Tropheryma whippleiCrystalline Keratopathy: Report of a Case and Updated Review of the Literature

Scott D Schoenberger,Sumeer Thinda,Stephen J Kim

doi:10.1155/2012/707898

Scott D Schoenberger, Sumeer Thinda + Show 1 more

Open Access

https://doi.org/10.1155/2012/707898

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Abstract

Purpose. To report a case of Tropheryma whipplei infection with crystalline keratopathy and review the recent literature on the presentation, diagnosis, and management of Whipple's disease. Methods. Detailed case presentation and extensive literature search of Pubmed for all years through February 2012 using the following search terms: Whipple's disease, Tropheryma whipplei, corneal deposits, crystalline keratopathy, and uveitis. Relevant articles were retrieved and analyzed. English abstracts were used for non-English articles. Cross-referencing was employed and reference lists from selected articles were used to identify additional pertinent articles. Results. Diagnosis of Whipple's disease remains challenging and untreated infection can result in mortality. Ocular signs and symptoms are usually nonspecific, but several independent cases have reported the presence of intraocular crystals or crystalline-like deposits. Conclusions. The presence of intraocular crystals or crystalline-like deposits may be an identifying feature of ocular Whipple's disease.

Highlights

Whipple’s disease is a chronic multiorgan bacterial infection due to Tropheryma whipplei with a variable clinical presentation and poor prognosis if untreated [1,2,3]
We report a patient who initially presented with vitritis and a striking crystalline keratopathy several years before the diagnosis of Whipple’s disease and his untimely death
The lymph node and aortic valve pathology blocks were sent to the Centers for Disease Control (CDC, Atlanta, Georgia) for review, and the presence of T. whipplei was confirmed by Polymerase chain reaction (PCR), immunohistochemistry, and PAS staining that showed periodic acid-Schiff- (PAS-)positive diastase-resistant organisms within macrophages (Figure 2)

Summary

Introduction

Whipple’s disease is a chronic multiorgan bacterial infection due to Tropheryma whipplei with a variable clinical presentation and poor prognosis if untreated [1,2,3]. Diagnosis is challenging and the mean time from symptom onset to diagnosis is 6.4 years [2]. We report a patient who initially presented with vitritis and a striking crystalline keratopathy several years before the diagnosis of Whipple’s disease and his untimely death. Our primary intent is to discuss the unique aspects of our case and review the available literature to determine identifiable ocular features of Whipple’s disease that may aid in diagnosis of future cases. Our secondary intent is to provide an updated review of the systemic and ocular manifestations of Whipple’s disease and recent advances in diagnosis and treatment

Report of a Case

Discussion

Findings

33 Female Subretinal