Not all that glitters is iron: Anterior segment optical coherence tomography in an unusual pseudoexfoliation syndrome

Pseudoexfoliation (PXS) syndrome is characterized by the deposition of abnormal fibrillar material on various anterior segment structures, including the corneal endothelium. Several studies have also documented an association between PXS and endothelial guttae.[1] We report a case of a 40-year-old patient who presented with a single, isolated, pigmented, foreign-body-like deposit on the corneal endothelium of the right eye. The patient had previously undergone Yttrium Aluminum Garnet (YAG) peripheral iridotomy in both eyes for occludable angles. On review of prior records, the endothelial pigment deposit was noted to be present even before the laser procedure. The patient had consulted multiple centers, where it was repeatedly diagnosed as an endothelial foreign body. Gonioscopy revealed features suggestive of PXS syndrome, including increased trabecular pigmentation and pseudoexfoliative material at the angle. There was no history of ocular trauma. Slit-lamp examination showed no epithelial or anterior stromal opacification to suggest an entry wound. Anterior segment optical coherence tomography (AS-OCT; Cirrus HD-OCT) demonstrated a well-defined hyperreflective lesion at the level of the endothelium without posterior shadowing, favoring pigment deposition rather than a metallic foreign body. Ringvold[2] has described the presence of phagocytosed melanin granules and intracellular vacuoles within corneal endothelial cells in eyes with PXS, which may explain the appearance of the pigmented endothelial deposit in this case. Pigment deposition on anterior segment structures is well recognized in pigment dispersion syndrome (PDS), where it typically shows a characteristic pattern such as a vertical Krukenberg spindle on the corneal endothelium and dense trabecular meshwork pigmentation [Figs. 1-4].[3] In contrast, the pigment deposition in the present case was focal, isolated, and associated with other features of PXS syndrome, supporting PXS rather than PDS as the underlying pathology.Figure 1: Oblique illumination of the cornea showing endothelial foreign body, surrounding stroma and epithelium appears intact without opacification (marked in white circle)Figure 2: Diffuse illumination showing pseudo exfoliative material (red arrow) and peripheral iridotomy at 2 o’clock (black arrow)Figure 3: Retro illumination showing pigment on endothelium (marked in white circle)Figure 4: Image showing left eye hyperreflective material on the endothelium, without back shadowing, suggestive of pigment like deposit on the endothelium (marked in white circle)Although removal of the deposit during cataract surgery, especially in the presence of a brown cataract, with subsequent histopathological examination could have provided definitive confirmation, such an approach was deferred since it was asymptomatic, and intervention carried a potential risk of endothelial cell loss. Additionally, histopathological and ultrastructural analysis may not always be feasible due to cost and availability constraints. This case underscores the diagnostic utility of AS-OCT in differentiating endothelial pigment deposition associated with PXS syndrome from true intracorneal foreign bodies. Awareness of such atypical presentations is important, particularly in eyes with PXS, where baseline endothelial compromise may already exist, and warrants caution during intraocular surgery.[4] Authors’ contributions Kalluri S Srividya: Manuscript writing, review of literature, concept, design, Siddharam S Janti : Manuscript editing, Preeti T, Sathwik R Nomula: Review of literature, editing. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.