A UNIQUE POSTERIOR SEGMENT MANIFESTATION OF WHIPPLE DISEASE Lik Thai Lim, MRCSEd (Ophth),* Ramana Moorthy, MD,† Salah Najm, MD,‡ Robert P. Nelson, Jr, MD,† Karen M. Wolf, MD,† Tim Lahm, MD† Purpose: To report a novel posterior segment manifestation of Whipple disease. Method: Single observational case study. Results: The white crystalline deposit in the retina, choroiditis, and vitritis resolved with treatment of Whipple disease. Conclusion: Whipple disease can be associated with panuveitis, multifocal choroiditis, and white crystalline deposits in the anterior segments and retina, a unique clinical finding. RETINAL CASES & BRIEF REPORTS 6:204–205, 2012 From the *Tennent Institute of Ophthalmology, Glasgow, UK; †Indiana University School of Medi- cine, Indianapolis, Indiana; and ‡Case University Hospitals, Cleveland, Ohio. Case Report A 23-year-old white man who presented with dyspnea, diarrhea, malnutrition, anemia, thoracic and abdominal lymphadenopathy, and severe bilateral visual loss was diagnosed with Whipple disease (WD) based on jejunal biopsy (Figure 1). The presenting visual acuity was 20/125 in each eye. Ocular examination revealed bilateral panuveitis with significant vitreous opacities and inferior snowballs and multifocal choroiditis with disk and macular edema (Figures 2 and 3). The peripheral retinal examination revealed bilateral, super- otemporal, white, preretinal, or inner retinal crystalline deposits that were very different from the deeper areas of choroiditis and preretinal vitreous snowballs (Figure 4). Oral rifampin and trimethoprim/ sulfamethoxazole therapy was begun. The inflammation worsened in each eye. A diagnostic/therapeutic vitrectomy was performed in each eye 2 months apart. Cytopathologic evaluation of the vitreous revealed foamy macrophages and a mixed inflammatory reaction, which was confirmed on flow cytometry. Qualitative polymerase chain reaction of the vitreous confirmed Tropheryma whippelii. During vitrectomy, the crystalline deposits could not be vacuumed off the surface of the retina. Intravitreal vancomycin, amikacin, and clindamycin were injected at the conclusion of surgery. The patient’s vision improved dramatically after vitrectomy in each eye. Systemic antibiotic therapy was continued for a total of 12 months. The superior peripheral crystalline deposits disappeared on 1-year follow-up. Final visual acuity was 20/30 in each eye with complete resolution of ocular inflammation and systemic symptoms without medications. Discussion Whipple disease is a systemic infectious condition caused by an intracellular bacillus, T. whippelii, characterized by malabsorption causing chronic diarrhea in middle aged men (mean age of 49 years). Weight loss, lymphadenopathy, fever, pneumonia, migratory arthritis, and peripheral edema may occur. 1–4 Despite being relatively young, our patient presented with multiple organ system involvement consistent with WD. Although WD typically causes neuro-ophthalmic manifestations such as papilledema, papillary abnormalities, gaze palsies, and ophthalmo- plegia, uveitic manifestations of anterior uveitis, retinitis, retinal vasculitis, and vitritis can also occur. 1–4 The peripheral crystalline white deposits within the inner retina in our case is not inconsistent with findings of similar deposits in the anterior segment as previously reported by Avila et al, 1 Williams et al, 2 and Rickman et al. 4 Our case report demonstrate that these whitish opacities may also occur in the retina in WD, which has not been previously reported. Histopathologic evaluation of these white opacities has not been performed because of their tenacious adherence to underlying tissues such as the cornea, 4 lens capsule, 2 or retina. Rickman et al 4 first described the use of the 16s ribosomal DNA primers to identify T. whippelii in vitreous speci- men. Similar qualitative polymerase chain reaction The authors declare no conflict of interest. Reprint requests: Lik Thai Lim, MRCSEd (Ophth), Tennent Institute of Ophthalmology, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK; e-mail: likthai@ doctors.org.uk 204