Case 25 Answers

by Professor Chua
 

A 40 year-old Malay woman who came from a remote village presented to our eye department in mid-September, 2005 with bilateral acute iridocylitis. She was previously well. Her vision was 6/12 in both eyes and slit-lamp examination revealed cells in the anterior chamber and the anterior vitreous. She was started on topical steroid which failed to control the inflammation. Investigation including chest X-ray and blood tests (full blood counts, VDRL and auto-immune screening) were normal. She was started on oral prednisolone of 60 mg po od. During the treatment, she complained of problem with her hearing in both ears. An ENT referral was made. Unfortunately, the patient failed to return for further follow-up from mid-Oct, 2005.

She reappeared in the clinic recently with bilateral severe visual loss and persistent ocular irritation. She also had bilateral profound sensory deafness and recent dermatological changes (Figure 1 and 2). During her period of absence from the clinic, she had been seeing a local traditional doctor for treatment but without effect. Her visual acuity was light perception. Slit-lamp examination revealed bilateral seclusion pupillae (Figure 3) without any views of the posterior segments. The intraocular pressures measured 4 mmHg on applanation tonometry. B-scan ultrasound showed the retina appeared flat. She was started on topical steroid, topical atropine and high dose prednisolone 100mg po od. The inflammation responded well to treatment but the pupil remained occluded despite intensive mydriatic treatment. Her vision improved to hand movement one week later.

Figure 1. Bilateral red eyes with poliosis, white eyebrows and whitelocks.

Figure 2. Vitiligo of the right leg.

Figure 3. Complete occlusion of the right and left pupils.

The plan is for her to undergo pupil stretch +/- iridectomy once the inflammation is controlled in the hope of improving her vision further.

a. What is the diagnosis?

Vogt-Koyanagi-Harada's syndrome (VKH) and more specifically Vogt-Koyanagi's syndrome. It is a systemic disorder involving many organ systems, including the eye, skin and nervous system.

 

VKH was first described by Vogt in 1906 and later Koyanagi in 1929. Their patients presented with bilateral anterior uveitis, vitiligo, poliosis, alopecia, and hearing loss. This syndrome became known as Vogt-Koyanagi's syndrome. In 1926, Harada reported patients with posterior uveitis, exudative retinal detachment with pleocytosis of the cerebrospinal fluid which was termed Harada's syndrome. With the appearance of more cases, the two syndromes were noted to have many overlapping similarities and eventually the condition was termed Vogt-Koyanagi-Harada's syndrome. Nevertheless, it remains useful to divide the syndrome into two types because of the variable involvement of different systems and visual prognosis (see table below¹).

b. What is the main differential diagnosis?

It is important to exclude sympathetic ophthalmia which may present with similar features. History of ocular injury or surgery is important to differentiate the two.

 

VKH can not be diagnosed using any blood or laboratory tests. The diagnosis is clinical and the American Uveitis Society² recommends that the following criteria be used in its diagnosis:

 

1. An absence of ocular trauma or surgery

 

2. Presence of at least 3 of the following 4:

• bilateral chronic iridocyclitis

• posterior uveitis (including multifocal exudative retinal detachments, and disc hyperaemia or oedema)

• neurological signs of tinnitus, neck stiffness, cranial nerve or central nervous system dysfunction or cerebrospinal pleocytosis

• cutaneous findings of alopecia, poliosis or vitiligo

Using these criteria, our patient meets the diagnosis of VKH.

c. What is the temporal relationship between the eye presentation and the systemic manifestations in this condition?

VKH can present with diverse symptoms, however, it can be divided into three phases and each phase is associated (but not exclusively) with each systemic involvement. The 3 phases are: the prodromal, uveitic and convalescent phases³.

• In the prodromal phase, variable degree of symptoms may appear and typically last a few dyas. Signs of meningism such as headache, fever, photophobia and neck stiffness may occur. Sometimes mental disorientation may occur. The CSF may show pleocytosis with raised intracranial pressure.

• In the uveitic phase, the patients present with ocular irritation and blurred vision. This usually lasts a few weeks.

• Finally the convalescent phase which typically occurs 3 months after the onset of the syndrome. It is characterized by cutaneous signs such as alopecia, poliosis, whitening of the hair and vitiligo. Sometimes, the cutaneous signs may occur a few years before the onset of uveitis.

Tinnitus and hearing impairment that affect a great majority of VKH patients may present at any phases.

Our patients who did not present with cutaneous signs at her initial presentation appear to follow the last two phases mentioned. In retrospect, this patient's hearing problem should have alerted the treating ophthalmologist to this syndrome and the patient started on high dose steroid as an in-patient.

References:

1. Albert DM & Jacobiec FA. Principles and Practice of Ophthalmology. Clinical Ophthalmology: Chapter 93: Vogt-Koyanagi-Harada (Uveomeningitic) Syndrome. 2nd edition. W.B. Saunders Company.

2. Snyder DA, et al. Vogt-Koyanagi-Harada's syndrome. Am J Ophthal 1980; 90: 69-75.

3. Choczaj-Kukula A, Janniger CK. Vogt-Koyanagi-Harada's syndrome: emedicine.com. Sept 2003.