Post-phacoemulsification cytomegalovirus corneal endotheliitis diagnosis and management

A 68-year-old British-Chinese gentleman with left ocular hypertension was referred to the inflammatory eye diseases clinic with a presumed left keratouveitis that developed six weeks after uneventful phacoemulsification surgerywith intraocular lens implantation. This had failed to resolve with oc. aciclovir 3% and intensive g. dexamethasone 0.1%. His left vision was 20/60, associated with dense corneal stromal edema (Figure 1a), keratic precipitates (KPs) (Figure 1b), mild anterior chamber inflammation, andan intraocularpressure (IOP) of 23mmHg. The posterior segment appeared healthywith no evidence of vitritis, choroiditis or retinitis, and a normal optic disc. An infectious etiology was suspected and a diagnostic multiplex quantitative polymerase chain reaction (qPCR) analysis of an aqueous humor (AqH) sample taken at the slit-lamp confirmed left corneal endotheliitis secondary to cytomegalovirus (CMV) (CMV, 150,000 DNA copies/ml; Epstein-Barr virus [EBV], weakly positive; and negative for herpes simplex virus, varicella zoster virus, fungal 18S-rRNA, and bacterial-16S rRNA). EBV DNAwas also detected in whole blood. The patient was commenced on oral valganciclovir 900 mg b.i.d. for 21 days followed by 450 mg b.i.d. and non-preserved g. dexamethasone 0.1% qid for three months. Initial improvement inhis clinical conditionwas supported by repeat whole blood and AqH qPCR detecting only EBV DNA (whole-blood 3,700 copies/ml, AqH 17,600 copies/ml). Although 95% of adults worldwide are believed to have serological evidence of EBV infection, intraocular EBV is strongly associated with central nervous system lymphoma in AIDS. A haematological opinion was thus sought and excluded systemic lymphoma. Interruption of valganciclovir treatment (due to non-availability in the community), resulted in an acute re-presentation with a severely painful left eye and reduced vision of hand movements at 30 cm. Examination revealed inferior haemorrhagic anterior scleritis (Figure 1c), a bullous keratopathy, and inferior KPs consistent with a diagnosis of recurrent CMV endotheliitis and associated scleritis, confirmed by qPCR of the AqH detecting CMV DNA (1800 copies/ml). IOP was 28 mmHg and fundal examination was normal. The patient was commenced on intravenous ganciclovir 500 mg once daily for 14 days switching to oral valganciclovir 900 mg b.i.d. for 21 days followed by a maintenance dose of 900 mg once daily, flurbiprofen 100 mg thrice daily for one month and intensive non-preserved g.dexamethasone 0.1%. Within one week there were significant improvements in the endotheliitis and scleritis, and by four weeks, only mild corneal decompensation and endothelial pigmentation remained (ventral aortic 20/30). Oral valganciclovir was discontinued after six months following a negative AqH qPCR for CMV DNA. Nevertheless, after continued progression of corneal endothelial dysfunction (endothelial DECLARATIONS