Bilateral Cytomegalovirus Retinitis in the Absence of Viremia

Special Article - Clinical Cases and Images

Austin J Clin Ophthalmol. 2015;2(3): 1048.

Bilateral Cytomegalovirus Retinitis in the Absence of Viremia

Christopher T. Shah1 and Ashvini K. Reddy1*

1Department of Ophthalmology, University of Virginia, USA

*Corresponding author: Ashvini K. Reddy, Department of Ophthalmology, University of Virginia, 1300 Jefferson Park Avenue, OMS 2860B, Charlottesville, VA 22908,

Received: March 17, 2015; Accepted: April 27, 2015; Published: April 28, 2015

Abstract

Purpose: To report a case of Polymerase Chain Reaction (PCR) Confirmed Bilateral Cytomegalovirus (CMV) retinitis in a patient with mantle cell lymphoma with no detectable CMV viremia.

Methods: Case report.

Patients/Results: A 67 year-old female with mantle cell lymphoma requiring multiple chemotherapeutic regimens and history of previously treated ganciclovir-resistant CMV colitis and viremia presented with bilateral necrotizing retinitis and panuveitis 3 months after her foscarnet was discontinued for undetectable viral loads. Her aqueous was positive for CMV by PCR; however, blood samples for CMV viral loads were negative on presentation as well as 3 months prior by serum PCR. She was treated with intravitreal and intravenous foscarnet and topical steroids with improvement initially, but ultimately developed a retinal detachment OD and severe macular edema with nonperfusion OS as well as bilateral uveitic glaucoma.

Conclusion: CMV retinitis can occur in the absence of contemporaneous CMV viremia. This report demonstrates the utility of intraocular sampling for PCR in such cases and suggests that, especially in the chronically ill, physicians should maintain a high index of suspicion given the necessity for urgent intervention.

Keywords: Cytomegalovirus; Retinitis

Introduction

Cytomegalovirus (CMV) retinitis is typically only described in chronically ill, immunocompromised patients in association with systemic viremia or other organ involvement [1,2]. We report a rare case of bilateral CMV retinitis in the absence of contemporaneous CMV viremia or other active manifestations of CMV.

Case Presentation

An HIV-negative, 67 year-old female with Mantle Cell Lymphoma (MCL) developed treatment-related immunosuppression following a course of Bendamustine and Rituximab. She subsequently developed CMV viremia and colitis; however, dilated fundus exam at that time by our service was negative for CMV retinitis and uveitis. Past ocular history was unremarkable without history of periocular or intravitreal steroid injections and was only notable for a remote history of a horseshoe tear OS treated with cryotherapy 20 years prior.

Her course was marked by persistent CMV viremia despite adequate valganciclovir therapy and she was ultimately found to have a UL97 mutation conferring resistance to ganciclovir but susceptibility to foscarnet. She was treated with a three month course of foscarnet, with which her viremia and colitis resolved. CMV viral loads by serum PCR were undetectable during her 3 month treatment course.

One month after starting the foscarnet, she experienced an episode of metamorphopsia which prompted presentation to her primary ophthalmologist, who saw no retinal changes at that time.

One week following completion of Foscarnet therapy, she developed flashes and floaters OU with decreased vision OD and preserved vision OS. Viral loads around this time were undetectable. Her vision continued to decline, and she again sought the care of her primary ophthalmologist. Intraocular Pressures (IOP) were elevated bilaterally and she had suspicious retinal lesions precipitating referral to a retina specialist, who referred the patient to our center.

At this time, she had no oral lesions or symptoms of CMV colitis; however, there was severe retinitis. The patient was admitted and started on empiric treatment with intravenous foscarnet. Admission labs were notable for white blood cell count of 3.8 k/microL, an undetectable CMV viral load in the blood, and a CD4 count of 171 per microL. Examination was remarkable for BCVA of 20/60 OU, IOP of 31 OD and 28 OS, and an afferent pupillary defect OD. Slit lamp examination revealed moderate fine keratic precipitates, 2+ cell, 1+ flare, with iris rubeosis and iris hemorrhages OU. Fundus examination was remarkable for severe peripheral retinal vasculitis and circumferential retinal atrophy (Figure 1) consistent with CMV chronic retinal necrosis [2]. Fluorescein angiography confirmed 360-degree retinal vascular occlusion in both eyes (Figure 1). She underwent anterior chamber paracentesis for viral PCR and intravitreal injection of foscarnet OD and remained admitted for continued intravenous foscarnet and serial dilated exams. Anterior chamber PCR was positive for CMV and negative for herpes simplex virus, varicella zoster virus, and toxoplasmosis. She was eventually discharged with a prolonged course of intravenous foscarnet and monitored with monthly CMV viral loads. All viral loads have been undetectable as of 5 months follow-up.

Citation: Shah CT and Reddy AK. Bilateral Cytomegalovirus Retinitis in the Absence of Viremia. Austin J Clin Ophthalmol. 2015;2(3): 1048. ISSN : 2381-9162