Case of the Month

Edited by Robert N. Johnson, MD

Case #111, Sept, 2018

Presented by:

Joseph Alsberge, MD

A 50-year-old man with an 8-month history of worsening vision in his left eye.

Figures 1 and 2: Color photo montage  of the right and left retina. Note the extensive areas of chorioretinal scarring in the posterior pole. The left eye has an area of subretinal hemorrhage and serous exudation in the macula.

Case History

A 50-year-old Middle Eastern man presented with the complaint of worsening vision in the left eye of 8 months duration.

On examination, visual acuity was 20/200 in the right eye and 3’/200 in the left eye. The anterior segment examination was unremarkable. Dilated examination revealed geographic atrophic chorioretinal scars projecting from the optic nerve and involving the macula in both eyes, and subfoveal hemorrhage in the left eye (Figures 1 and 2). Fluorescein angiography revealed early hypofluorescence of the atrophic scars secondary to choriocapillary loss with late staining in both eyes, as well as findings consistent with subfoveal choroidal neovascularization in the left eye (Figures 3-5). Optical coherence tomography revealed retinal atrophy with disruption of the photoreceptors and retinal pigment epithelium in the right eye and subfoveal hyper-reflective material in the left eye (Figures 6 and 7).

Figures 3-5: Fluorescein angiograms of the left and right eye. The early phase of the left eye shows lacy hyperfluorescence in the macula and in the late phases, there is significant leakage. Multiple areas of chorioretinal scarring show hypofluorescence early with some mild late staining.

Figures 6 and 7: OCT-SD studies of the right and left macula. Areas of outer retinal and RPE atrophy are noted in both eyes. In the left macula, note the area of subretinal fluid and hyperreflective material corresponding to hemorrhage on the color photo (Figure 2), and area of choroidal neovascularization seen on the fluorescein angiogram (Figures 3 and 4).

What is your Diagnosis?

Differential Diagnosis

Serpiginous choroiditis, tuberculous serpiginous-like choroiditis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), relentless placoid chorioretinitis, sarcoidosis, syphilis.

 

Additional History and Diagnosis

The patient reported a several-year history of episodic blurry vision in both eyes. A prior provider had performed an extensive work up for infectious and systemic inflammatory etiologies, which was negative. He had been previously treated with systemic steroids and noncorticosteroidal immunosuppressives, though he was no longer on these agents. Given this history, and the pattern and location of the chorioretinal scars, the diagnosis of inactive serpiginous choroiditis with choroidal neovascularization was made. He was given an intravitreal injection of bevacizumab in the left eye and asked to return in one month’s time for ongoing monitoring for recurrent inflammation and to monitor response to the bevacizumab injection.

Discussion

Serpiginous choroiditis, also sometimes referred to as geographic choroiditis or helicoid peripapillary choroidopathy, is a rare, recurrent, bilateral, and progressive ocular inflammatory disease of unknown etiology that typically occurs in healthy patients between the ages of 30 and 70.1

The acute phase of the disease is classically characterized by deep grey-white or yellow geographic placoid lesions that project from the optic nerve and spread centrifugally.2 Patients typically lack marked intraocular inflammation, although one third of cases may have vitreous cell.1 Over the course of weeks, the acute placoid lesions resolve and become atrophic chorioretinal scars. Recurrent episodes are common, and new active lesions often develop at the edge of atrophic areas, although they also may occur in entirely separate locations.

Patients are often asymptomatic in the early phases of the disease, or they may notice small paracentral scotomas related to the placoid lesions.2 Patients develop significant central vision loss, however, if lesions advance to the fovea.

Fluorescein angiography in the acute phase of the disease classically shows early hypofluorescence of the placoid lesions due to edema of the outer retina and retinal pigment epithelium and non-perfusion of the choriocapillaris, while in later frames of the study the lesions become hyperfluorescent due to staining.2 In late disease, as demonstrated by the case presented here, the atrophic areas are initially hypofluorescent due to destruction of choriocapillaris, and become progressively more hyperfluorescent in later frames due to staining.

It is important to consider tuberculous serpiginous-like choroiditis in the differential diagnosis and should be of particular concern in patients who present with serpiginous-like lesions and live in, or come from, areas with a high prevalence of tuberculosis.2 In contrast to serpiginous choroiditis, tuberculous serpiginous-like choroiditis tends to have significant vitritis and presents with multifocal lesions in both the posterior pole and periphery, often sparing the juxtapapillary area.3

Acute episodes of inflammation are typically treated with high dose systemic steroids (e.g., oral prednisone, 60-80 mg/daily). Recurrences may occur when steroids are tapered, requiring the addition of noncorticosteroid immunosuppressives.4 Choroidal neovascularization is a late complication in 25% of serpiginous choroiditis cases, often developing at the edge of chorioretinal atrophy, and may be treated with anti-VEGF injections.5

Take Home Points

  • Serpiginous choroiditis is an idiopathic, recurrent, bilateral, and progressive ocular inflammatory disease that is characterized by geographic placoid lesions that project from the optic nerve and spread centrifugally.
  • Tuberculous serpiginous-like choroiditis should be considered in the differential diagnosis in the appropriate context and ruled out with systemic testing.
  • Acute episodes of serpiginous choroiditis should be treated with high dose systemic steroids. Recurrences are common during steroid tapering, however, requiring the addition of noncorticosteroid immunosuppressives.

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References

  1. Mirza RG, Jampol LM. White Spot Syndromes and Related Diseases. In: Ryan SJ, Schachat AP, Sadda SR, eds. Retina. Fifth ed. Elsevier:1337-1380.
  2. Agarwal A. Inflammatory Diseases of the Retina. In: Agarwal, A. Gass’ Atlas of Macular Diseases. Fifth ed. Elsevier:948-1063.
  3. Vasconcelos-Santos DV, Rao PK, Davies JB, et al. Clinical features of tuberculous serpiginous-like choroiditis in contrast to classic serpiginous choroiditis. Arch Ophthalmol 2010;128:853-858.
  4. Akpek EK, Baltatzis S, Yang J, et al. Long-term immunosuppressive treatment of serpiginous choroiditis. Ocul Immunol Inflamm 2001;9:153-67.
  5. Song MH, Roh YJ. Intravitreal ranibizumab for choroidal neovascularisation in serpiginous choroiditis. Eye (Lond) 2009;23:1873-1875.

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