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Posterior polymorphous dystrophy (PPD): incidental diagnosis in a patient with herpes zoster keratouveitis 

Poster Details

First Author: R.Vera Echevarria SPAIN

Co Author(s):    L. Marinas Garcia   B. Garcia-Valcarcel   B. Monsalve              

Abstract Details

Purpose:

To present the case of a herpes zoster keratouveitis, where the examination leads to the diagnosis of Posterior Polymorphous Dystrophy (PPD). In addition, it shows the keratouveitis progression and secondary glaucoma associated.

Setting:

Cornea and Ocular Surface Section. Gregorio Marañon General University Hospital. Madrid. Spain.

Methods:

PPD is a rare congenital disorder with autosomal dominant inheritance. It affects endothelial cells and Descement’s membrane. Usually asymptomatic and with slow progression. Slit lamp examination shows characteristic endothelial bands, vesicles and opacities. The confocal microscopy is the gold standard diagnostic test. A 64 – years old male patient presenting with hypertensive anterior keratouveitis due to Herpes Zoster in the right eye (RE) treated with valaciclovir, dexamethasone eye drops and topical timolol 0,5%. The best-corrected visual acuity (BCVA) was 20/20. Slit lamp examination revealed irregular opacities of the posterior corneal surface in both eyes and keratic precipitates in RE.

Results:

Specular microscopy showed pleomorphims and polymegathism and the confocal microscopy revealed the diagnosis of PPD. No abnormalities in gonioscopy or disc cup were found, but elevated intraocular pressure (IOP) and alteration in visual fields were discover. Treatment with hypotensive eye drops was provided with no improvement. Non-penetrating deep sclerectomy was performed in RE. Subsequently disciform keratitis was developed, treated with valaciclovir and topical steroids resulting in prompt resolution of the episode. Posteriorly, a decrease on BCVA, elevated IOP, reduction in thickness of Retinal Nerve Fibers Layer (RNFL) and worsening of the visual fields occurred. Therefore, treatment with topical timolol 0,5% was initiated.

Conclusions:

PPD diagnosis could be delayed if there are no many signs on the slit lamp examination, leading to confusion with other corneal anomalies. 15% of cases may be associated with elevated IOP and gonioscopy findings such as iris processes, peripheral anterior sinechiae or prominent Schwalbe ring. A full ophthalmic assessment should be performed given the strong association between PPD and glaucoma. In this case, herpes zoster seemed the main cause of glaucoma and no correlation among these entities was found.

Financial Disclosure:

NONE

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