European Review for Medical and Pharmacological Sciences 2012; 16(4 Suppl): 84-89 Ocular and : five case reports

F. KIANERSI, A. NADERI BENI*, H. GHANBARI, F. FAZEL

Department of , Isfahan University of Medical Sciences and Health Services, Isfa- han, Iran and *Department of Ophthalmology, Shahrekord University of Medical Sciences an Health Services, Shahrekord, Iran

Abstract. – BACKGROUND: Ocular toxoplas- This disease typically affects the posterior pole mosis is a potentially blinding cause of posterior of a single eye and the lesions can be solitary, . Retinal detachment is rare complication multiple or satellite to a pigmented retinal scar be of ocular toxoplasmosis. present9-10. In addition, the head can AIM: To report the clinical course and prog- 11 nosis of retinal breaks and detachments occur- also be involved in ocular toxoplasmosis . ring in patients with ocular toxoplasmosis. The frequency and visual impact of retinal de- PATIENTS AND METHODS: This study was a tachment (RD) in ocular toxoplasmosis (OT) are retrospective, non-comparative case series of not well defined.In this study, clinical records of five patients with ocular toxoplasmosis who had five patients with retinal detachment after ocular consulted us with retinal detachment. toxoplasmosis were reviewed. RESULTS: All of the participants had retinal detachment after severe and treatment resistant toxoplasmic retinochoroiditis, leaving one of them with decreased visual acuity to light per- Patients and Methods ception in spite of treatment and final visual acuity was 20/100 or better in four patients. We reviewed 1000 charts of patients followed CONCLUSIONS: The functional prognosis for at the Ocular Immunology and Uveitis Service the patients with retinal detachment was poor. of the Feiz Eye Hospital from 2003 to 2009 in- Careful retinal examination in ocular toxoplas- mosis is warranted, especially in patients with clusively. 193 of patients had toxoplasmosis severe intraocular inflammation. uveitis (82 males and 111 females). Among 193 patients 5 patients had retinal detachment. Clin- Key Words: ical records of five patients with ocular toxo- Ocular toxoplasmosis, Retinal detachment. plasmosis and retinal detachment were reviewed for details of clinical presentation, ophthalmic history, complications and visual outcome at fi- nal follow up. Introduction

Toxoplasmosis is the most common cause of Case Reports posterior uveitis in the world. The seroprevalence of Toxoplasma gondii is different throughout the Case 1 world. It is caused by the obligate intracellular A 29-year-old man presented to our Depart- protozoan Toxoplasma gondii1 and infection is ment with the complaint of decreased central vi- widespread in nature2. sion in his right eye for 3 weeks and redness, pho- The cause of toxoplasmic retinochoroiditis tophobia and pain from 1 week ago. He reported may be a recurrence of congenital toxoplasmosis. no underlying systemic disease or recent health However, acquired ocular disease is more com- change or medication and no family history of mon3-7. More than 82% of congenitally infected unusual . On examination, his visual individuals not treated as infants will develop acuity was 20/60 in his right eye and 20/20 in his retinal lesions by the time they reach the adoles- left eye. Intraocular pressure (IOP) was 10 mm cence8. It is estimated that about two-thirds of Hg in his right eye and 12 mm Hg in his left eye. patients with toxoplasmosis have recurrent There was +1 reaction of anterior chamber and 2+ episodes of inflammation3. anterior vitreal cells (with more cells in the poste-

84 Corresponding Author: Afsaneh Naderi Beni, MD; e-mail: [email protected] Ocular toxoplasmosis and retinal detachment: five case reports rior vitreous) with some of right the left eye. Dilated examination of the fundus re- eye. Dilated fundoscopic examination revealed a vealed 3 + cells in the vitreous and funduscopy 2 disc diameter (DD), elevated, creamy retinal le- showed a diffuse, elevated white lesion in the left sion in the superior nasal right fundus adjacent to 1 DD in size located half a DD temporal to a darker area in right eye. His chest x-ray was the fovea. A focal condensation of inflammatory normal. In addition, fluorescent treponemal anti- cells within the vitreous was seen overlying the body, complete blood cell count, C-reactive pro- area of active . Toxoplasma IgG titer tein, erythrocyte sedimentation rate, Lyme and was 3.7 (reference range, 0 to 0.92), indicating pri- Bartonella assays were negative. The purified or exposure to toxoplasmosis. On the follow-up (2 protein derivative (tuberculin) was negative. How- weeks) visit the patient’s visual acuity in the affect- ever, Toxoplasma IgM and IgG titers were re- ed eye had decreased to 20/300. In spite of this markable only for an increased toxoplasma IgG therapy the patient developed retinal detachment titer: 3.6 (reference range, 0 to 0.92), indicating after 2 weeks of therapy in temporal and infratem- prior exposure to toxoplasmosis. poral. A horseshoe tear was developed in the pe- The Toxoplasma IgG results combined with riphery of temporal retina. The retinal detachment our patient’s history led us to suspect ocular tox- treated with scleral buckeling and retina was at- oplasmosis. Although the diagnosis was not de- tached. She had no reactivation of toxoplasmosis in finitive, our suspicion was strong enough to merit the 4 years follow up. immediate therapy. He was started on standard regime of toxoplasmosis (oral regimen of sulfadi- Case 3 azine 500 mg qid and pyrimethamine 50 mg and A 37-year-old Iranian man was referred for after 1 day, 20 mg of prednisone). In addition, he treatment of decreased visual acuity and 1-week was prescribed topical glucocorticoids, folinic duration of pain of his right eye to our Ocular Im- acid, and mydriatic agents. After six months the munology and Uveitis Service. The patient did patient returned to our Service with decrease of not have any significant past medical history or visual acuity. On examination, his best corrected allergies, and was on no systemic medications. visual acuity was poor light perception in right He was using topical prednisolone acetate 1% eye and failed to improve with glasses or a pin- suspension hourly and scopolamine 0.25% solu- hole aperture. The IOP were –12.2 and 17.3 mm tion twice daily in his right eye. His visual acuity of Hg in the right and left eyes respectively. Ex- was 20/400 in the right eye and 20/20 in the left amination revealed a dense relative afferent eye. Slit-lamp examination revealed keratic pre- pupillary defect (RAPD) in the right eye and pos- cipitates on the corneal endothelium, 3+ cells in terior segment could not be visualized due to the the anterior chamber and pigment deposits on the mature formation. On B-scan posterior anterior capsule of the of the right eye. Dilat- segment was seen with total retinal detachments ed fundus examination showed an active toxo- in a closed funnel configuration. A decision was plasmic lesion infero-temporal to the macula, and made to attempt rehabilitation of one eye. After 3+ vitreous cells. Toxoplasma IgG titer was 3.1 lensectomy and were performed on (reference range, 0 to 0.92), indicating prior expo- the right eye, uncorrectable retinal fibrosis with a sure to toxoplasmosis. The patient was begun on total closed funnel traction retinal detachment toxoplasmosis treatment. One month after recov- with shortening and fibrosis were noted. ery the patient presented with worsening visual acuity in the affected eye (light perception with Case 2 correct light projection) due to acutely sympto- A 16-year-old Iranian woman, presented to the matic RD in his right eye. The retina was de- Ocular Uveitis Service of the Feiz Eye Clinic with tached from 1 to 5 o’clock because a peripheral complaints of a decreased visual acuity and red- U-shaped tear at 1 o’clock. The macula was at- ness in her left eye of 5-day duration and an tached and corrected visual acuity was 20/25 in episode of intense pain in the left eye 2 weeks prior the right eye and 20/20 in the left. The RD was to the visit. The pain persisted for 1 week. She did repaired on the day of presentation under general not have any allergies. Her past medical history anaesthetic. Subretinal fluid was drained, was unremarkable. The patient’s visual acuity was cryotherapy was applied, and 20% sulfur hexaflu- 20/20 in the right eye and 20/50 in the left eye. oride gas was injected. A 276 segmental circum- There were keratic precipitates on the corneal en- ferential silicone explant was then sutured to the dothelium, and 3 + cells in the anterior chamber of in the supero-nasal quadrant. Early postop-

85 F. Kianersi, A. Naderi Beni, H. Ghanbari, F. Fazel erative progress was satisfactory, with a flat retina week. The patient was not respond to our treat- and high indent. After 8 weeks the patient was ment. On B-scan posterior segment was seen with presented with worsening of visual acuity in right retinal detachment. We considered deep vitrecto- eye. In examination a new and peripheral RD was my. A diffuse, elevated white lesion in the right found infero-temporally in the right eye due to retina 4 disc diameter in size located superior to the peripheral U-shaped tear at the 7 o’clock position, fovea with tractional retinal detachment was seen. 6 clock hours away from the sealed original tear There was an area of associated perivascular adjusted to the old toxoplasmosis scar. A three- sheathing. An active right retinochoroiditis with as- port pars plana vitrectomy was carried out, with sociated retinal vasculitis was diagnosed. The pa- internal drainage of subretinal fluid, 30% sulfur tient underwent right deep vitrectomy with silicone hexafluoride exchange, and indirect laser photo- oil tamponade and 360° endophotocoagulation. coagulation around the tear. The retina has since The patient had undergone an evaluation that remained attached in 3 years follow up and the disclosed an antitoxoplasmic IgG antibody level ocular toxoplasmosis does not recurrent. of 2.2 ELISA units (normal range 0-0.9). Case 4 A 27-year-old Iranian man was referred for Results treatment of decreased visual acuity of his left eye of 1-week duration to Ocular Immunology We have described five patients with retinal and Uveitis Service. His visual acuity was 20/20 detachment and ocular toxoplasmosis. The clini- in the right eye and 20/50 in the left eye. The an- cal features are summarized in Table I. All pa- terior segments were normal and the intraocular tients were Iranian. All participants had a severe pressures were 16 mm Hg right eye, 14 mm Hg inflammation. One patient had a prolonged left eye. Funduscopy showed a diffuse, elevated course. All patients had permanent decrease in white lesion in the left retina 2-3 disc diameter in their best-corrected visual acuity. One patient had size located in peripheral of disc. The vitreous of a decrease in visual acuity to light perception in his left eye had 3+ anterior vitreal cells (with the affected eye due to uncorrectable retinal fi- more cells in the posterior vitreous). Toxoplasma brosis with a total closed funnel traction retinal IgG titer was 3.00 (reference range, 0 to 0.92), detachment with shortening and fibrosis. Inter- indicating prior exposure to toxoplasmosis. The estingly, in one case the retina has since re- patient was begun on standard regime of toxo- mained attached in 3 years follow up with +1 to plasmosis treatment. After 3 weeks the patient +2 vitritis with the active scar. presented with worsening visual acuity in the af- fected eye (light perception with correct light projection) due to acutely symptomatic RD in his Discussion left eye due to U-shaped tear. The RD was re- paired with scleral buckeling and cryopexy. The In this study, we identified 5 patients for reti- retina has since remained attached in 3 years fol- nal detachment among 193 patients with ocular low up with +1 to +2 vitritis with the active scar. toxoplasmosis. The occurrence of retinal detach- ment (RD) and retinal breaks (RBs) was signifi- Case 5 cantly associated with severe attacks of ocular A 26-year-old Iranian man was referred to our toxoplasmosis (OT) and preceding (diagnostic) Department for an acute decrease in his right-eye vitrectomy. vision, persisting for 4 weeks, after several days of Rhegmatogenous retinal detachment occurs . His best-corrected visual acuity when retinal break (RB) allows liquid vitreous was 20/400 right eye and 20/20 in left eye. Slit- access to subretinal space. Incidence is about 1 in lamp examination revealed motton fat keratic pre- 10,000/year; retinal breaks can be found in 97% cipitates on the corneal endothelium, 3+ cells in of cases. Most tears are located superiorly be- the anterior chamber and 330 degree of posterior tween 10 and 2 o’clock positions. Risk factors synechiae and pigment deposits on the anterior are age, history of retinal detachment in the fel- capsule of the lens of the right eye. The posterior low eye (15%), high /axial length (7%), segment could not be visualized. The left fundus family history, lattice degeneration, trauma, was normal. The patient was begun on oral and cataract surgery, diabetes, and Nd:YAG laser topical steroid and topical and mydriatic for one posterior capsulotomy12. The frequency and visu-

86 Ocular toxoplasmosis and retinal detachment: five case reports scleral buckling silicone oil threatening lesion alongthe superonasal arcade supero-temporal the macula to infro-temporal to the macula peripheral to disc threatening lesion superior to fovea Characteristic Case 1 Case 2 Case 3 Case 4 Case 5 Brief summary of the course of ocular toxoplasmosis in five patients. Brief summary of the course ocular toxoplasmosis in five retinal detachment preceding RD/RB Complications Atrophic eye None Recurrence of RD None None Visual acuity at flare-upVisual Duration of inflammation to Final visual acuity 6 months of RDType 20/60Location of retinal break attack vitreitis during OT Severe Yes attackSystemic treatment of OT Light perception TractionalPreceding RD/RB Antiparasitics with RD/RBTreatment 2 weeks Unknown 20/50 20/40 Antiparasitics with upper quadrant Temporal Yes 1 lensectomy Vitrectomy Upper nasal quadrant 1 month Antiparasitics with Scleral buckling Rhegmatogenous quadrant Temporal 20/400 Antiparasitics with corticosteroids 20/100 Tractional Rhegmatogenous Cryopexia, None 3 weeks Yes corticosteroids Rhegmatogenous 20/50 20/30 Scleral buckling Tractional 4 weeks corticosteroids 1 Vitrectomy Yes 20/400 20/100 Yes Age (years)Sex of involvement eye/area Affected Right eye/macula- Left eye/ 29 Male Right eye/ 16 Left eye/ Female Right eye/macula- 37 Male 27 Male Male 26 Table I. Table RB = retinal break; RD = retinal detachment; OT = ocular toxoplasmosis. RB = retinal break; RD detachment; OT

87 F. Kianersi, A. Naderi Beni, H. Ghanbari, F. Fazel al impact of RD in OT are not well defined. Or- It is feasible that it is not the corticosteroids ganization of vitreous infiltrates may lead to trac- themselves but the severe that may be re- tional detachment. A rhegmatogenous detach- lated to the development of RD or RB. Incidence ment can start from a retinal hole at the site of a of rhegmatogenous RD increases above the age former toxoplasmosis scar where the adhesion to of 50 years. However, patients with myopia over the pigment epithelium and sclera was strongest. 8 Diopters tend to develop RDs when they are Bodanowitz et al13 reported retinal tear in retini- young20. tis associated with toxoplasmosis. Retinal tears or The mean age of our patients with RD and rhegmatogenous retinal detachment are rare com- RBs was 32 years, but none of these patients had plications of toxoplasmic retinochoroiditis. How- myopia exceeding 4 Diopters. ever, a tear may occur due to vitreoretinal traction Most breaks in our patients were located in the following post inflammatory structural alteration superior quadrants. This is similar to the cases of of the vitreous. RBs in patients with OT reported earlier and to It is noteworthy that the attacks of active OT the location of retinal breaks in retinal detach- preceding the manifestation of RD or RB were ment in a general population14,21. in most patients, characterized by severe intraoc- However, Byer18 found 61% of asymptomatic ular inflammation. Fulminant vitritis preceding retinal breaks to be located in the inferior quad- RD in OT was also described in previous case re- rants. Visual prognosis of RD complicating OT ports14-15. Lafaul et al16 suggested that the pres- was poor, because half of the patients who expe- ence of peripheral hypertrophy of the chorioreti- rienced RD became legally blind (visual acuity nal scar was a sign of congenital toxoplasmosis ≤20/200). The patients with attached RBs re- with choroidal vascularization. Proliferate protru- tained their visual potential. This may be because sion of the sensory retinal layer and a secondary these RBs were diagnosed during ophthalmolog- scar caused by congenital toxoplasmosis might ic examinations needed for an active attack of OT be retinal tear in active ocular toxoplasmosis ini- and were, therefore, treated early. It was reported tiated with severe intraocular inflammation. that in noninflamed eyes, asymptomatic breaks Such a severe inflammation of the vitreous may did not proceed toward retinal detachment22. We lead to vitreous traction causing not only the trac- believe that the laser treatment of (asymptomatic) tional but also a rhegmatogenous retinal detach- attached RB in an eye with recurrent intraocular ment. Therefore, one would expect that the vitrec- inflammation is justified. First, there is an addi- tomy may protect severely inflamed eyes against tional risk factor consisting of vitreous traction, the development of RD. These interventions may and second, the visual prognosis after surgical re- also have contributed to the later development of a pair for RD in OT was poor. detachment. Although retinal detachment can oc- We have not an absolute explanation for the cur as a complication of pars plana vitrectomy, it is high frequency of RD after severe attacks of OT. to be expected that in most cases this may occur We believe that this high frequency and poor vi- shortly after the vitrectomy has been performed. sual prognosis warrant the careful retinal exami- Ocular toxoplasmosis therapy includes antimi- nation during active OT, especially in patients crobial drugs with or without the presence of corti- with additional risk factors for RD such as my- costeroids. Several drugs have been proposed in- opia and severe intraocular inflammation. cluding pyrimethamine, sulfadiazine, spiramycin, In summary, the timing of toxoplasma infec- clindamycin and trimethoprim-sulfamethoxazol17,18. tion leading to ocular disease is rarely known. The use of corticosteroids in OT is controver- However, current evidence suggests that many sial; these drugs are mainly used to alleviate se- more people are affected by postnatal than by vere inflammatory reaction. Therefore, corticos- prenatal toxoplasmosis. This has major public teroids may influence the development of vitreo- health implications. Considerable expertise and retinal traction and preretinal membranes. expense is concentrated on screening and health Four patients with OT who had RD or RB information to reduce the risks of toxoplasmosis used systemic corticosteroids with antiparasitics due to prenatally acquired infection, principally agents for the treatment of OT before the onset of to reduce the risks of ocular morbidity in the long RD. monotherapy may induce ex- term. Primary preventive strategies should in- tremely severe inflammation resulting in fulmi- clude children and adults at risk of ocular disease nant ocular disease; its use is, therefore, not rec- as a result of postnatal infection and should not ommended19. be confined to pregnant women.

88 Ocular toxoplasmosis and retinal detachment: five case reports

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