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Retinal Manifestations of Systemic Disease – Part 1

Sundeep Dev, MD

VRSF Retinal Update 2019

VitreoRetinal Surgery, PA

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The Retina and Systemic diseases

Retinitis/Vasculitis

Vitreous cells

Serous detachments

Choroidal lesions

Pigmentary retinopathies

Choroidal folds/Choroidal masses

Retinal Vascular abnormalities May be unilateral or bilateral

May or may not have systemic symptoms 2

Placoid Outer retinitis

50 yo male Va = 20/50 3

FA LATE

Phlebitis

“hot nerve”

Outer retinitis

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Retinitis Differential Diagnosis Infectious: Viral -

HSV/HZV - Acute Retinal Necrosis (ARN), Progressive Outer Retinal Necrosis (PORN)

CMV Fungal - especially Candida Bacterial - Syphilis, Tuberculosis, Septic embolus,

Bartonella, Meningococcus, Cat-scratch (Bartonella) Parasitic - Toxoplasmosis, Cysticercosis, OnchocerciasisInflammatory: Sarcoidosis Behcet’s diseaseNeoplastic: Masquerade syndromes - Lymphoma 5

Retinal Vasculitis

Retinal Phlebitis

Sarcoidosis

Tuberculosis

Syphilis

Multiple sclerosis

Pars planitis

Eales’ disease

Antiphospholipid antibody syndrome

HIV

Frosted branch angiitis

Retinal Arteritis

Behcet’s disease

Collagen vascular disease – Systemic Lupus, Polyarteritis nodosa, Wegener’s granulomatosis

Toxoplasmosis

Viral – HZV/HSV/CMV/HIV

Antiphospholipid ab synd.

Eales’ disease

Syphillis6

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Lab Results:

RPR Positive

FTA-ABS Positive

Treponemal antibody current best test

Diagnosis: Syphilitic Retinitis

Treated with Neurosyphilis regimen

IV PCN G 2 million units q 4 hours for 14 days

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Inner RetinitisOS

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OS

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Bacterial Septic Embolus

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Septic Bacterial Retinitis with overlying vitreous involvement

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Roth spot

White centered hemorrhage – fibrin/immune complex. Classic description in endocarditis. Also in leukemia, vasculitis, diabetes, but non-specific.

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Endogenous Candidal Infection

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Systemic Fungal infection

Classic “fluff balls”

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Acute Retinal Necrosis

Patchy peripheral retinitis that coalesce. Vitreous haze/inflammation. HZV/HSV. Immunocompetent patients.

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ARN

Hemorrhage often seen with retinitis16

Acute Retinal Necrosis (ARN)

Note: Vitreous haze17

Acute Retinal Necrosis (ARN) OCT (Inferior)

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OS

Progressive Retinal Necrosis – usually HZV/HSV. PCR test helpful

Clear vitreous, immunocompromised patients

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Progressive Outer Retinal Necrosis

Note: Clear Vitreous20

Rocky Mountain Spotted Fever

Courtesy of GK Shah, MDRickettsial infection 21

Retinitis with severe vaso-occlusion

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Cytomegalovirus Retinitis (CMV Retinitis)

Immunosuppressed, though may occur in diabetics after steroid injections. Treat with oral Valganciclovir and intravitreal Foscarnet or Ganciclovir.

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CMV Retinitis

Complex RD requiring SO in extensive cases. 24

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Cat-scratch

Test for Bartonella hensalae and quintana 25

Cat-scratch early onset

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Cat Scratch

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Cat Scratch

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Toxoplasmosis

Any positive blood titer (IgM or IgG) helpful. PCR helpful. Acquired or Congenital (adjacent to a hyperpigmented scar)

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Toxoplasmosis-Consuming Undercooked Venison

Bactrim DS works well. Can densensitize patients. Intravitreal Clindamycin for vision threatening disease. 30

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Retinal Vasculitis

Think collagen vascular disease 31

Vasculitis-Lupus

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Vasculitis-Lupus

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Vasculitis-Lupus

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Vasculitis-Lupus

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Wegener’s (ANCA +) Vasculitis

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Wegener’s (ANCA +) Vasculitis

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Behcet’s disease

Retinitis/Vaso-occlusive arteritis. Oral/genital ulcers. 38

Behcet’s disease

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Classic Masqueraders

Always keep these conditions in the differential of retinitis/vasculitisas they can mimic many different

conditions

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Syphillis - “The great masquerader”

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Sarcoidosis

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Sarcoidosis

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Tuberculosis

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Lymphoma - another “great masquerader”

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Unusual Vitreal Cells

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51 y.o with progressive floaters OS

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Fundus Photos

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Fluorescein Angiogram

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OCT- OS

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Differential Diagnosis of Chronic Large Vitreous Cells

Chronic fungal endophthalmitis Lymphoma Sarcoidosis Amyloidosis Whipple’s disease Metastatic cancer cells Inflammatory Bowel disease Tuberculosis Lyme disease Idiopathic Intermediate uveitis

Often require a vitreous biopsy to diagnose51

Diagnosis: Whipple’s disease

Vitreous biopsy Blood/Vireous PCR

positive for T. whippellipositive

Light microscopy:

Whipple’s disease Chronic multisystem bacterial

disease: weight loss, chronic diarrhea, vague abdomnalpain, migratory arthralgias

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Pars Planitis

Lyme

Tuberculosis

Syphillis

Sarcoidosis

Multiple sclerosis

Toxocariasis

Idiopathic

Snowbank

Snowballs

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Endogenous Fungal Infection

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Vitreous Amyloidosis

Glass wool like or cobweb sheets. Clear with vitrectomy 55

Gündüz, Shields, Shields, Eagle Ophthalmol 1998;105:600-605

Metastatic Melanoma

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Lymphoma

Sheets of clumped cells in vitreous57

Lymphoma

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Ocular Lymphoma

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Ocular Lymphoma

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Serous Detachments

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Serous Detachments

Differential Diagnosis: Central serous retinopathy – steroids, catecholamines,

pregnancy, renal transplant

CNV

Optic pit

Posterior Scleritis

VKH (Vogt-Koyanagi-Harada Syndrome)

Rhegmatogenous RD

Circumscribed Choroidal Hemangioma

Tumor (amelanotic melanoma, metastasis)

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fibrin

CSR in pregnancy – 8 months

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CSR in pregnancy

Subretinalprecipitates

1 month post partum

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Vogt-Koyanagi-Harada Syndrome

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VKH – bilateral uveitis, serous RD’s, associated with cutaneous, auditory, and neurologic abnormalities.

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Punctate leaks on FA

VKH

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Vitiligo69

Vogt-Koyanagi Syndrome (VKH)

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Vogt-Koyanagi Syndrome (VKH)

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Vogt-Koyanagi Syndrome (VKH)

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Vogt-Koyanagi Syndrome (VKH)

Need steroids or immunosuppression. Rapid response, but careful to taper very slowly. Can follow EDI Choroidal thickness. Dex implant may help.

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Optic Nerve Pit with Schisis

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Optic Nerve Pit with Schisis

Management – Laser, Vitrectomy/Laser, Retinal fenestrationTakes a long time to see results

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Choroidal Lesions

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47 year old healthy Asian-Indian male. Blurry spot OD, 20/25 OU. No cells.

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Differential Diagnosis of Multifocal Placoid Chorioretinal Lesions AMPPE (Acute Multifocal Posterior Placoid

Epitheliopathy)

Serpiginous Choroidopathy

Ampiginous or “Relentless” Choroidopathy

Masquerade Syndromes - Lymphoma, TB, Syphillis

Multifocal Choroiditis – West Nile Virus, Lyme, Pneumocystis, PIC, Idiopathic

Histoplasmosis

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AMPPE

Occasionally associated with cerebral vasculitis, , neurosensory hearing loss, erythema nodosum suggesting immune mediated vascular alterations

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AMPPE

Multiple placoid lesions

Often bilateral

HLA B7 / DR2

Recurrences rare

Vision loss with foveal involvement

Steroids may speed recovery

Usually resolves within 4-8 weeks, but reported to last up to 6 months

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Serpiginous

Peripapillary; bilateral, progressive. Males more common. Recurrences can be months to years apart. CNVM can develop. Requires immunosuppressive therapy. Test for TB.

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Serpiginous

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Serpiginous

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Serpiginous

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Diagnosis: Ampiginous/Relentless placoid choroiditis.Associated with TBQuantiferon gold testing.Patient quiescent after 9 months of TB rx.

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Multifocal Choroiditis

Can be associated with subretinal fibrosis. CNVM can cause vision loss. Immunosuppressive Rx.

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West Nile Virus

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Lyme Choroiditis

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Pneumocystis Carinii choroiditis

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Ophthalmomysis

Courtesy of E. Holz, MD92

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OPHTHALMOMYIASIS

Larval form of flies from the order Diptera

Botflies: cattle, sheep, horses, deer, rodents, human

Most common: Cuterabra (rodent botfly) Usual host: rabbits, squirrels, field mice, rats,

chipmunks

Eggs transported to eye by fly or hands

Bore into eye may occur in AC, vitreous, SR space

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Stay tuned for Part 295