilateral CMV retinitis. There is a diffuse frosted branch angiitis in both eyes. In the upper image, the right eye shows fibrous opacification of the superior chorioretinal area. In the lower image, the left eye shows a superior zonal region of atrophy from prior infection bordered by hemorrhage from an active necrotizing retinitis. There is involvement, but relative sparing of the paramacular region.

This patient shows resolving CMV retinitis with zonal atrophy superotemporally (asterisk) and active retinitis with opacification of the retina along the superotemporal arcade (arrowhead). There is a hemispheric retinal detachment (arrows) in the inferior fundus. Multiple areas of retinal folds and fibrous proliferative vitreoretinopathy have complicated the detachment.

Note the presence of macular CMV retinitis in the left color fundus photograph. The right photograph demonstrates active patches of peripheral retinitis in a different patient.

This patient has active CMV retinitis with diffuse perivascular necrosis of the peripheral retina. There are patchy hemorrhages bordering the infected tissue. There is also peripheral retinal atrophy, consistent with healing in areas with previously active infection (arrows).

These two patients have frosted branch angiitis secondary to CMV infection.

Upper image courtesy of Michael P. Kelly and Dr. Everett Ai

This patient has CMV retinitis and papillitis. Following antiviral medication, there is resolution of the infection, but note the presence of optic atrophy and sheathed retinal vessels and persistent macular exudates.

Courtesy of Dr. Richard Spaide

This montage demonstrates a well-demarcated zone of peripheral atrophy in the temporal fundus following CMV retinitis.

Light microscopy of a case of acute necrotizing retinitis due to CMV shows large cells (neurons) containing eosinophilic intranuclear and intracytoplasmic inclusions.

Acute Retinal Necrosis Syndrome

Acute retinal necrosis (ARN) syndrome can affect immunocompromised or immunocompetent patients and most typically occurs in otherwise healthy individuals. Clinical characteristics include (1) concentric areas of peripheral retinal necrosis with discrete borders; (2) relentless posterior progression of disease or development of new foci in the absence of antiviral therapy; (3) eventual macular involvement in the absence of antiviral therapy; (4) presence of an occlusive obliterative angiopathy; and (5) marked anterior and vitreal inflammation. Optic atrophy and rhegmatogenous retinal detachment due to multiple ragged round retinal tears at the border of resolved disease may complicate the course in a significant number of patients. The etiology of ARN is due to the herpes group of viruses that affect all layers of the retina. Herpes simplex virus type 1 and 2 (HSV-1, HSV-2) and varicella zoster virus (VZV) are the most common causative agents. Patients with HIV or AIDS are at risk for developing ARN following herpes zoster ophthalmicus, even after the skin lesions resolve.

These patients with acute retinal necrosis syndrome all show peripheral full-thickness retinitis with well-demarcated borders, whitening or opacification of the retina, occlusive retinal vasculitis, and vitritis. Retinal hemorrhage is not a prominent feature of this syndrome, but is present at the margins of necrotic tissue in some eyes. These patients all had evidence of herpes group virus infection. The patient on the right had an ischemic process that extended rapidly into the posterior pole.

This patient has diffuse peripheral retinal necrosis in a concentric pattern with vitreous inflammation consistent with ARN. Note the absence of retinal hemorrhage that is characteristic of the lesions. The confluent yellow–white appearance with irregular scalloped posterior margins and sharp transition between involved and non-involved portions of the retina is typical.

Courtesy of Dr. Alex Aizman

This patient with ARN shows necrotizing retinitis with obliterative angiopathy in the area of retinal infection. FA shows retinal ischemia associated with this necrotizing obliterative angiopathy.

Courtesy of Dr. Tatiana Forofonova

These patients with the ARN syndrome demonstrate eosinophilic intranuclear inclusions (left and middle image) . Inflammatory cells can be seen around retinal vessels (right image) and are predominantly mononuclear cells. Zones of retinal pigment epithelium (RPE) proliferation and migration underlying thin, necrotic retina are also evident.

Acute Retinal Necrosis Syndrome: Herpes Simplex Type 1

In the ARN syndrome secondary to herpes virus, there are some characteristic, but not pathognomonic changes seen with herpes simplex type 1 versus type 2. In this patient with bilateral ARN due to herpes simplex type 1, there is predominantly a retinitis characterized by inflammatory changes (frosted branch angiitis) around infected vessels.

Acute Retinal Necrosis Syndrome: Herpes Simplex Type 2

In this patient with bilateral ARN due to herpes simplex type 2 infection, the predominant characteristic is that of ischemia or an obliterative necrotizing vasculitis.

Virtually all viral infections in the herpes group have been identified to be a causative factor for acute retinitis. The patient above had a concomitant herpes dendritic corneal ulcer associated with ARN of the right eye. The patient below demonstrated herpes zoster ophthalmicus associated with ARN of the left eye. Each patient had optic nerve inflammation or papillitis.

This immunosuppressed patient with hypogammaglobulinemia developed bilateral ARN soon after administration of the Varivax vaccine and while being treated with multiple immunosuppressant therapies. Note the peripheral full-thickness retinal necrosis with retinal hemorrhages in the right eye and focal necrosis in the temporal macula of the left eye. The vaccine strain of herpes zoster was isolated through PCR of a vitreous sample.

Courtesy of Dr. Ashleigh Levinson

Progressive Outer Retinal Necrosis

Progressive outer retinal necrosis (PORN) is a severe variant of a necrotizing herpetic retinopathy in profoundly immunocompromised patients with CD4 counts less than 5/mm ³ . It is believed to be the second most frequent opportunistic retinal infection after CMV retinitis in patients with AIDS. Clinical laboratory evidence suggests that the varicella zoster virus is the causal agent. Early clinical manifestations include patchy deep retinal lesions in the posterior pole and peripheral fundus, unlike ARN, which is typically peripheral in the early stages of disease. These discrete areas of retinal opacification are usually multiple and can range in size from 50 to several thousand microns in diameter. The retinitis is characterized by primary involvement of the outer retina with sparing of the inner retina until later stages of the disease process. The acute lesions progress rapidly, resulting in confluent patches of full-thickness necrosis with minimal or no aqueous or vitreal inflammation. A retinal vasculopathy is not characteristic of PORN. A perivascular lucency thought to represent early removal of necrotic debris or edema can result in a pattern of scarring that has a “cracked-mud” appearance. Optic nerve involvement, including swelling and atrophy, may occur. End-stage PORN leads to retinal detachment and blindness.

These are patients with PORN with initial focal and multifocal areas of outer retinitis involving the macula. There is leakage seen with FA.

This patient with PORN demonstrates progressive confluency of the outer retinal necrosis. The retinal vessels are seen to be anterior to the outer retinal infection.

Left image courtesy of Dr. Richard Spaide

More widespread and diffuse infection is seen as the initial lesion becomes dense and confluent and, in some areas, involves full-thickness retina as in these patients with PORN.

Variable manifestations of multifocal areas of infection with progression in the outer retina are seen in these patients with PORN.

This montage shows a myriad of changes in PORN. First, there are zonal areas of outer retinal and pigment epithelial atrophy in regions of antecedent acute infection (short arrows). Acute infection is also seen elsewhere, particularly in the nasal quadrant (arrowheads). This patient has also been treated with laser photocoagulation in the temporal and superotemporal periphery (long arrows). Marked ischemic changes and sheathing are also noted in the retinal vasculature, particularly in the nasal juxtapapillary region. A “cracked-mud” appearance is seen surrounding some vessels (pink arrows). Presumably this lucency around the vessels is due to resolving necrotic debris or edema.

This patient developed bilateral PORN from VZV. Note in the color fundus photos the diffuse peripheral outer retinal whitening with scattered retinal hemorrhages.

Note on the FA the leakage in the areas of confluent infection both in the macula of the right eye and in the periphery of both eyes. Spectral domain (SD)-OCT of right eye (bottom left photo) shows retinitis and cystoid macular edema (CME). SD-OCT after intravenous antiviral treatment (bottom right photo) for VZV (patient was PCR positive) shows diffuse retinal atrophy and cavitation with resolution of CME and retinitis.

Courtesy of Dr. Purnima Patel

Epstein–Barr Virus Retinitis

The Epstein–Barr virus (EBV) may very rarely cause a retinitis that produces whitening of the retina with patterns that have indistinct margins and very minimal hemorrhage and inflammation.

This patient presented with a diffuse retinitis in the posterior pole and periphery of both eyes due to presumed EBV infection ( left images ). After resolution diffuse atrophy and pigment mottling are shown in the posterior pole and periphery of both eyes and a dense atrophic scar is noted in the macula of the left eye ( right images ).

Courtesy of Drs. Stephen Jae Kim and Daniel F. Martin

Rubeola Virus: Subacute Sclerosing Pancencephalitis (SSPE)

Rubeola virus may very rarely cause a chronic progressive encephalitis and retinitis that affects primarily children and young adults. It is the result of a persistent infection of immune-resistant measles virus (rubeola). A variety of central nervous system (CNS) abnormalities are associated with this infection, and there may be ocular manifestations that can lead to blindness.

This patient has diffuse atrophy and mottling of the retinal pigment epithelium (RPE) due to SSPE, which was relentlessly progressive.

This patient with SSPE experienced a relentless retinitis that left a diffuse pattern of chorioretinal degeneration illustrated with widefield fundus autofluorescence. Large patches of chorioretinal atrophy are seen in both eyes.

Congenital Rubella Syndrome

The congenital rubella syndrome, caused by the rubella virus, may be associated with microphthalmos, congenital cataracts, iris abnormalities, and coloboma formation of various ocular structures. Children born to mothers who contracted rubella in the first trimester of pregnancy are at high risk of deafness and pigmentary retinopathy that consists of patchy RPE atrophy and pigment epithelial mottling and hyperplasia referred to as “salt and pepper” retinopathy. Active ocular inflammation and/or retinitis are not appreciated with this syndrome. In later life, such patients are subject to choroidal neovascularization and disciform scarring of the macula.

This patient has congenital rubella retinopathy demonstrating patchy RPE atrophy and pigment epithelial mottling and hyperplasia throughout the fundus.

This patient developed secondary choroidal neovascularization in the central macula. The vascular proliferation has evolved to a fibrous disciform scar (left, arrows) that stains on the FA.

Coxsackievirus

Acute Retinal Pigment Epitheliitis

Coxsackievirus may be the etiology of infectious diseases of the retina and pigment epithelium with predilection for the central macula. A multifocal perifoveal reaction associated with pigment epithelial changes and macular edema, as well as a papillitis, is a very rare clinical presentation associated with this virus. A more typical association is the acute idiopathic maculopathy (AIM) syndrome, wherein there is increasing evidence of coxsackievirus as an etiological agent.

Coxsackievirus has been implicated in a perifoveal pigment epitheliitis, which is a very rare disorder, referred to as Krill disease. Note the pigmentary lesions surrounded by haloes in the macula of each eye in this patient ( left two images ) with an antecedent febrile illness and mouth ulcers (not shown). The two images on the right were unilateral cases.

Two images on left courtesy of Dr. Richard G. Gieser

Acute Idiopathic Maculopathy

Acute idiopathic maculopathy (AIM) is a rare disorder that affects healthy young adults. Patients present with sudden central vision loss, usually in one eye. Symptoms often follow a viral prodrome, presumed to be coxsackievirus that is the etiology of hand-foot-mouth disease. During the acute phase, a neurosensory detachment overlying a grayish plaque at the level of the RPE is seen, often eccentric to the fovea, which may simulate the appearance of choroidal neovascularization. Associated intraretinal hemorrhages, few vitreous cells, and a mild papillophlebitis may be present and the FA shows rapid and severe subretinal leakage. Most cases resolve spontaneously over several weeks with near-complete recovery of vision. A lasting “bull’s-eye” pigment epithelial maculopathy is typically seen following resolution of the acute lesion.

This 45-year-old man with AIM presented with a 3-day history of vision loss. Visual acuity was 20/200. An irregular exudative detachment of the neurosensory retina is noted. There is an area of intraretinal hemorrhage superior to the macula and subfoveal placoid thickening at the level of the RPE. The FA shows early blockage with rapid and severe late leakage into the neurosensory macular detachment. The lesion resolved spontaneously.

One year later, irregular pigment epithelial hyperpigmentation surrounds a central area of presumed subretinal fibrosis in the foveal region.

The corresponding FA of the same patient shows a concentric area of RPE atrophy consistent with a “bull’s-eye” maculopathy. Visual acuity improved to 20/25.

This patient with AIM has an eccentric neurosensory retinal detachment with intraretinal hemorrhages corresponding to multiple focal areas of hypofluorescence on the fluorescein angiogram within the detached area. There is also an underlying placoid area of fluorescence corresponding to flat, inflammatory change at the level of the RPE. There is significant leakage into the neurosensory detachment, which simulates choroidal neovascularization or inflammatory disease. In this patient, there was an additional AIM lesion inferior to the disc (right image) in the fellow eye. The patient, whose daughter was afflicted with coxsackievirus infection, experienced a similar febrile, prodromal illness prior to her visual symptoms.

This patient with AIM presentation shows a circumscribed area of detachment of variable size and translucency. This singular manifestation in the fundus was associated with a sudden and profound decline in vision, which recovered spontaneously.

This patient had AIM with a patch of subretinal whitening beneath the neurosensory detachment. Note the associated intraretinal hemorrhage (arrow). Severe subretinal leakage is seen with the FA. The optic nerve was also inflamed, exhibiting late staining on the FA, indicative of a papillitis.

This patient had AIM and, 18 months after resolution of the acute manifestations, developed choroidal neovascularization. The neovascular lesion is bordered by a margin of subretinal hemorrhage, and demonstrates a “classic” appearance with FA (type 2 neovascularization).

Following resolution of the acute manifestations, AIM patients commonly demonstrate a “bull’s-eye” appearance to the macula. Hyperpigmentation is present at the site of the acute placoid subretinal inflammatory lesion. The concentric or “bull’s-eye” pattern of atrophy corresponds to the previous macular detachment.

This patient with acute idiopathic maculopathy (AIM) presented with a well-demarcated yellow–white lesion with subtle punctate intraretinal heme and gray central pigment (top left). The FA shows significant pooling within the neurosensory detachment. OCT of the lesion confirms the presence of subretinal fluid (arrow). Eight months after initial presentation, visual acuity was stable at 20/20. The bottom right photo shows RPE hyperpigmentation and depigmentation in a “bull’s-eye” pattern with resolution of hemorrhages.

Courtesy of Dr. Yannis Paulus

Rift Valley Virus

Rift Valley fever is a viral zoonosis that primarily affects animals but also has the capacity to infect humans. The virus is a member of the Phlebovirus genus, and it was first identified in the Rift Valley of Kenya. It is transmitted to humans from direct or indirect contact with the blood of an infected animal or the bite of an insect, most commonly the Aedes mosquito. Systemic disease, including hemorrhagic fever and meningoencephalitis, may occur. When it affects the eye, there is usually retinal vascular involvement, which may include hemorrhage, vasculitis, and occlusive disease.

A Rift Valley infection in this patient has produced a retinitis with cotton-wool spots and lipid exudation in a macular star pattern.

Courtesy of Dr. Maurice Luntz

West Nile Virus

West Nile virus, a member of the flavivirus family, is transmitted by a mosquito and may result in severe systemic, CNS, and ocular manifestations. In the fundus, a vitritis with scattered creamy-yellow circular or round chorioretinal lesions may develop that resembles multifocal choroiditis. As they heal, they leave atrophic areas. Linear scars are characteristic. Retinal hemorrhages and exudates may also be noted.

This patient with West Nile virus has diffusely scattered, punched-out multifocal chorioretinal lesions following the acute infection.

Courtesy of Dr. Nicole Hauptman-Siegel

These two patients experienced a West Nile virus systemic illness. Each developed a multifocal curvilinear area of atrophic and pigmentary abnormalities that resemble a multifocal choroiditis. The lesions are actually random and do not follow what appears to be a choroidal vascular pathway.

Left two images courtesy of Dr. Ron Adelman

This patient had an acute West Nile virus infection with severe systemic symptoms, which included coma. There was also bilateral ocular involvement. Following the acute infection, which involved the retina, there were multifocal areas of chorioretinal atrophy with some confluency of contiguous lesions. The FA, taken at a later date, shows staining of the many atrophic chorioretinal scars. The random distribution of these lesions resembles multifocal choroiditis without fibrosis or significant hyperpigmentation. The lower right color montage of the fellow eye has similar manifestations.

Courtesy of Dr. Mark Johnson

This is a case of West Nile Virus chorioretinitis. The color fundus photos show numerous mid-peripheral cream-colored round chorioretinal lesions (some with a halo configuration) that stain with FA. IgM and IgG antibodies were positive for West Nile Virus.

Courtesy of Dr. Susan Anderson-Nelson

Dengue Virus Maculopathy

The dengue virus is an RNA virus (of the flavivirus family) with four distinct serotypes that is widespread in tropical climate locales. It causes the clinical disease dengue fever, which is marked by high fevers, joint and bone pain, headache, and rash. In its most severe form victims suffer from dengue hemorrhagic fever, which is characterized by the aforementioned symptoms in addition to thrombocytopenia and often multiorgan failure. Dengue virus can cause a maculopathy associated with blurred vision and/or a scotoma. Fundus findings include macular and/or optic disc edema, retinal hemorrhages, venous sheathing, and yellow subretinal dots. More recently, multimodal imaging findings typical of acute macular neuroretinopathy have been described in these patients. Oral and intravenous steroids are the mainstay of treatment as the pathogenesis is postulated to be immune-mediated.

This patient with dengue has normal-appearing fundi in color photos but the infrared images reveal the characteristic hyporeflective paracentral lesions of acute macular neuroretinopathy (AMN). SD-OCT shows loss of the interdigitation zone and attenuation of both the inner segment ellipsoid band and the external limiting membrane (ELM) with corresponding hyper-reflectivity of the Henle layer. Vision improved to 20/30 OU after systemic steroid treatment.

Courtesy of Dr. Eduardo Cunha de Souza and Munk, M.R., Jampol, L.M., Cunha Souza, E., et al., 2016. New associations of classic AMN. Br. J. Ophthalmol. 100(3), 389-394

Toxoplasmosis

Toxoplasmosis infection is caused by the obligate intracellular protozoan, Toxoplasma gondii . There are two stages of the life cycle of the protozoan found in humans. The tachyzoites, measuring about 6 µm in length, comprise the first stage and the bradyzoites, thousands of which may be contained in cysts measuring up to 200 µm in diameter, comprise the second stage. Toxoplasmosis retinitis is the most common retinal infection and is typically only active in one eye at a time.

In immunocompromised patients, the CNS is the preferred site of infection, with cerebral toxoplasmosis reported in as many as 40% of autopsy eyes. Ocular toxoplasmosis is much less common than cerebral toxoplasmosis, accounting for less than 1% of AIDS-related retinal infections in the USA. Systemic infection with T. gondii is most commonly asymptomatic and approximately 500 million people worldwide have antibodies to the organism.

Most toxoplasmosis infections are in otherwise healthy hosts and symptoms are due to reactivation of organisms. Pre-existing chorioretinal scars indicate prior infection. Primarily acquired toxoplasmosis infection of the retina with an absence of pre-existing chorioretinal scars is more typical in AIDS patients. Acute toxoplasmic lesions (primary or reactivated) are focal yellow–white areas of necrotizing retinitis associated with a severe vitritis (“headlight in the fog”). The lesions have fluffy borders with few scattered hemorrhages. Vascular sheathing may be prominent (Kyrieleis plaques).

Congenital Toxoplasmosis Scars

These patients all demonstrate congenital toxoplasmosis scars with discrete areas of hyperpigmentation and a variable degree of associated atrophy. Note the presence of fibrosis within some of these scars (arrows).

Top row right image courtesy of Alan Campbell, CRA

Acute Toxoplasmic Lesions

Acute toxoplasmosis presents with a focal yellow–gray inflammatory infiltrate within the retina. Satellite lesions may also be noted. A variable degree of overlying vitreous inflammation is typically present and a papillitis may also be noted. The acute lesions are often seen in contiguity with an old pigmentary scar (arrows) indicating reactivated disease.

This patient with acute toxoplasmosis presented with iritis and vitritis as well as a cream-colored inflammatory lesion in the macula (top photo). SD-OCT revealed inner retinal opacification. With intravitreal and oral antibiotics as well as oral steroids the vitritis and inner retinitis both resolved.

These patients show the variable presentation of an acute toxoplasmosis lesion in the fundus. Note the proximity to chorioretinal pigmentary scars and associated inflammatory activity in the fundus. Conversion from an acute to a healed lesion is appreciated in the two cases in the bottom row.

Top row middle image courtesy of Dr. Emmett Cunningham

Miliary Toxoplasmosis

This HIV patient had miliary toxoplasmosis retinitis. Multifocal yellow–white retinitis is seen (left) . Macular hemorrhage was noted 5 weeks later due to venous occlusive disease. There were also numerous additional infectious lesions evident at that time.

Courtesy of Dr. William Freeman

Toxoplasmosis with Kyrieleis Plaques

Although first described in association with tuberculosis retinitis by Dr. Werner Kyrieleis, calcific plaques on the walls of blood vessels are a well-known complication of toxoplasmosis retinitis. Note the refractile calcific-like lesions (arrows) along the course of inflamed vessels in these patients. The noncorrelating histopathology suggests that there are inflammatory cells that aggregate on the walls of the vessels to produce mineralization seen clinically. The bottom images (left and middle) show an acute toxoplasmosis lesion with Kyrieleis plaques near the disc. The retinitis is associated with staining of the optic nerve and a neurosensory retinal detachment in the macula, as illustrated in the FA (bottom right). Kyrieleis plaques are also seen in a variety of other infectious, inflammatory, and infiltrative disorders associated with retinal vasculitis.

Bottom row courtesy of Dr. Ketan Laud

Toxoplasmosis and Choroidal Neovascularization

Patients with toxoplasmosis retinitis may experience a significant decline in vision from recurrent infection or from secondary choroidal neovascularization.

This patient has an acute exudative macular detachment (arrows) from choroidal neovascularization, which emerged from the edge of a healed congenital toxoplasmosis scar. The FA shows classic type 2 neovascularization in the central macula, bordered inferiorly by a chorioretinal scar. Unfortunately, there was recurrent neovascularization with subretinal hemorrhage and fluid (right), leading ultimately to a disciform scar.

Right image courtesy of Dr. Alan Berger

This patient had a congenital toxoplasmosis scar. At the age of 11, she developed acute choroidal neovascularization with subretinal hemorrhage and an exudative macular detachment (left). She was treated with anti-angiogenic therapy, which induced consolidation of the fibrovascular lesion and resolution of the serosanguineous detachment. However, there was a legacy of a macular scar.

This patient had congenital toxoplasmosis, and she developed choroidal neovascularization at the nasal margin of the scar with serosanguineous detachment.

This patient demonstrates an old fibrotic and pigmentary toxoplasmosis scar. There is fibrosis (arrowheads) temporal to an occluded vessel, which is surrounded by pigmentation. Choroidal neovascularization (CNV) occurred on the foveal side of the scar (arrows) with bleeding into the fovea. Laser treatment was carried out to ablate the neovascular lesion, resulting in obliteration of the membrane and the development of an atrophic scar surrounding the lesion with sparing of the fovea (right image).

Giardiasis

Giardia is a protozoan organism that can cause ocular complications such as pigment epithelial changes in a “salt and pepper” pattern. Giardiasis may also be associated with mild non-specific intraocular inflammation.

These patients have chronic giardiasis infection and associated retinal vasculitis. Note the presence of sheathing and infiltration of the retinal vessels in each case.

Leprosy

Leprosy or Hansen disease is caused by Mycobacterium leprae . Systemic manifestations are the result of nerve damage leading to structural deformities of the hands and feet. Cutaneous scarring, which includes a remarkable leonine appearance of the face, is characteristic of this disease. Ocular manifestations include sclerokeratitis and cataract formation. Retinal manifestations are very rare as the organism prefers infestation of tissues associated with cooler environments.

This patient has leprosy with corneal infiltration and multiple cutaneous lesions. An old inactive retinal phlebitis is noted with perivascular retinal atrophy and hyperpigmentation and scarring.

Courtesy of Dr. Karen M. Gehrs

Tuberculosis

Tuberculosis remains a major cause of morbidity and mortality worldwide. The HIV-infected population accounts for most of the increase in the prevalence of this infection. Choroidal tubercles and tuberculomas are the most common manifestation of ocular tuberculosis. Uveitis, optic neuritis, and retinal vasculitis in the posterior pole and peripheral fundus may also be encountered.

Tuberculosis may produce a retinal vasculitis. Note the evidence of retinal vascular staining and leakage with the FA. Hemorrhages into the vitreous may occur from new blood vessel proliferation (right image).

The histopathology images show inflammatory cells in the walls of vessels with associated mineralization. In the right image, we see that there are calcific-like plaques within the walls of the arterioles referred to as Kyrieleis plaques, which are a non-specific change in the retinal vasculature following inflammation or infection.

This patient with tuberculosis retinitis has calcific deposition in the walls of the arterioles (Kyrieleis plaques) following acute inflammation. Tuberculosis was the first disease associated with these calcific plaques, as was described by Dr. Werner Kyrieleis.

Courtesy of Dr. Richard Rosen

Tuberculosis may present as focal and multifocal choroidal granulomas. Small granulomas are seen in this patient (left). Larger, multifocal lesions are seen with vitreous inflammation in the second case (second from left) . The third patient has multifocal chorioretinitis and optic neuritis from tuberculosis. The FA image shows leakage in the retina and optic nerve.

Courtesy of Dr. Richard Spaide

These are two cases of focal tuberculous choroiditis with an overlying localized exudative macular detachment. The OCT images show fibrous adherence between the detached retina and the retinal pigment epithelium and associated subretinal fluid.

Courtesy of Amjad Salman, MS

These two patients have choroidal granulomas from tuberculous choroiditis. The granuloma is close to the optic nerve (left). Radial exudate is extending into the macula and a dependent exudative detachment is noted inferiorly (arrows). The second color montage includes images of a large fibrotic choroidal granuloma with active associated hemorrhage. Blood and exudation have gravitated inferiorly to form a dependent detachment (arrows).

Left image courtesy of Dr. Scott Sneed

This patient has widespread retinal vascular inflammation with a frosted-branch angiitis presentation, retinal venous occlusive disease with scattered hemorrhages, and optic neuritis from tuberculosis.

This patient has tuberculosis that resembles a placoid choroidopathy. The FA shows blockage of the choroidal fluorescence with multifocal punctate staining within the acute placoid lesion. This presumed variant of serpiginous choroidopathy with a progressive course resembling relentless placoid chorioretinitis or so-called ampiginous chorioretinopathy is common in India in patients with tuberculosis.

Courtesy of Dr. Benjamin Freilich

These are three patients with acute choroidal granuloma due to tuberculosis with variable degrees of exudative retinal detachment (left images). Following treatment there was resolution of the exudation and regression of the choroidal granuloma with the development of chorioretinal atrophy and fibrous scarring in each of the three cases (right images).

This is a case of tuberculosis presenting as serpiginous choroiditis. The color fundus photographs show a large chorioretinal scar throughout the posterior pole with an active border of yellow-gray subretinal infiltrates (arrows) . Mottled fluorescence of the lesion is seen with FA and fundus autofluorescence.

The spectral domain (SD) OCT shows a cross section through the active subretinal infiltrate (arrow) . With a regimen of isoniazid, rifampin, ethambutol, and pyrazinamide the chorioretinal infiltrates resolved as seen in the final color fundus image.

This patient with tuberculosis presented with a macular star, disc hyperemia, and vitreous haze as seen in the color photograph of the right eye (top) . Indocyanine green (ICG) angiography reveals a macular choroidal nodule (arrow), and FA shows staining of the nodule and late disc leakage. SD-OCT reveals a large subfoveal choroidal granuloma with associated subretinal fluid. The vision worsened due to noncompliance with medication.

Courtesy of John A. Gonzales, MD

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