Korean J Ophthalmol.  2009 Jun;23(2):74-79. 10.3341/kjo.2009.23.2.74.

Edema of the Photoreceptor Layer in Vogt-Koyanagi-Harada Disease Observed Using High-Resolution Optical Coherence Tomography

• Lee JE
• Park SW
• Lee JK
• Choi HY
• Oum BS
• Kim HW

• KMID: 754721
• DOI: http://doi.org/10.3341/kjo.2009.23.2.74

Abstract

PURPOSE: To evaluate the characteristics of fluid accumulation in the uveitic stage of Vogt-Koyanagi-Harada (VKH) disease using high resolution optical coherence tomography (OCT3).
METHODS
Twenty-eight eyes in 14 patients with VKH disease were reviewed retrospectively. These 28 eyes were divided into 19 eyes with intraretinal fluid (C group) and 9 eyes without intraretinal fluid (N group). Changes in visual acuity and fluid accumulation observed using OCT were compared between the two groups.
RESULTS
Visual acuity at the time of presentation was significantly worse in the C group than in the N group (p=0.005). The photoreceptor layer appeared to be double-layered due to a cystoid space in the C group. Layered structures and strands found in the cystoid space. Expanding sponge-form edema led to the development of a cystoid space in the photoreceptor layer. Intraretinal fluid resolved earlier than subretinal fluid. There were no observed differences in visual acuity between the two groups after four days of treatment.
CONCLUSIONS
Accumulation of intraretinal fluid was related to poor initial visual acuity, but not to final visual acuity. High resolution OCT findings indicate that edema of the photoreceptor layer participates in the development of a cystoid space.

Keyword

Cystoid space; Optical coherence tomography; Photoreceptor layer; Vogt-Koyanagi-Harada disease

MeSH Terms

Adolescent
Adult
Diagnosis, Differential
Female
Follow-Up Studies
Humans
*Image Enhancement
Macular Edema/etiology/*pathology
Male
Middle Aged
Photoreceptor Cells, Vertebrate/*pathology
Retrospective Studies
Tomography, Optical Coherence/*methods
Uveomeningoencephalitic Syndrome/*complications/pathology
Young Adult

Figure

• 

  Fig. 1 Cross-sectional images taken using high resolution optical coherence tomography (OCT) show the various features of the cystoid space in acute Vogt-Koyanagi-Harada disease. (A) A cystoid space that does not involve the center of the fovea on a 9 mm vertical OCT scan of the left eye from a 33-year-old man. Visual acuity was 20/20. Sponge-like edema is noted in the photoreceptor layer (arrows). (B) Optically empty cystoid spaces with thin outer walls (arrowheads) are seen on a 9 mm horizontal OCT scan from a 43-year-old woman. Sponge-like edema expanding toward the cystoid space is seen in the photoreceptor layer (arrows). (C) A 9 mm horizontal OCT scan from a 21 year-old man. A layered structure is noted in the cystoid space (short arrows). (D) A 9 mm horizontal OCT scan from a 46 year-old woman demonstrates a large cystoid space with a thin outer wall (arrowheads). Multiple vertical strands are noted in the space (short arrows). In the every case, all of the retinal layers can be identified inside the cystoid space. The outer boundary of a portion of the cystoid space continues to the photoreceptor layers (A and C, arrowheads).

• 

  Fig. 2 Serial optical coherence tomography (OCT) scans from a 47 year-old woman with Vogt-Koyanagi-Harada disease demonstrate the development of a cystoid space in the photoreceptor layer. Serous retinal detachment and sponge-like edematous changes of the photoreceptor layer are noted in the horizontal (A) and vertical (B) scans. A highly-reflective band representing the junction of the inner and outer segments is noted (arrows). The next day, before the initiation of intravenous steroid treatment, horizontal (C) and vertical (D) OCT scans show that the intraretinal cystoid space had ballooned to oppress the subretinal space (arrowheads). All retinal layers are identified, including the outer nuclear layer (arrow, ON) and the outer plexiform layer (arrow, OP).

• 

  Fig. 3 Serial optical coherence tomography (OCT) scans from a 32-year-old man diagnosed with Vogt-Koyanagi-Harada disease. (A) Intraretinal fluid is noted on a 9 mm horizontal OCT scan of the right eye after three days of treatment with oral prednisolone. The margins of the cystoid space are indicated by arrows. (B) Methylprednisolone (125 mg) was infused intravenously for three days. On day six, as the outer boundary of the intraretinal space degraded, the cystoid and the subretinal spaces became interconnected (arrows). Defects or notches are seen at the margins of the previous cystoid space (arrowheads). An oral steroid was prescribed again. (C and D) The subretinal fluid gradually resolved over one month.

• 

  Fig. 4 Serial 9 mm vertical optical coherence tomography (OCT) scans from the right eye of a 43-year-old woman diagnosed with Vogt-Koyanagi-Harada disease. (A) An OCT scan before steroid treatment demonstrates a large cystoid space involving the fovea (arrows). (B) On the third day, multiple layered structures are noted in the cystoid space. (C) On day seven, the cystoid space decreased in size and the layered structure became more distinct. (D) After two weeks of steroid treatment, a small volume of subretinal fluid was noted under the fovea without a cystoid space.

• 

  Fig. 5 Changes in visual acuity during acute episodes of Vogt-Koyanagi-Harada disease during systemic steroid therapy according to location of the fluid. The visual acuity of the group with a cystoid space was significantly worse compared to the group without a cystoid space. The differences in visual acuity between the two groups was found no later than four days after treatment began (*p<0.05).

• 

  Fig. 6 An explanation of the formation of an outer retinal cystoid space caused by photoreceptor layer edemain acute Vogt-Koyanagi-Harada (VKH) disease. (A) Acute swelling of the photoreceptors unfolds the discs of the outer segments. (B) If the changes in the photoreceptor are reversible, the photoreceptors recover as the edema improves. (C) If the changes are irreversible, the plasma membrane is degraded and the separated outer segments are removed by phagocytosis of the RPE. In either case, subretinal fluid will emerge in the area of the cystoid space after the resolution.

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