Multimodal imaging ocular sarcoidosis

Jaime Larrea; Luz Catherine Martinez Malo; Daniel Jaramillo-Velásquez; Carlos Mario Rangel

doi:10.4103/PAJO.PAJO_19_19

CASE REPORT

Year : 2019 | Volume : 1 | Issue : 1 | Page : 14

Multimodal imaging ocular sarcoidosis

Jaime Larrea, Luz Catherine Martinez Malo, Daniel Jaramillo-Velásquez, Carlos Mario Rangel
Department of Ophthalmology, Retina and Vitreous Section, Fundación Oftalmológica De Santander Clínica Carlos Ardila Lulle, Bucaramanga, Colombia

Date of Submission	13-Aug-2019
Date of Acceptance	29-Sep-2019
Date of Web Publication	06-Nov-2019

Correspondence Address:
Dr. Carlos Mario Rangel
Department of Retina and Vitreous, Fundacion Oftalmologica de Santander Clinica Carlos Ardila Lulle, Bucaramanga
Colombia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/PAJO.PAJO_19_19

Abstract

The study objective was to present multimodal imaging of definite ocular sarcoidosis (OS) in a young adult. A 52-year old woman consulted for 5 days of photophobia, ocular pain on movement, and redness in her left eye. Slit-lamp examination showed bilateral inferior granulomatous keratic precipitates, high cellullarity in the anterior chamber, posterior synechiae and hypopigmented subretinal lesions in the inferior vascular arcade, and nasal retina. In addition to the complete ophthalmic examination fundus photography (FP), fluorescein angiography (FA) and optical coherence tomography-angiography (OCT-A) were performed.Multimodal imaging of both eyes of the patient was performed. FP showed periphlebitis, hypopigmented subretinal lesions in the inferior temporal vascular arcade, and nasal retina. FA showed late diffuse capillary leakage from the subretinal lesions and optic nerve. OCT-A showed decreased choroidal capillary density, small hypo-intense gray areas of retinal capillary hypoperfusion, and presence of confluent and isolated areas void of flow in the deep plexus and choriochapillaris. With these findings on the multimodal imaging, chest computed tomography scan, and negative laboratory workup for other diseases, a presumptive diagnosis of OS was made which was subsequently confirmed with a positive skin nodule biopsy. The combination of OCT-A and other diagnostic methods allows a more detailed evaluation of structural and functional clinical findings in OS. There are few studies on OCTA findings in OS. Further studies with larger samples are needed in order to establish specific OCTA findings that could be used as diagnostic criteria.

Keywords: Choroidal density, choroidal granulomas, ocular sarcoidosis, optical coherence tomography angiography, panuveitis, periphlebitis

How to cite this article:
Larrea J, Martinez Malo LC, Jaramillo-Velásquez D, Rangel CM. Multimodal imaging ocular sarcoidosis. Pan Am J Ophthalmol 2019;1:14

How to cite this URL:
Larrea J, Martinez Malo LC, Jaramillo-Velásquez D, Rangel CM. Multimodal imaging ocular sarcoidosis. Pan Am J Ophthalmol [serial online] 2019 [cited 2023 Mar 28];1:14. Available from: https://www.thepajo.org/text.asp?2019/1/1/14/270447

Introduction

Sarcoidosis is a multisystemic disease characterized by the accumulation of T lymphocytes and mononuclear phagocytes that form noncaseating epithelioid granulomas in the affected organs. The etiology of the disease is unknown, although genetic, environmental, and infectious agents have been hypothesized as triggers. The disease has a worldwide distribution, affecting both genders and all ages and races, but its prevalence is higher in the third and fourth decades of life.^[1] About 30%–60% of patients with sarcoidosis exhibit ophthalmic manifestations, and bilateral granulomatous intraocular inflammation is a frequent presentation.^[1]

The incidence of ocular sarcoidosis (OS) in developed countries has been found to vary, with higher rates reported in London and New York (27% and 20%, respectively) than in France and Eastern Europe (11% and 4%, respectively).^[1] Sarcoidosis was thought to be uncommon in developing countries such as Colombia; de-la-Torre et al. in 2009 reported the clinical patterns of uveitis in Colombia and found that the prevalence of uveitis due to sarcoidosis was 0.3%.^[2]

Eye sarcoid disease may occur in the absence of apparent systemic involvement or may be the main site of disease without significant clinical disease elsewhere. On presentation, almost 90% of patients will have an abnormal chest radiograph in the form of infiltrates, although not all may be symptomatic. The extrapulmonary manifestations of the disease include involvement of the skin, nervous system, eye, heart, liver, and a variety of other organs.^[1]

Many imaging modalities such as fluorescein angiography (FA), optical coherence tomography (OCT), and OCT-angiography (OCT-A) have been used for the diagnosis, follow-up, identification, and monitoring of patients with uveitis and its sequelae such as vasculitis, retinal or choroidal neovascularization, and cystoid macular edema (CME).^{[3],[4],[5],[6],[7],[8]}

OCT-A combines structural as well as functional information on blood flow within the retinal tissue. It is a newly developed, dye-less, imaging modality that provides a three-dimensional (3D) assessment of retinal and choroidal microvasculature. The absence of dye leakage phenomenon in OCT-angiograms allows a clearer visualization of vascular structures.^[9] The clinical application of OCT-A has been studied in inflammatory disorders, such as ocular tuberculosis, Behçet's disease, and multifocal choroiditis, and just a few in OS.^{[3],[4],[5],[6],[7],[8]}

The aim of this study is to illustrate the clinical presentation of OS in a young adult, with multimodal imaging combining traditional and novel imaging techniques, including FA, fundus photography, swept-source OCT, and OCT-A.

Case Report

A 52-year-old woman presented to the emergency room at Fundacioón Oftalmoloógica de Santander –FOSCAL (Floridablanca, Colombia) with a 5-day history of photophobia, ocular pain on movement, and redness in the left eye. On admission, the best-corrected visual acuity was 20/30 in the right eye and 20/25 in the left eye. Slit-lamp examination of the anterior segment showed bilateral inferior granulomatous keratic precipitates, high cellularity (cells 3+) in the anterior chamber in both eyes, and posterior synechiae in the left eye. Fundoscopy disclosed bilateral mild vitritis, peripheral snowballs, periphlebitis, optic disc edema, and multiple subretinal elevated lesions along the inferior temporal vascular arcade, highly suggestive of optic disc granuloma and multifocal choroiditis. The patient was admitted for complementary exams that included chest computed tomography (CT) scan and chest X-ray that showed bilateral hilar lymphadenopathy (BHL). The patient underwent FA (Zeiss Fundus Camera FF 450 plus, Jena, Germany), B-scan OCT (Topcon DRI OCT Triton, swept-source OCT, Topcon, Japan), and OCT-A (Optovue Inc., Fremont, CA, USA) [Figure 1] and [Figure 2]. With these findings on the multimodal imaging and negative laboratory workup for other diseases (e.g., ocular tuberculosis), a presumptive diagnosis of OS was made. Hence, we asked the dermatology unit to perform a skin nodule biopsy, which resulted positive for noncaseating granulomatous inflammation. Hence, a diagnosis of definite OS was made.

Figure 1: Multimodal imaging. Ocular fundus montage image (A) showing hypopigmented subretinal lesions in the inferior vascular arcade and nasal retina; (B) Fluorescein angiography revealing late diffuse capillary leakage from the subretinal lesions; (C) Optical coherence tomography B-scan (Topcon DRI OCT Triton) shows the presence of a hyporeflective lesion beneath the optic nerve. (D) Optical coherence tomography-Angio Montage (Optovue) show decreased choroidal capillary density. (E) Optical coherence tomography-angiograms (Optovue) of the superficial capillary plexus shows small hyporeflective gray areas of retinal capillary hypoperfusion/non-perfusion (E1), deep capillary plexus revealed the presence of areas of capillary hypoperfusion (E2) external retina is avascular (E3) and choriocapillaries show the presence of both confluent and isolated areas void of flow (E4)

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Figure 2: Multimodal imaging. Ocular fundus montage image (A) showing hypopigmented subretinal lesion adjacent to terminal vessels of the inferior vascular arcade and inferior to the fovea; (B) Fluorescein angiography reveals late diffuse capillary leakage from optic disc (C) Optical coherence tomography B-scan (Topcon DRI OCT Triton) shows the presence of a hyporeflective lesion in the choroid below the fovea (D) Optical coherence tomography-Angio Montage (Optovue) show a decrease in choroidal capillary density, (E) Optical coherence tomography-angiograms (Optovue) of the superficial capillary plexus show small hypo-intense, gray areas of retinal capillary hypoperfusion/non-perfusion (E1), deep capillary plexus revealing the presence of areas of capillary hypoperfusion (E2) external retina is avascular (E3) and choriocapillaries show the presence of both confluent and isolated flow void areas (E4)

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Discussion

Ocular involvement in sarcoidosis is present in up to one-third of patients and is frequently the first manifestation before the underlying systemic disease has been diagnosed,^[1] as was the case of our patient.

Sensitive biomarkers of disease activity have not yet been identified. As elevated activity of angiotensin-converting enzyme and lysozyme is associated with disease activity, it may be supportive but not specific for diagnosis. In patients with newly diagnosed anterior uveitis presenting with granulomatous mutton-fat corneal precipitates, snow balls, vitreous body base condensations, mild retinal periphlebitis, as well as multifocal choroiditis (creamy choroidal lesions at different stages of activity and approximately 500 μm in diameter), a diagnosis of sarcoidosis has to be considered and tuberculosis should be excluded, even in the absence of a known systemic disease.^[10]

The 2009 IWOS conference established four levels of certainty for the diagnosis of OS, namely, (1) biopsy-supported diagnosis with compatible uveitis labeled as definite OS; (2) if biopsy was not done but chest X-ray was positive showing BHL associated with a compatible uveitis, the condition was labeled as presumed OS; (3) if biopsy was not done and chest X-ray did not show BHL but there were three of the above intraocular signs and two positive laboratory tests, the condition was labeled as probable OS; and (4) if lung biopsy was done and the result was negative but at least four of the above signs and two positive laboratory investigations were present, the condition was labeled as possible OS.^[10]

Our patient had definite OS (classic uveitic findings including granuloma), hilar lymphadenopathy on chest CT-scan, and a skin nodule biopsy, which resulted positive for noncaseating granulomatous inflammation.

Posterior segment granulomas are uncommon and are typically found in the choroid or optic nerve; in our case, we found them in both.^[4] The presence of a hyporeflective, round-shaped area with homogeneous content in the choroidal stroma which generates a cone of increased transmission of the OCT signal through the underlying structures suggests the presence of a choroidal granuloma (CG). Large CG can compress the surrounding vasculature, impairing choroidal blood flow and appearing as areas void of blood flow on OCT-A. The presence of an increased transmission effect underneath the granulomas distinguishes the lesions from normal choroidal vessels.^[4]

Uveitis often have specific OCT/OCT-A findings according to the involved ocular tissues and the kind of inflammatory/infectious process that characterizes them.^[5],[7] Increased permeability of the vessel walls is one of the key features of inflammation. This results in leakage of inflammatory proteins and migration of inflammatory cells from the intravascular space toward the inflamed area. Inflammatory cells floating in the anterior chamber or in the vitreous are commonly seen as hyperreflective dots on OCT scans. Vitreous haze is also detectable on OCT images as an increased brightness of the vitreous cavity. An increase of the choroidal thickness is an indirect measurement of increased blood flow within the choroid induced by the inflammation and can be monitored to evaluate the inflammatory status of the eye over time. CME is the first cause of blindness in uveitis and is easily visualized on OCT/OCT-A, as it can quantify CME in a noninvasive way.^[5]

Conclusions

We reported multimodal imaging of a patient with definite OS; in this case, panuveitis presented as the first manifestation of systemic sarcoidosis not previously diagnosed. OCT-A is a newly developed, dye-less, noninvasive imaging modality that provides a 3D analysis of the retinal and choroidal microvasculature and should be used in combination with other methods for allowing a more detailed evaluation of structural and functional clinical findings in OS. There are few studies on OCT-A findings in OS; hence, further studies with larger samples are needed in order to establish specific OCT-A findings that could be used as diagnostic criteria and to identify biomarkers of the functional outcome in OS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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