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Year : 1967  |  Volume : 15  |  Issue : 3  |  Page : 117-119

Vogt-Koyanagi syndrome

Department of Ophthalmology, Irwin Hospital, New Delhi, India

Date of Web Publication21-Jan-2008

Correspondence Address:
Hari Mohan
Department of Ophthalmology, Irwin Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Mohan H, Gupta A N. Vogt-Koyanagi syndrome. Indian J Ophthalmol 1967;15:117-9

How to cite this URL:
Mohan H, Gupta A N. Vogt-Koyanagi syndrome. Indian J Ophthalmol [serial online] 1967 [cited 2023 Jan 31];15:117-9. Available from: https://www.ijo.in/text.asp?1967/15/3/117/38700

Vogt-Koyanagi Syndrome is a rare type of non-traumatic bilateral uveitis associated with vitiligo, poliosis, alo­pecia and dysacousia. Commonly oc­curing in the fourth decade of life, the complete syndrome is rare (Jaffe, 1950). Included under the broad group of uveomeningeal syndrome, the condition has strong resemblance with sympathe­tic ophthalmitis and Harada's disease (Ponte, 1957; Hager, 1957).

While it is considered that there is no effective treatment of the condition (Reed and associates, 1958), it has been suggested that corticosteroids in heavy doses may be of value (Crow­ford, 1953; Sorsby, 1964). Ocular sur­gery in all uveomeningeal syndromes is said to be poorly tolerated (Wadsworth, 1959) and should not be done unless absolutely necessary (Ruiz, Barranco and Garrachon, 1956).

A case of complete Vogt-Koyanagi syndrome in a young adult, who re­covered useful vision after surgery for complicated cataract in the right eye, is reported.

  Case Report Top

M.S., a 19-year old Mohammedan male was admitted on 22-5-62 with a history of gross deterioration of vision since three years, occurring simultane­ously in both eyes following recurrent sore eyes. Patchy depigmentation of skin and whitening of eyebrows and eyelashes [Figure - 1] were noted 3 months after the onset of the disease. The ocular symptoms were preceded by vague headache and malaise closely followed by patchy fall of scalp hair and defective hearing. The scalp hairs have since regrown but the deafness has persisted in a milder form. Vitiligo and poliosis have remained unchanged.

He was a young adult of moderate physique. Patchy depigmentation was present over the areola of the right nipple [Figure - 2], on the back, and on the medial aspect of thighs and scrotal region. Systemic examination showed no abnormality.

The left eye was phthisical with band degeneration of the cornea [Figure - 1] fol­lowing cataract (complicated) extrac­tion elsewhere 1½ years ago.

The right eye showed mild ciliary flush with obscuration of iris pattern, extensive posterior synechia and com­plicated cataract. Perception and pro­jection of light were accurate. The ocu­lar tension was normal. Slit lamp ex­amination revealed fresh and old K.Ps., cells in the anterior chamber and mo­derate aqueous flare. The routine in­vestigations for uveitis were negative. Audiometry revealed moderate impair­ment.

The patient was treated with topical atropine and hydrocortisone, sub-con­junctival injection of mydricain and hydrocortisone biweekly and systemic prednisolone 60 mgs. daily. The aqueous flare rapidly disappeared but cells in the anterior chamber and K.Ps. persisted for a few weeks. On 28-7-62 systemic predenisolone was tapered off but subconjunctival hydro­cortisone injection once weekly and topical atropine and hydrocortisone were continued. The eye remained quiet and on 9-8-62 an extracapsular cataract extraction with complete iri­dectomy was performed. Post-opera­tively subconjunctival hydrocortisone was continued biweekly. The progress was uneventful and the patient was discharged on 28-8-62 on topical atro­pine and hydrocortisone t.i.d. and sub­conjunctival hydrocortisone once week­ly for 6 weeks. On 18-10-62 the patient was seen again and the vision in the operated eye improved to 6/36 p with + 9.0 D sph. and + 2.0 D cyl. 180°. Slit lamp examination showed no acti­vity of the old inflammation. The fundus examination showed very hazy media through which the disc that could be seen appeared normal. Other details could not be made out. The patient was advised to use topical atropine and hydrocortisone for four weeks more and report for follow up.

The patient reported in March, 1963 when the eye was completely quiet, vi­sion remaining unchanged. The patient had stopped all treatment. He was ad­vised to continue topical hydrocorti­sone for 3 months. The patient was last seen in October, 1964, when the eye was quiet and the corrected vision was 6/36.

  Discussion Top

Vogt - Koyanagi - Harada Syndrome and sympathetic ophthalmitis are closely related conditions grouped un­der uveomeningeal syndrome (Ponte, 1957; Hager, 1957). However, unlike sympathetic ophthalmitis, the skin allergy to uveal pigment is variable in Vogt-Koyanagi-Harada group. Recent­ly a virus aetiology has been suggested by Sugiera and associates (1953) and Erbaken (1962). The former authors reported the actual isolation of the virus from the eyes of patients suffering from this condition and demonstrated positive neutralization test against this virus with the serum of another pa­tient suffering from the same disease. Erbaken (1962) also claimed to have isolated a virus from the cerebrospinal fluid of a patient suffering from Vogt-­Koyanagi-Harada disease. In the light of this recent knowledge Hague's (1944) view, of an encephalitic process result­ing in hypothalamic involvement which in turn is responsible for pigmentary disturbances and associated symptoms, sounds reasonable. The meningeal phase noticed during the initial stages in majority of the cases, often asso­ciated with hypothalamic symptoms, possibly lends further support to the above view.

The treatment of the condition is difficult and the result doubtful. Reed and associates (1958) and Boke (1959) doubted the beneficial effect of systemic corticosteroids when the disease involved both the anterior and pros­terior segments. Boke (1959) found that these cases eventually progressed to blindness inspite of the use of corti­costeroids. It was significant in our case that though the inflammation con­tinued for over 3 years, controlled in­termittently by treatment given else­where, the institution of heavy doses of systemic and local corticosteroids soon brought the condition under control. The surgery tolerated well in the right eye suggests that the outlook of intra­ocular surgery performed after ade­quate control of inflammation may not be very gloomy particularly in the later part of the disease. Possibly the seve­rity of uveal inflammation gradually decreases with the passage of time.

The interesting features of the case reported above were early onset (oc­curring in the 2nd decade) the occur­rence of the complete syndrome, the widespread pigmentary changes, the persistence of deafness, the beneficial effect of corticosteroids on the course of the disease and the recovery of work­able vision after surgery for compli­cated cataract in the right eye.

  Summary Top

A case of complete Vogt-Kayanagi Syndrome in a young adult has been described. The patient recovered use­ful vision after surgery for complicated cataract in the right eye.[15]

  References Top

Babel. J. (1939). Schweiz. med. wchnschr. 69, 1136. Cited by Jaffe, 1950.  Back to cited text no. 1
Boke, W. (1959), Klin. Mbl. Augenheilk. 154, 407.  Back to cited text no. 2
Crowford, H. E. (1953), Hawaii med. J. 13, 26.  Back to cited text no. 3
Erbaken. S. (1962), Amer. J. Ophthal. 53, 368.  Back to cited text no. 4
Hager, G. (1957), Klin. Mbl. Augen­heilk. 131, 89.  Back to cited text no. 5
Hague, E. B. (1944), Arch. Ophthal. 31, 520.  Back to cited text no. 6
Jaffe, N. S. (1950), Amer. J. Ophthal. 33, 571.  Back to cited text no. 7
Koyanagi, Y. (1929), Klin. Mbl. Augen­heilk. 82, 194.  Back to cited text no. 8
Ponte, F. (1957), Cior. ital. oftal. 10, 203. Cited by Wadsworth (1969).  Back to cited text no. 9
Ruiz Barranco, F. & Garrachon, J. (1956) Rev. Clin. espan. 63, 101. Cited by Wadsworth (1959).  Back to cited text no. 10
Reed, H.: Lindsay, A.; Silversides; J. L.; Speakman, J.; Monckton, G.. and Rees, D. L., (1958), Cann. Med. Ass. J. 79, 451.  Back to cited text no. 11
Sorsby, A. (1964), "Modern Ophthal­mology", Vol. 4, p. 675, London, But­terworth.  Back to cited text no. 12
Sugierra, S., Fukanda, M., and Eda, K. (1953), Acta. Sec. Ophthal. Jap., 57, 117.  Back to cited text no. 13
Wadsworth. J. A. C. (1959), Arch. Ophthal. 61, 495.  Back to cited text no. 14
Vogt, A. (1906), Klin. Mlb. Augenheilk. 44, 228. Cited by Jaffe. (1950).  Back to cited text no. 15


  [Figure - 1], [Figure - 2]


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