The White Re)na Joseph Alsberge, MD January 20, 2018
58 y/o man with floaters and pain OD x 2 weeks PMH: oral and genital herpes Va OD 20/50 Anterior OD: KP and 3+ AC cell Posterior: Vitri)s, occlusive vasculi)s, and mul)- focal peripheral white necro)zing re)ni)s
Va OS 20/16 Anterior OS: Quiet Posterior: Quiet with no re)ni)s
Diagnosis?
The White Re)na Infec)ons Viral Herpes Simplex Herpes Varicella Zoster Cytomegalovirus Dengue Fever West Nile Virus Chikungunya Parasite Toxoplasmosis Bacterial Syphilis Bartonella TB Fungal Candida Aspergillus Immune Behcet s disease Iatrogenic Surgical trauma Air Infusion Trauma Commo)o re)nae Tumor Lymphoma Congenital Myelinated NFL White without pressure Ischemia
Varicella Zoster and Herpes Simplex Viruses Vaso- occlusive peripheral re)ni)s, vasculi)s, vitri)s (the acute re)nal necrosis syndrome) Classically occurs in healthy, immuno- competent, adults
Cytomegalovirus Re)ni)s with hemorrhage and satellite lesions Slowly progressive, occurs in severely immunocompromised pa)ents (AIDS, bone marrow transplant recipients)
Chikungunya Virus Mosquito- borne Alphavirus, causes an acute, poten)ally fatal, febrile illness, Most outbreaks occur in the tropics Chikungunya re)ni)s tends to occur in the posterior pole with minimal vitri)s
Dengue Virus Mosquito- borne Flavivirus, causes Dengue Fever : headache, myalgia, rash and other symptoms Has been associated with a wide range of ocular inflammatory disease, including re)ni)s Courtesy Prof. Khalid Tabbara
West Nile Virus Mosquito- borne Flavirvirus that can cause severe encephali)s Has been associated with posterior pole re)ni)s with hemorrhage Courtesy Prof. Rathinam Sivakumar
The world is heavily interconnected You never know when a pa)ent with a mosquito- borne re)ni)s may land in your office!
Toxoplasmosis gondii Focal, round, necro)zing re)ni)s in the posterior pole with associated vitri)s The so- called headlight in the fog appearance Raw meat consump)on (oysters, pork) and exposure to cat feces are risk factors for infec)on by this parasite
Toxoplasmosis gondii Another case of toxoplasmosis re)ni)s Acute focal re)ni)s in the posterior pole adjacent to an old pigmented scar This is highly sugges)ve of toxoplasmosis and indicates reac)vated disease
Syphilis Syphilis can cause essen)ally any form of ocular inflamma)on However, re)ni)s with overlying superficial precipitates highly sugges)ve Rates of this bacterial disease have been rising in recent 4 days years later no treatment
Bartonella henslae Gram- nega)ve bacillus that is endemic in cats Classically associated with neurore)ni)s (i.e., op)c disc edema and macular star), can also cause mul)focal re)ni)s
Candida albicans Fungus that can cause ocular infec)on via endogenous route For example, in hospitalized pa)ents with indwelling catheters Candida re)ni)s may be focal or mul)focal, and can break through into the vitreous to produce a vitreous abscess
Lymphoma Always consider lymphoma: The Great Masquerader Can mimic infec)ous necro)zing re)ni)s Failure to respond to an)microbial therapy is a clue, )ssue diagnosis is defini)ve
Back to our pa)ent 58 y/o man OD 20/50 Pain and vision loss OD x 2 weeks PMH: oral and genital herpes Anterior: KP and 3+ AC cell Vitri)s, occlusive vasculi)s, mul)- focal peripheral white re)ni)s
Diagnosis: Acute Re@nal Necrosis Syndrome due to VZV or HSV
Clinical findings Diagnos)c tes)ng Treatment Complica)ons Acute Re)nal Necrosis
Anterior segment findings Granulomatous large KP Stellate KP
ARN can present with concomitant scleri)s and elevated IOP
Posterior segment findings Peripheral necro)zing re)ni)s With non- perfusion and segmental arteriolar occlusion
Bilateral acute re)nal necrosis (BARN) Occurs in 1/3 rd of cases Onset of fellow eye typically within months Herpes virus likely progresses through the op)c chiasm from one eye to the other
PCR Tes)ng and Isolate Frequency Consider PCR tes)ng of ocular fluid (aqueous or vitreous) PCR highly sensi)ve and specific (95% and 97%, respec)vely) for herpesviridae VZV accounts for majority of cases of ARN However in children and young adults, it is more ooen HSV- 2
Treatment: systemic an)viral is mainstay Valacyclovir (Valtrex ) 1-2 gm PO TID Intravitreal an)viral agents (foscarnet or ganciclovir) op)onal Oral steroids op)onal Consider hospitaliza)on for IV an)viral therapy if non- compliance issues or evidence of systemic infec)on Resolving re)ni)s 4 weeks following ini)a)on of treatment
Treatment: systemic an)viral is mainstay Valacyclovir (Valtrex ) 1-2 gm PO TID Intravitreal an)viral agents (foscarnet or ganciclovir) op)onal Oral steroids op)onal Consider hospitaliza)on for IV an)viral therapy if non- compliance issues or evidence of systemic infec)on Re)ni)s resolved at 4 months follow up
However, not all cases respond to standard therapy Case report of ARN that progressed despite IV acyclovir and ganciclovir Switch to foscarnet lead to resolu)on of re)ni)s Gene)c analysis revealed mul)- drug resistant strain of HSV- 2 In cases that fail to respond to standard therapy, consider drug resistance
Another considera@on in cases that fail to respond to standard an@viral therapy Vitreore)nal lymphoma can masquerade as infec)ous re)ni)s to a striking degree
Rhegmatogenous re)nal detachment is a common complica)on of ARN As ac)ve infec)on resolves, breaks can develop in areas of necro)c re)na
Rhegmatogenous Re)nal Detachment in ARN RRD occurs in 50-75% of eyes with ARN (weeks to months aoer ini)al presenta)on)
Some have advocated early prophylac)c barrier laser Ra)onale: prevent progression to detachment should breaks occur
The White Re)na Infec)ons Viral Herpes Simplex Herpes Varicella Zoster Cytomegalovirus Dengue Fever West Nile Virus Chikungunya Parasite Toxoplasmosis Bacterial Syphilis Bartonella TB Fungal Candida Aspergillus Immune Behcet s disease Iatrogenic Surgical trauma Air Infusion Trauma Commo)o re)nae Tumor Lymphoma Congenital Myelinated NFL White without pressure Ischemia
Thank You