DJ Forster

N Kouda

NA Rao

NY Far

RH Roe

RW Read

S Attia

Y Usui

Journal of Ophthalmic Inflammation and Infection

English

Sana Khochtali

Nesrine Abroug

Mohamed Salah Hani

Imen Khairallah-Ksiaa

Bechir Jelliti

Moncef Khairallah

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Khochtali et al. Journal of Ophthalmic Inflammation and Infection  (2015) 5:27 DOI 10.1186/s12348-015-0057-9BRIEF REPORT Open AccessConcurrent acute Vogt-Koyanagi-Haradadisease in one eye and chronic disease inthe fellow eyeSana Khochtali, Nesrine Abroug, Mohamed Salah Hani, Imen Khairallah-Ksiaa, Bechir Jelliti and Moncef Khairallah*AbstractBackground: Vogt-Koyanagi-Harada (VKH) disease is a granulomatous panuveitis, usually involving both eyes at thesame time or within a few days or weeks. Acute and chronic diseases are characterized by distinct clinical features,treatment modalities, and visual outcomes. We report an atypical case of probable VKH disease, with features ofacute disease in one eye and chronic disease in the fellow eye.Findings: A 53-year-old female presented with exudative retinal detachment associated with mild vitritis in theright eye and anterior uveitis, vitritis, and sunset-glow fundus in the left eye. Based on clinical findings and resultsof multimodal imaging including fundus photography, spectral-domain optical coherence tomography, fluoresceinangiography, indocyanine green angiography, and B-scan ultrasonography, a diagnosis of acute VKH disease in theright eye and chronic VKH disease in the left eye was made. The patient received systemic corticosteroids andmycophenolate mofetil. After a 15-month follow-up, the right fundus eye was normal, and there was a sunset-glowfundus in the left eye.Conclusions: VKH disease may begin with asymptomatic unilateral ocular involvement. The patient may presentonly when the fellow eye is affected. A significant delay before involvement of the second eye leads to atypicalfeatures of acute disease in one eye and chronic disease in the other eye.Keywords: Vogt-Koyanagi-Harada disease; Exudative retinal detachment; Sunset-glow fundusFindingsIntroductionVogt-Koyanagi-Harada (VKH) disease is often defined asa bilateral, chronic granulomatous panuveitis associatedwith central nervous system, auditory, and integumentarymanifestations. The diagnosis is established on the basis ofthe Revised Diagnostic Criteria for VKH disease [1]. Thedisease is bilateral and naturally evolves through fourconsecutive stages. The prodromal stage including neuro-logical and auditory manifestations is followed after fewdays by the acute uveitic stage. Acute VKH disease typic-ally presents in the form of localized or multifocal exuda-tive retinal detachment (ERD) associated with intraocularinflammatory reaction. It usually affects both eyes at thesame time. However, onset may be unilateral, and the sec-ond eye may become involved 2 to 4 weeks later [1–3].* Correspondence: moncef.khairallah@rns.tnDepartment of Ophthalmology, Fattouma Bourguiba University Hospital,Faculty of Medicine, University of Monastir, Monastir, Tunisia© 2015 Khochtali et al. Open Access This artiInternational License (http://creativecommons.oreproduction in any medium, provided you givthe Creative Commons license, and indicate ifThe chronic convalescent stage occurs several weeks afterthe acute stage and is characterized by the development ofocular depigmentation, the sunset-glow fundus being themost specific feature, associated or not with integumentarydepigmentation. The chronic recurrent stage usually mani-fests with granulomatous anterior uveitis and is associatedwith higher risks of complications and vision loss [2, 3].We report herein an atypical case of probable VKH dis-ease with concurrent findings of acute disease in one eyeand chronic disease in the fellow eye, at first presentation.Case reportA 53-year-old female with a history of arterial hyperten-sion presented to our department, with bilateral visionblurring for 5 days. She reported no previous ocularcomplaints or neurological or auditory symptoms.Best corrected visual acuity (BCVA) was 20/32 in theright eye (RE) and 20/100 in the left eye (LE). Slit-lampexamination showed no anterior chamber inflammationcle is distributed under the terms of the Creative Commons Attribution 4.0rg/licenses/by/4.0/), which permits unrestricted use, distribution, ande appropriate credit to the original author(s) and the source, provide a link tochanges were made.Khochtali et al. Journal of Ophthalmic Inflammation and Infection  (2015) 5:27 Page 2 of 4in the RE, but noted few keratic precipitates, +1 cellsin the anterior chamber, and localized posterior syne-chiae in the LE. Ocular pressure was 14 mmHg inboth eyes. There were +1 vitreous cells in both eyesand 2+ vitreous haze in the LE. Fundus examinationrevealed optic disc hyperemia and a shallow ERD inthe posterior pole in the RE and diffuse depigmen-tation producing a sunset-glow appearance with somepigmentary changes in the LE (Fig. 1).Spectral-domain optical coherence tomography(SD-OCT) showed ERD with a few subretinal septaand undulations of the retinal pigment epithelium(RPE) in the RE. Findings were unremarkable in theLE (Fig. 1).Fluorescein angiography findings included multifocaldelayed choroidal perfusion, multiple areas of pinpointleakage, and optic disc hyperfluorescence in the RE andareas of RPE changes that were more prominent aroundthe optic disc in the LE (Fig. 2). Indocyanine green angiog-raphy revealed hypofluorescent dark dots in intermediateand late phases in the RE and showed no obviousabnormalities in the LE (Fig. 2). B-scan ultrasonographyFig. 1 Color fundus photography, optical coherence tomography, and ultrapresentation shows optic disc hyperemia and peripapillary exudative retinahaze and sunset-glow fundus. c, d Optical coherence tomography shows epigment epithelium undulations in the right eye and normal findings in thchoroidal thickening mostly around the optic disc, in the right eye, and a nshowed diffuse medium-reflective choroidal thickeningpredominating around the optic nerve head in the RE anda normal choroidal thickness in the LE. The axial lengthwas 23.1 mm in the RE and 23.15 mm in the LE (Fig. 1).Medical history excluded ocular trauma or surgery.Findings in each eye apart were very suggestive of VKHdisease, in its acute phase in the RE and in its chronicphase in the LE. Such asymmetry being atypical, aworkup was ordered to rule out conditions mimickingVKH disease. Results of systemic examination by theinternist, complete blood cell count, sedimentation rate,C-reactive protein, syphilis serology, skin tuberculin test,chest X-ray, and oculo-cerebral magnetic resonance im-aging were unremarkable.The patient was given oral prednisone initiated witha dose of 1 mg/kg/day and then gradually taperedover 6 months. In addition to that, she receivedmycophenolate mofetil (2 mg a day) for a 12-monthduration. After a 15-month follow-up, the patient wasstill receiving 5 mg of prednisone a day. BCVA was20/20 in the RE and 20/25 in the LE. Right funduswas normal (Fig. 3). There was a sunset-glow fundussonography at presentation. a Fundus photograph of the right eye atl detachment. b Fundus photograph of the left eye shows +2 vitreousxudative retinal detachment with a few subretinal septa and retinale left eye. e, f B-scan ultrasonography shows a medium-reflectiveormal choroidal thickness in the left eyeFig. 2 Fluorescein angiography and indocyanine green angiography at presentation. a Early-phase fluorescein angiogram of the right eyereveals multifocal areas of delayed choroidal perfusion. c Late-phase fluorescein angiogram of the right eye shows multiple areas ofpinpoint leakage and optic disc hyperfluorescence. b, d Fluorescein angiogram of the left eye shows areas of retinal pigment epithelialchanges mainly around the optic disc. e, f Intermediate-phase indocyanine green angiogram shows numerous hypofluorescent dark dotsin the right eye and no obvious abnormalities in the left eyeKhochtali et al. Journal of Ophthalmic Inflammation and Infection  (2015) 5:27 Page 3 of 4in the LE associated with RPE changes and a fewperipheral nummular atrophic lesions (Fig. 4). Therewere no recurrences or development of integumentarymanifestations. Ocular involvement was isolated inour patient, and therefore, a diagnosis of probableFig. 3 Right color fundus photography at the last follow-up. Fundusphotograph of the right eye, 15 months after presentation, showsnormal findingsVKH disease was made according to the RevisedDiagnostic Criteria for VKH disease [1].This case report was approved by the ethics committeeof Fattouma Bourguiba University Hospital, Monastir,Tunisia.DiscussionOur patient had an unusual presentation of VKH diseasewith features of acute disease in one eye and chronicdisease in the fellow eye. Other causes of ocular inflamma-tion including malignant hemopathies or other neoplasms,syphilis, tuberculosis, or sarcoidosis were excluded in ourpatient. The diagnosis of posterior scleritis could be ruledout based on the lack of ocular pain and clinical and ultra-sonographic findings.VKH disease is usually characterized by simultaneousonset of inflammation in both eyes, although the involve-ment of the second eye may occur 2 to 4 weeks after thefirst eye [1–3]. There have been very rare cases of VKHdisease that remained unilateral after a long follow-upperiod. However, subclinical disease in the fellow eye wasnot ruled out in these patients by indocyanine green angi-ography or B-scan ultrasonography, prior to the initiationof systemic corticosteroids [4]. Unilateral features of acuteVKH disease have also been described in a 4-year-old boy.Fig. 4 Left color fundus photography at the last follow-up.Composite fundus photograph of the left eye, 15 months afterpresentation, shows a sunset-glow fundus with retinal pigmentepithelial changes and a few peripheral nummular atrophic lesionsKhochtali et al. Journal of Ophthalmic Inflammation and Infection  (2015) 5:27 Page 4 of 4However, ultrasonography in this case revealed choroidalthickening in both eyes, which was consistent with bilat-eral involvement [5]. A long delay between the first andsecond eye involvement, ranging from 11 months to6 years, has been previously reported in four patients witha history of unilateral panuveitis before the definitive diag-nosis of VKH disease was established. Baseline subclinicalinflammation of the initially unaffected eye might havebeen overlooked in these cases [6, 7]. On the other hand,prolonged initial unilateral ocular involvement in VKHdisease may be misdiagnosed as posterior scleritis [8].Among these patients with a long interval before thesecond eye was affected, concomitant sunset-glow fundusin one side and ERD consistent with acute VKH disease inthe other side has been noted in only two patients [6, 7].To the best of our knowledge, our report is the first todescribe concurrent acute VKH in one eye and sunset-glow fundus in the fellow eye at initial presentation. Thisstriking contrast between the right and the left eyesmight suggest a significant delay before the second eyewas affected. However, our patient did not report anyocular complaints when the first eye was affected. Shepresented only when vision blurring became bilateral.The mechanism of delayed involvement of the secondeye remains unclear. Early bilateral involvement withminimal inflammation in the RE seems unlikely inour patient. In fact, without any treatment, sunset-glowfundus would have developed in both eyes.In summary, patients with VKH disease may presentwith atypical features of chronic disease in one eye andacute disease in the fellow eye. Initial unilateral ocularinflammation, progressing relentlessly to chronic disease,may remain asymptomatic until the fellow eye is involved.Accurate diagnosis is essential for prompt initiation ofcorticosteroid and immunosuppressive therapy to reducethe risk of visual morbidity.AbbreviationsBCVA: best corrected visual acuity; ERD: exudative retinal detachment; LE: lefteye; RE: right eye; RPE: retinal pigment epithelium; SD-OCT: spectral-domainoptical coherence tomography; VKH: Vogt-Koyanagi-Harada.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsSK carried out the writing of the manuscript. NA, MSH, and IKK participatedin the collection and interpretation of the data. BJ participated in theinterpretation of the data. MK carried out the critical revision and correctionof the manuscript. All authors read and approved the final manuscript.AcknowledgementsThis work has been supported by the Ministry of Higher Education andResearch of Tunisia.Received: 9 July 2015 Accepted: 14 August 2015References1. Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L,Pivetti-Pezzi P, Tessler HH, Usui M (2001) Revised diagnostic criteria forVogt-Koyanagi-Harada disease: report of an international committee onnomenclature. Am J Ophthalmol 131(5):647–522. Attia S, Khochtali S, Kahloun R, Zaouali S, Khairallah M (2012)Vogt–Koyanagi–Harada disease. Expert Rev Ophthalmol 7(6):565–5853. Rao NA, Gupta A, Dustin L, Chee SP, Okada AA, Khairallah M, Bodaghi B,Lehoang P, Accorinti M, Mochizuki M, Prabriputaloong T, Read RW (2010)Frequency of distinguishing clinical features in Vogt-Koyanagi-Haradadisease. Ophthalmology 117(3):591–9.e14. Usui Y, Goto H, Sakai J, Takeuchi M, Usui M, Rao NA (2009) PresumedVogt-Koyanagi-Harada disease with unilateral ocular involvement: report ofthree cases. Graefes Arch Clin Exp Ophthalmol 247(8):1127–325. Forster DJ, Green RL, Rao NA (1991) Unilateral manifestation of theVogt-Koyanagi-Harada syndrome in a 7-year-old child. Am J Ophthalmol15;111(3):380–26. Roe RH, Rathinam SR, Wong RW, McDonald HR, Jumper JM, Cunningham ETJr (2009) Delayed fellow eye involvement in patients with Vogt-Koyanagi-Harada disease. Br J Ophthalmol 93(5):701–27. Far NY, Liu DT (2012) Probable Vogt-Koyanagi-Harada disease with initialunilateral ocular manifestation in a hepatitis C carrier. J Ophthalmic InflammInfect 2(4):235–78. Kouda N, Sasaki H, Harada S, Yamada Y, Takahashi N, Sasaki K (2002) Earlymanifestation of Vogt–Koyanagi–Harada disease as unilateral posteriorscleritis. 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Concurrent acute Vogt-Koyanagi-Harada disease in one eye and chronic disease in the fellow eye

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Concurrent acute Vogt-Koyanagi-Harada disease in one eye and chronic disease in the fellow eye

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