Retinal Manifestations of Systemic Disease Part 1

Transcription

1 The Retina and Systemic diseases Retinal Manifestations of Systemic Disease Part 1 Sundeep Dev, MD VRSF Retinal Update 2019 VitreoRetinal Surgery, PA 1 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 FA LATE Outer retinitis hot nerve 50 yo male Va = 20/50 3 Phlebitis 4 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, Onchocerciasis Inflammatory: Sarcoidosis Behcet s disease Neoplastic: 5 Masquerade syndromes - Lymphoma Retinal Phlebitis Sarcoidosis Tuberculosis Syphilis Multiple sclerosis Pars planitis Eales disease Antiphospholipid antibody syndrome HIV Frosted branch angiitis Retinal Vasculitis 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 Syphillis 6 1

2 Lab Results: Inner Retinitis OS 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 7 8 OS Bacterial Septic Embolus 9 10 Septic Bacterial Retinitis with overlying vitreous involvement Roth spot 11 White centered hemorrhage fibrin/immune complex. Classic description in endocarditis. Also in leukemia, vasculitis, diabetes, but non-specific. 12 2

3 Endogenous Candidal Infection Systemic Fungal infection Classic fluff balls Acute Retinal Necrosis ARN Patchy peripheral retinitis that coalesce. Vitreous haze/inflammation. HZV/HSV. Immunocompetent patients. 15 Hemorrhage often seen with retinitis 16 Acute Retinal Necrosis (ARN) Acute Retinal Necrosis (ARN) OCT (Inferior) Note: Vitreous haze

4 Progressive Retinal Necrosis usually HZV/HSV. PCR test helpful Progressive Outer Retinal Necrosis Clear vitreous, immunocompromised patients 19 OS Note: Clear Vitreous 20 Rocky Mountain Spotted Fever Retinitis with severe vaso-occlusion Courtesy of GK Shah, MD Rickettsial infection Cytomegalovirus Retinitis (CMV Retinitis) CMV Retinitis Immunosuppressed, though may occur in diabetics after steroid injections. Treat with oral Valganciclovir and intravitreal Foscarnet or Ganciclovir. 23 Complex RD requiring SO in extensive cases. 24 4

5 Cat-scratch Cat-scratch early onset Test for Bartonella hensalae and quintana Cat Scratch Cat Scratch Toxoplasmosis Toxoplasmosis-Consuming Undercooked Venison Any positive blood titer (IgM or IgG) helpful. PCR helpful. Acquired or Congenital (adjacent to a hyperpigmented scar) 29 Bactrim DS works well. Can densensitize patients. Intravitreal Clindamycin for vision threatening disease. 30 5

6 Retinal Vasculitis Vasculitis-Lupus Think collagen vascular disease Vasculitis-Lupus Vasculitis-Lupus Vasculitis-Lupus Wegener s (ANCA +) Vasculitis

7 Wegener s (ANCA +) Vasculitis Behcet s disease 37 Retinitis/Vaso-occlusive arteritis. Oral/genital ulcers. 38 Behcet s disease Classic Masqueraders Always keep these conditions in the differential of retinitis/vasculitis as they can mimic many different conditions Syphillis - The great masquerader Sarcoidosis

8 Sarcoidosis Tuberculosis Lymphoma - another great masquerader Unusual Vitreal Cells y.o with progressive floaters OS Fundus Photos

9 Fluorescein Angiogram OCT- OS Differential Diagnosis of Chronic Large Vitreous Cells Diagnosis: Whipple s disease 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 diagnose 51 Vitreous biopsy Blood/Vireous PCR positive for T. whippelli positive Light microscopy: Whipple s disease Chronic multisystem bacterial disease: weight loss, chronic diarrhea, vague abdomnal pain, migratory arthralgias 52 Snowbank Snowballs Pars Planitis Lyme Tuberculosis Syphillis Sarcoidosis Multiple sclerosis Toxocariasis Idiopathic Endogenous Fungal Infection

10 Vitreous Amyloidosis Metastatic Melanoma Glass wool like or cobweb sheets. Clear with vitrectomy 55 Gündüz, Shields, Shields, Eagle Ophthalmol 1998;105: Lymphoma Lymphoma Sheets of clumped cells in vitreous Ocular Lymphoma Ocular Lymphoma

11 Serous Detachments 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) CSR in pregnancy 8 months fibrin CSR in pregnancy Vogt-Koyanagi-Harada Syndrome Subretinal precipitates 1 month post partum

12 VKH bilateral uveitis, serous RD s, associated with cutaneous, auditory, and neurologic abnormalities. VKH 67 Punctate leaks on FA 68 Vogt-Koyanagi Syndrome (VKH) Vitiligo Vogt-Koyanagi Syndrome (VKH) Vogt-Koyanagi Syndrome (VKH)

13 Vogt-Koyanagi Syndrome (VKH) Optic Nerve Pit with Schisis Need steroids or immunosuppression. Rapid response, but careful to taper very slowly. Can follow EDI Choroidal thickness. Dex implant may help Optic Nerve Pit with Schisis Choroidal Lesions Management Laser, Vitrectomy/Laser, Retinal fenestration Takes a long time to see results year old healthy Asian-Indian male. Blurry spot OD, 20/25 OU. No cells

14 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 AMPPE AMPPE Multiple placoid lesions Often bilateral HLA B7 / DR2 Occasionally associated with cerebral vasculitis,, neurosensory hearing loss, erythema nodosum suggesting immune mediated vascular alterations 81 Usually resolves within 4-8 weeks, but reported to last up to 6 months Recurrences rare Vision loss with foveal involvement Steroids may 82 speed recovery Serpiginous Serpiginous Peripapillary; bilateral, progressive. Males more common. Recurrences can be months to years apart. CNVM can develop. Requires immunosuppressive therapy. Test for TB

15 Serpiginous Serpiginous Multifocal Choroiditis Diagnosis: Ampiginous/Relentless placoid choroiditis. Associated with TB Quantiferon gold testing. Patient quiescent after 9 months of TB rx. 87 Can be associated with subretinal fibrosis. CNVM can cause vision loss. Immunosuppressive Rx. 88 West Nile Virus Lyme Choroiditis

16 Pneumocystis Carinii choroiditis Ophthalmomysis 91 Courtesy of E. Holz, MD 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 94 Stay tuned for Part