You are in: eMedicine Specialties > Ophthalmology > RETINA Acute Retinal NecrosisArticle Last Updated: Jun 22, 2006AUTHOR AND EDITOR INFORMATIONAuthor: Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine Andrew A Dahl is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society Coauthor(s): David T Wong, MD, FRCS(C), Associate Professor of Ophthalmology, Director of Fellowship Programs, Department of Ophthalmology, St Michael's Hospital, Faculty of Medicine, University of Toronto, Canada; Saad Waheeb, MB, BCh, FRCS(C), Consulting Staff, Department of Ophthalmology, King Abdulaziz University Hospital Editors: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences Author and Editor Disclosure Synonyms and related keywords: ARN, BARN, bilateral ARN, Kirisawa's uveitis INTRODUCTIONBackgroundAcute retinal necrosis was first described in the Japanese literature in 1971 and termed Kirisawa uveitis. During the past 2 decades, acute retinal necrosis syndrome (ARN) has been a source of fear, frustration, and fascination for many ophthalmologists. Unfortunately, it usually is a visually devastating condition for the patient. PathophysiologyARN may be a result of dormant viral reactivation in the retina. The exact etiology of this reactivation is still elusive; however, there is likely an immunogenetic predisposition to the disease. FrequencyUnited StatesARN accounts for 5.5% of uveitis cases over a 10-year period. InternationalIn Switzerland, ARN accounts for 1.7% of uveitic cases. Mortality/MorbiditySignificant visual loss may occur. Retinal detachment complicates most cases (64%) and is a major cause of legal blindness in ARN. RaceNo racial predilection exists. SexThis condition appears to have a predilection for males; however, it is not clear to what extent. AgeARN is a disease of young healthy individuals aged 20-50 years.
CLINICALHistoryTypically, this is a disease of immunocompetent individuals. Initially, patients may complain of the following:
Physical
CausesMost cases of ARN have been reported to be caused by the following:
DIFFERENTIALSBehcet Disease Endophthalmitis, Fungal HIV Ocular Manifestations of Syphilis Retinitis, CMV Sarcoidosis Toxoplasmosis
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| Drug Name | Acyclovir (Zovirax) |
|---|---|
| Description | Has affinity for viral thymidine kinase and once phosphorylated causes DNA chain termination when acted on by DNA polymerase. Patients experience less pain and faster resolution of cutaneous lesions when used within 48 h from rash onset. May prevent recurrent outbreaks. Early initiation of therapy is imperative. |
| Adult Dose | 1500 mg/m2/d IV divided tid for 7-10 d, followed by 800 mg PO 5 times/d for 14 wk to reduce risk of bilateral involvement |
| Pediatric Dose | Not established; discuss with pediatrician or infectious disease specialist |
| Contraindications | Documented hypersensitivity |
| Interactions | Concomitant use of probenecid or zidovudine prolongs half-life and increases CNS toxicity of acyclovir |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Caution in renal failure or when using nephrotoxic drugs |
| Drug Name | Ganciclovir (Cytovene, Vitrasert) |
|---|---|
| Description | Synthetic guanine derivative active against cytomegalovirus (CMV). An acyclic nucleoside analog of 2'-deoxyguanosine that inhibits replication of herpes viruses both in vitro and in vivo. Levels of ganciclovir-triphosphate are as much as 100-fold greater in CMV-infected cells than in uninfected cells, possibly due to preferential phosphorylation of ganciclovir in virus-infected cells. For patients who experience progression of CMV retinitis while receiving a maintenance treatment with either dosage form of ganciclovir, the re-induction regimen should be administered. |
| Adult Dose | 5 mg/kg IV q12h for 2 wk |
| Pediatric Dose | Not established; discuss with pediatrician or infectious disease specialist |
| Contraindications | Documented hypersensitivity; do not use if absolute neutrophil count <500 million cells/L or the platelet count is <25 x 109 cells/L |
| Interactions | Concomitant administration with cytotoxic drugs such as dapsone, vinblastine, Adriamycin, pentamidine, flucytosine, vincristine, amphotericin B, trimethoprim/sulfamethoxazole combinations, or other nucleoside analogs may result in additive toxicity in bone marrow, spermatogonia, and germinal layers of skin and GI mucosa (coadminister only if potential benefits outweigh risks); coadministration with imipenem-cilastatin may cause generalized seizures (use only if potential benefits outweigh risks); serum creatinine may increase following concurrent use of ganciclovir with either cyclosporine or amphotericin B; in presence of probenecid, ganciclovir renal clearance is reduced; bioavailability may increase when didanosine is administered either 2 h prior to or simultaneously with ganciclovir; bioavailability of ganciclovir may decrease in presence of zidovudine, while bioavailability of zidovudine is increased in presence of ganciclovir |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Clinical toxicity of ganciclovir includes granulocytopenia, anemia, and thrombocytopenia; since oral ganciclovir is associated with higher rate of CMV retinitis progression, compared to IV formulation, use only when benefits outweigh risks (advanced HIV disease); half-life and plasma/serum concentrations of ganciclovir may be increased as a result of reduced renal clearance; dosages > 6 mg/kg IV may result in increased toxicity; rapid infusions may result in increased toxicity; initially, reconstituted solutions of IV ganciclovir have a high pH (11); phlebitis or pain may occur at site of IV infusion despite further dilution in IV fluids; administration of ganciclovir should be accompanied by adequate hydration; photosensitization (photoallergy or phototoxicity) may occur |
Systemically interfere with events leading to inflammation.
| Drug Name | Prednisone (Deltasone, Orasone, Meticorten) |
|---|---|
| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
| Adult Dose | 0.5-1 mg/kg/d PO qd or divided bid for 8 wk Ophthalmic: 1 gtt q1h to qid |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections and fungal or tubercular skin infections; GI disease |
| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
Inhibit the cyclooxygenase system, decreasing the level of thromboxane A2, which is a potent platelet activator.
| Drug Name | Aspirin (Bayer Aspirin, Ascriptin, Anacin) |
|---|---|
| Description | Treats mild to moderate pain and headache. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. |
| Adult Dose | 325 mg PO qd |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, nasal polyps, asthma; due to association of aspirin with Reye syndrome, do not use in children ( <16 y) with flu |
| Interactions | Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose lowering effect of sulfonylurea drugs |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with history of blood coagulation defects, or taking anticoagulants |
| Media file 1: The white area is necrotic retina. | |
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| Media file 2: Severe vitritis with occlusive arteriolitis | |
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Acute Retinal Necrosis excerpt
Article Last Updated: Jun 22, 2006