Case study: Vogt-Koyanagi-Harada syndrome in patient with one eye
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Vogt-Koyanagi-Harada syndrome is a rare autoimmune multiorgan disease that is characterized by granulomatous uveitis with a spectrum of auditory, neurological and skin manifestations.
Bilateral involvement, occurring either at the time of presentation or sequentially, is considered a hallmark of Vogt-Koyanagi-Harada (VKH) disease and is proposed as a diagnostic criterion by the International Workshop on Vogt-Koyanagi-Harada Disease, yet case reports of unilateral VKH disease exist in the literature. In this column, we report an atypical case of VKH in a one-eyed patient whose clinical course and investigation are consistent with VKH.
Case summary
A 34-year-old woman presented to our outpatient department with complaints of vision blurring in the left eye for the past 1.5 months, insidious in onset, progressive in nature and associated with pain and redness. There was a history of associated mild fever with severe headache. She had defective hearing and hair loss. She had a corneal opacity in the right eye from birth with no visual potential. She had no history of trauma, treatment or surgery in either eye.
Source: Dhivya Ashok Kumar, MD, FRCS, FICO, FAICO, Brindha Somasundaram, MBBS, DNB, and Amar Agarwal, MS, FRCS, FRCOphth
On general examination, she had telecanthus, madarosis, frontalis overaction, narrow palpebral aperture, beaked nose and deviated nasal septum (Figure 1). There was loss of hair noted on the scalp. Left eye unaided visual acuity was 6/36 with no improvement with pinhole. IOP recorded by noncontact tonometry was 14 mm Hg. She had circumciliary congestion with few endothelial keratic precipitates. Anterior chamber showed grade 4 cellular reaction (SUN classification) with moderate flare. The iris was boggy with Koeppe nodules in the pupillary margin and posterior synechiae at 9 to 12 clock hours. The anterior chamber was shallow with a miotic pupil (Figure 2). The lens was clear, but retina details could not be made out due to poor dilation and photophobia.
Investigations such as OCT and fundus fluorescein angiography were not possible due to nystagmus and poor dilation. Finally, we performed B-scan ultrasound (Figure 3), which revealed multifocal serous detachments with retinal elevation. The choroidal thickness was increased. All laboratory investigations were negative. Audiometry showed combined type of hearing loss in both ears. MRI of the brain showed partial agenesis of the distal part and splenium of the corpus callosum with pericallosal lipoma (Figure 4). Neurology was consulted, and electroencephalogram was performed.
The patient was started on intravenous pulse steroids (methylprednisolone 1 g per day for 3 days) and was then kept on oral corticosteroids with slow tapering. Topical steroids and cycloplegics were prescribed, and the patient was followed. Clinical improvement was noticed with serial B-scan with clearing of the media, resolution of serous fluid and improvement in visual acuity with treatment.
Vogt-Koyanagi-Harada syndrome
The uveomeningeal syndrome VKH is described as quadriphasic with prodromal, acute uveitic, convalescent and chronic phases. The ocular manifestations of VKH include panuveitis, granulomatous anterior uveitis, exudative retinal detachment, choroiditis and multifocal serous detachment. Associated optic disc edema is noted in 87% of cases. Ciliary body effusion causing shallow anterior chamber and secondary angle closure has also been reported.
The prodromal stage is characterized by fever, headache and nausea, which are nonspecific. This is followed by the uveitic stage, which shows the ocular manifestations. The third stage, or convalescent, is represented by healing of ocular signs with formation of depigmented fundus in the posterior segment, seen as “sunset glow fundus.” The limbus can become depigmented, forming Sugiura’s sign.
VKH typically occurs in two distinct phases: early and late. Early VKH is characterized by exudative retinal detachments, with or without anterior chamber and vitreous inflammation, combined with neurologic symptoms and signs. Late VKH is characterized by a chronic anterior uveitis or chronic panuveitis, a characteristic sunset glow fundus and cutaneous changes (alopecia, poliosis, vitiligo).
The diagnosis of VKH is essentially clinical. The most widely used revised diagnostic criteria, developed by an international committee on VKH, classify patients as complete VKH, incomplete VKH and probable VKH.
Role of additional investigations
Although clinical examination forms the mainstay of diagnosis, a few additional evaluations aid in confirmation. In the uveitic phase, fundus fluorescein angiography characteristically reveals numerous punctuate hyperfluorescent dots at the level of the retinal pigment epithelium. These dots gradually enlarge and stain the surrounding subretinal fluid with pooling of dye in the subretinal space. OCT is the other noninvasive alternative to look for serous detachments. However, both were not possible in this patient due to nystagmus and miotic pupil. Ultrasound imaging helps in scenarios in which other modalities may not be possible. Bilaterality of this patient was not made out due to the phthisical state of her fellow eye, although hearing loss was present in both ears. The presence of prodromal signs, clinical ocular presentations and ultrasound imaging favored unilateral VKH in this case. An early and aggressive high-dose systemic steroid with slow taper improved signs and symptoms.
Rare association
Clinical presentation of VKH in this one-eyed patient with a preexisting phthisical eye precluded us from one of the criteria of bilaterality noted in VKH. There was an additional neurological sign noted in MRI as presence of corpus callosal agenesis. There was also a lesion suggestive of pericallosal lipoma on imaging. Although, agenesis of the corpus callosum can be congenital, lipoma may be acquired. Nevertheless, such a rare combination of corpus callosal change with ocular panuveitis has not been reported earlier in literature. Neurological associations have been noted in VKH as headache, seizures, giddiness, meningitis signs and cranial nerve palsies, but colossal agenesis and associated disorders are not noted.
Conclusion
VKH is commonly seen in pigmented individuals and has a genetic link with HLA-DRB1. The inflammatory signs are related to the T-cell mediated immune response against melanocytes. Clinical evaluation and ancillary tests are the main mode of diagnosis. Timely steroid administration helps in prompt management by regaining vision. Rarely, systemic immunomodulators may be required in refractory cases. Early therapy and slow tapering aid in reducing the complication by reducing the length of the disease and decreasing extraocular manifestations of VKH.
- References:
- Cunningham ET Jr, et al. Ocul Immunol Inflamm. 2014;doi:10.3109/09273948.2014.939530.
- O’Keefe GA, et al. Surv Ophthalmol. 2017;doi:10.1016/j.survophthal.2016.05.002.
- Read RW, et al. Ocul Immunol Inflamm. 2000;doi:10.1076/ocii.8.4.227.6457.
- Sakata VM, et al. Autoimmun Rev. 2014;doi:10.1016/j.autrev.2014.01.023.
- Tsui E, et al. Ocul Immunol Inflamm. 2018;doi:10.1080/09273948.2017.1353638.
- For more information:
- Amar Agarwal, MS, FRCS, FRCOphth, is director of Dr. Agarwal’s Eye Hospital and Eye Research Centre. Agarwal is the author of several books published by SLACK Incorporated, publisher of Ocular Surgery News, including Phaco Nightmares: Conquering Cataract Catastrophes, Bimanual Phaco: Mastering the Phakonit/MICS Technique, Dry Eye: A Practical Guide to Ocular Surface Disorders and Stem Cell Surgery and Presbyopia: A Surgical Textbook. He can be reached at 19 Cathedral Road, Chennai 600 086, India; email: aehl19c@gmail.com; website: www.dragarwal.com.
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