History

58 year-old white female referred to UAMS for evaluation of “uveitis”. The patients reported being diagnosed with “uveitis” 3 years ago but were not given a specific diagnosis. Her chief complaint on presentation was blurred vision and seeing a lot of floaters in both eyes. Review of systems was significant for chronic headache and relatively recent onset hearing problems.

Medical History

1. Polymyalgia rheumatica
2. Hypertension

Examination

• Vision: OD: 20/40, OS: 20/40
• Pupils: Round and reactive with no APD
• Motility: Full OU
• Intraocular pressures: Normal OU
• Slit lamp examination: Significant for occasional cell, no flare, no precipitates or nodules OU.
• Fundus Examination:
1. OD: Figure 1-A and B
2. OS: Figure 2-A and B

Figure 1-A: hazy view of the right fundus due to vitreous cells. Note the subretinal white lesions		Fig 1B: a nasal shot showing the yellow creamy oval subretinal lesions
Fig 2A: posterior pole of the left eye showing similar lesions.		Fig 2B: a nasal shot showing the diffuse yellow creamy oval subretinal lesions. Note that these lesions can coalesce and form a plaque

Ancillary Tests

• Fluorescein Angiogram:

Fig 3 A,B,C,D,E and F: Early and late frames of fluorescein angiogram showing diffuse vascular leakage. Not that the white lesions seen on the color pictures are not as prominent on FA.

Laboratory Work-up

• CBC/diff: Normal
• ESR: 9
• ANA: Positive (titer=160)
• VDRL: Nonreactive
• MH-ATP: Nonreactive
• Lyme titer: 0.24 (within ref. Range)
• ACE: 31
• HLA-A29 Positive
• PPD: Negative with reactive controls
• Chest X-ray: Normal

Differential Diagnosis

• Tuberculosis (TB): Typically associated with other constitutional symptoms and physical findings suggestive of tuberculosis.



• Syphilis: In addition to the associated systemic findings, retinal findings are usually associated with RPE hyperpigmentation.



• Sarcoidosis: The ocular inflammation associated with this condition is typically granulomatous with mutton fat precipitates and iris nodules. Patients usually have marked candle wax dripping and sheathing of retinal vessels.



• Pars planitis: vitritis is typically mild and concentrated mainly in the anterior vitreous. The pars plana has the characteristic snowbanking. Affected individuals are usually young adults. No retinal lesions seen in this disease
.


• VKH: affects darkly pigmented individuals more than Caucasians. This is an inflammatory disease that involves the choroid with a secondary retinal involvement. It is associated with hearing loss, poliosis, vitiligo, meningeal signs and exudative retinal detachment. A fluorescein angiogram will demonstrate multifocal choroiditis with relatively intact retinal vasculature.



• Acute posterior multifocal placoid pigment epitheliopathy (APMPPE): affects young adults and is usually associated with minimal vitritis. The retinal lesions are bilateral, placoid and located mainly in the posterior pole. These lesions typically resolve within 6 weeks leaving RPE changes. Fluorescein angiography shows a distinctive pattern of early blockage and late staining.



• Multiple evanescent white-dot syndrome (MEWDS): Unilateral disease affecting young adults. Lesions are white small and deep located in the posterior pole. Vitritis is lacking or very minimal. Recovery is expected in 6 weeks with no sequallie.



• Multifocal choroiditis: Present with bilateral multiple small white lesions associated with vitritis. These lesions result in hyperpigmented spots similar to the punched out lesions seen in ocular histoplasmosis.



• Intraocular lymphoma: presents with vitreous cell, cellular infiltration of the vessels and medium size to large white placoid subretinal lesions.

Diagnosis

Birdshot Retinochoroiditis:

• A rare bilateral disease that mainly affects middle-aged women. It is characterized by posterior uveitis, choroiditis and distinctive posterior pole findings. Birdshot Retinochoroiditis is chronic recurrent disorder with a strong HLA-A29 association.



• Symptoms: Include blurry vision, floaters, photopsia, nyctalopia and dyschromatopsia. Significant visual loss is usually seen when there is CME.



• Signs: The anterior segment is typically quiet or may have few cells in the anterior chamber. It is very unusual to see synechie or large keratic precipitates. Significant amount of vitreous cell is usually seen. The retina is significant for multiple small creamy-yellow round-oval lesions located in the level of outer retina to inner choroid. The borders of these lesions are hazy and not well demarcated and they may coalesce and form a relatively large plaque. These lesions are spread mainly in the posterior pole and decrease in number anteriorly. Cystoid macular edema is commonly seen in the exacerbation phase and is the main cause of vision loss.



• Diagnosis: The typical presentation along with the characteristic fundus findings is usually sufficient to make the diagnosis. Fluorescein angiogram may demonstrate extensive leakage of small and large retinal vessels with associated CME. Interestingly, the retinal lesions are not as prominent on FA as they are on indirect ophthalmoscopy and the lesions that hyperfluoresce do not necessarily correspond to the lesions seen on exam. This may be due to the difference in activity and age of these different lesions. The demonstration of an HLA-A29 phenotype is an additional confirming piece of information to make the correct diagnosis.



• Treatment: is aimed at treating the macular edema and vitritis. Steroids systemically and regionally in the form of a subtenon injection have been used for acute excacerbations. Low-dose Cyclosporin has been shown to be beneficial as a long-term agent.