Imaging trends in pediatric uveitis

(Image credit: AdobeStock/Parilov)

Reviewed by Debra Goldstein, MD, FRCSC

A number of imaging modalities may aid in the identification and management of uveitis, according to Debra Goldstein, MD,FRCSC.

Goldstein, the director of uveitis in the Department of Ophthalmology and Magerstadt Professor of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, delivered a presentation on the value of imaging in uveitis at the 33rd Annual Jack Crawford Day, the annual pediatric ophthalmology update organized by the University of Toronto. Goldstein described how optical coherence tomography (OCT), OCT-enhanced depth imaging (EDI), fluorescein fundus angiography (FFA), and indocyanine green (ICG) angiography are invaluable in the assessment of patients with uveitis.

Goldstein detailed the case of a patient with intermediate uveitis and 20/20 vision who complained of not being able to see well. Examination revealed vitreous cells but no clinical vasculitis. Goldstein said OCT can provide many insights in such instances.

“We can get useful information out of an OCT,” she said. “In addition to looking at the usual imaging, retinal thickness maps can add a lot of information.”

The OCT appeared normal on first glance in the right eye and there was mild cystic macular edema (CME) in the left eye (Figure 1). The retinal thickness map, however, revealed diffuse thickening (Figure 2), especially around the vessels, suggesting retinal vasculitis, which was confirmed on fluorescein angiography (Figure 3).

Figure 1: Optical coherence tomography of a patient with intermediate uveitis, demonstrating only mild cystic macular edema in the left eye.

Figure 2: Retinal thickness maps of the same patient reveal diffuse retinal thickening, not easily appreciated in Figure 1.

Figure 3: Fluorescein angiography demonstrates diffuse “ferning” small vessel vasculitis from the disc to the periphery in both eyes. (Images courtesy of Debra Goldstein, MD)

The patient was treated with immunomodulatory therapy, which resolved the vasculitis, Goldstein explained.

“Imaging was absolutely invaluable,” she said. “The fundus exam was not exciting. The OCT was minimally interesting except for the thickness map, and the fluorescein angiogram helped us.”

In another case, Goldstein detailed, a 17-year-old patient who had received 6 anti-VEGF injections received a diagnosis of CME secondary to a central retinal vein occlusion (CRVO) in the right eye. The patient later developed asymptomatic anterior uveitis in the right eye, thought to be secondary to aflibercept injections. The patient was treated with topical steroids and intravitreal bevacizumab injections were resumed.

Subsequently, the patient developed CME in the left eye, Goldstein said. Clinical exam revealed mild anterior and intermediate uveitis in both eyes. Fluorescein angiography of the posterior pole from the referring physician revealed only CME bilaterally. Widefield fundus angiography, however, revealed significant retinal vasculitis in both eyes.

“She had never had a CRVO,” Goldstein said. “She has bilateral intermediate uveitis with retinal vasculitis. She was started on immunosuppressive therapy and has done very well now for 4 years, with resolution of vasculitis and no recurrence of the CME.”

Goldstein also detailed a case of a 17-year-old patient with bilateral anterior and intermediate uveitis, with disc neovascularization in the right eye and presumed tubulointerstitial nephritis and uveitis syndrome.

In such cases, providers would be tempted to treat retinal neovascularization (NVD) with panretinal photocoagulation. In this instance, however, the FFA revealed widespread retinal vasculitis without any retinal ischemia.

“The fluorescein angiogram defined the NVD but very importantly showed us that there was no ischemia,” Goldstein said, noting there was no indication to use laser photocoagulation because the NVD resolved with treatment of the intraocular inflammation. “We just treat the uveitis.”

A subsequent case of a 16-year-old patient who had been referred for bilateral chronic anterior uveitis also reinforced the importance of imaging, said Goldstein, who noted that the patient was being treated with prednisolone acetate drops and the condition recurred when there was a taper of topical medication.

“Every single time they tapered the prednisolone acetate, the uveitis recurred, so he was referred to my service,” Goldstein said. “In this case, the OCT and FFA were unremarkable, but on exam there were very subtle small choroidal lesions.”

ICG angiography revealed hundreds of small choroidal lesions, changing the diagnosis from anterior uveitis to panuveitis. The prior therapy of topical steroids was inadequate, and the disease responded to immunotherapy.

Goldstein also detailed the case of an 8-year-old girl who presented with eye redness and decreased vision. Unfortunately, she had received a diagnosis of conjunctivitis and was treated with topical antibiotics and steroids. Visual acuity was CF in both eyes. She had 4 or more anterior chamber cells, anterior vitreous cells, and haze, disc edema, and exudative retinal detachments.

Imaging in this case was characteristic of Vogt-Koyanagi-Harada (VKH), Goldstein said. OCT revealed retinal detachment in the posterior pole, and EDI revealed a diffusely infiltrated choroid. Fluorescein angiography was also characteristic of VKH, with early punctate hyperfluorescence and pooling of the fluid in the subretinal space. After ruling out tuberculosis, the patient was treated with high-dose systemic steroids and biologic therapy with infliximab. Inflammation resolved and visual acuity recovered to 20/30 in both eyes.

“The take-home points for this case are that VKH can present in all age groups,” Goldstein said.

The development of widefield angiography has dramatically changed the ability to assess peripheral retinal pathology, and EDI provides the ability to assess choroidal inflammation.

“EDI provides easily obtainable information in cases such as VKH and birdshot, and ICG can provide even more information about the choroid,” Goldstein concluded.

Debra Goldstein, MD, FRCSC

P: 312-695-8150

This article is adapted from Goldstein’s presentation at the 33rd Annual Jack Crawford Day, an annual pediatric ophthalmology update organized by the University of Toronto. She has no financial disclosures related to this content.