|
OJHAS Vol. 10, Issue 2:
(Apr-Jun 2011) |
|
|
| Vogt-Koyanagi-Harada
Syndrome in a 10 Years Old Child |
|
Aalia R Sufi,
Sumera Zargar, Tejit Singh,
Department of Ophthalmology, Government Medical
College, Srinagar, Jammu & Kashmir, India. |
|
|
| |
|
|
|
|
|
|
Address for Correspondence |
610- F Bagh-
e-Haider,
Hyderpora Byepass,
Srinagar, Jammu & Kashmir,
India.
E-mail:
aaliarasool_s@yahoo.com |
|
|
|
|
|
Sufi AR, Zargar S, Singh T. Vogt-Koyanagi-Harada
Syndrome in a 10 Year Old Child. Online J Health Allied Scs.
2011;10(2):24 |
|
|
|
|
Submitted: May 29,
2011; Accepted: Jul 16, 2011; Published: Jul 30, 2011 |
|
|
|
|
|
|
|
| |
|
| Abstract: |
|
The Vogt-Koyanagi-Harada (VKH) syndrome is a rare systemic
disorder of uveitis, dysacousia, vitiligo, premature graying of the hair, eyebrows and eyelashes, and meningoencephalitis.
Although frequently unrecognised,VKH may affect children.
We report a case of a 10 year old girl who presented with headache and dimness of vision and was diagnosed
as papillitis on the basis of bilateral disc edema. However over the course of time developed skin changes
(poliosis,vitiligo over lower back) and depigmented patches in inferior fundus suggesting diagnosis of VKH disease.
Thus the diagnosis is difficult in the absence of extraocular manifestations. In such cases the diagnosis is based
on clinical evolution of the disease.
Key Words:
Vogt-Koyanagi-Harada Syndrome; Poliosis; Vitiligo; Uveitis.
|
|
Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral granulomatous panuveitis associated with cutaneous, neurological and auditory manifestations.1 Vogt was the first to report a case in 1906.2 Later similar cases were reported by Koyanagi in 1929,3 after which the entity was identified as Vogt-Koyanagi syndrome characterised by anterior uveitis with poliosis, vitiligo and auditory disturbances. In 1926 Harada reported a patient with uveitis affecting the posterior segment with retinal detachment and meningeal irritation.4 These two disorders are known to overlap in many aspects and
are now combined as a single entity as Vogt-Koyangi-Harada disease.1,5
The etiopathogenesis of VKH syndrome remains unknown. Clinically the disease is categorized into distinct phases:
-
Prodromal Phase: consisting of neurological symptoms-headache, fever, tinnitus followed by blurring of vision 1 or 2 days later.
-
Uveitic Phase: consisting of bilateral exudative retinal detachment, vitritis, disc hyperemia.
-
Chronic Phase: consisting of depigmentation of inferior fundus ( sunset glow appearance), Dalen-Fuchs nodules.
-
Recurrent Phase: consisting of anterior uveitis and subretinal neovascularisation.
Systemic associations
such as auditory signs (tinnitus, vertigo) and neurological signs (fever, headache, cerebrospinal fluid pleocytosis) occur at the onset of the disease. Dermal signs (poliosis,vitiligo) occur after 2-3 months of onset.6
A ten years old female child complained of headache and nausea
of four days duration. This was followed by blurring of vision for which she reported to the eye
OPD. There was no history
of ocular trauma or surgery. Evaluation revealed visual status of 20/400 in the right eye and 20/800 in the left eye. Her
pupils reacted sluggishly to light. Fundus examination showed hyperemic discs with blurred margins. MRI was done which was
normal. Based on the findings, patient was diagnosed as bilateral papillitis following which she received three doses of
intravenous methylprednisolone 500 mg for three days and was discharged on 40 mg prednisolone which was gradually tapered.
Her vision improved to 20/30 in the right eye and 20/60 in the left eye. However the patient reported back after two weeks
with complaint of diminution of vision. Examination revealed vision of 20/80 in both eyes. Slit lamp examination showed
posterior synechiae with pigment dispersion over the cornea and anterior lens capsule and fine keratic precipitates in both
eyes. Fundus examination could not be done in view of hazy media. Routine investigations were normal. Patient was treated as
a case of uveitis and again put on 40 mg prednisolone. Later systemic steroids were withdrawn as the patient complained of
weight gain and was put on topical steroids. After a period of two months patient developed graying of eyelashes (Fig. 1)
and hair (Fig. 2) with patches of vitiligo over lower back (Fig. 3). On subsequent follow up patient‘s fundus revealed clearing
of media with the appearance of pin head size granulomas in the inferior quadrant and within a week depigmented patches appeared
in the inferior quadrant of the fundus (Fig. 4, Fig. 5). The patient was kept on 5 mg prednisolone on alternate days and attained
visual acuity of 20/30 in the right eye and 20/40 in the left eye.
The patient was diagnosed as a case of Vogt-Koyanagi-Harada
syndrome according to the revised criteria for diagnosis of VKH.7 However this diagnosis was attained after the
disease manifested its extraocular associations of poliosis, vitiligo and developed sunset glow appearance of the fundus later
in the course of the disease. Also the reason due to which the initial diagnosis of VKH was not made was that VKH is primarily a
disease of adults with maximum frequency of onset in the thirties. It is rarely seen in children. According to a survey in Aravind
Eye hospital, three out of 98 patients (about 3%) attending the uveitis clinic were children less than 16 years of age.8
Our case also highlights the rare initial presentation of VKH
as bilateral disc edema. Retinal edema has been reported to present as the first sign of retinal involvement which is followed
by exudative retinal detachment.9 However our patient did not develop retinal detachment. This can be attributed to
the early administration of steroids which prevented development of retinal detachment.
Thus the diagnosis of the Vogt-Koyanagi-Harada (VKH) syndrome,
especially in children is difficult due to the rarity of its occurrence in this age group, the variable onset of clinical signs
and symptoms in the course of the disease and absence of diagnostic serological parameters.
- Moorthy RS, Inomata H, Rao
NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol 1995;39:265-292.
- Vogt A. Fruhzeitiges
Ergrauen der Zilien und Bemerkungen uber den sogenannten plotzlichen Eintritt
dieser Veranderung. Klin Monatsbl Augenheilk 1906;44:228-242.
- Koyanagi Y. Dysakusis,
Alopecia und Poliosis bei schwerer Uveitis nicht trauma-tischen Ursprungs.
Klin Monatsbl Augenheilk 1929;82:194-211.
- Harada E. On the acute
diffuse choroiditis. Acta Soc Ophthalmol Jpn 1926;30:356-378.
- Suguira S. Vogt-Koyanagi-Harada
disease. Jpn J Ophthalmol 1978;22:9-35.
- Inomata H. Vogt-Koyanagi-Harada
Disease. In: Yanoff M, Duker JS, editors. Ophthalmology. 2nd ed. Missouri:
Mosby; 2004. pp. 1196-1198.
- Russel WR, Holland GN, Rao
NA, Tabarra KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic
criteria for Vogt-Koyanagi-Harada disease: report of an International
Committee on Nomenclature. Am J Ophthalmol 2001;131:647-652.
- Rathinam SR, Vijayalakshmi
P, Namperumalsamy P, Nozik RA, Cunningham ET. Vogt-Koyanagi-Harada syndrome in
children. Ocular Immunology & Inflammation 1998;6(3):155-161.
- Sawhney R, Jain R, Kumar S,
Pannu BKS, Sood S. Glucose intolerance and Vogt-Koyanagi-Harada Syndrome.
Asian Journal of Ophthalmol 2002;4:8-10.
|
