Ocular Sarcoidosis

Fig. 14.1
External photograph of sarcoid granuloma on the face

In children, sarcoidosis usually manifests as a triad of rash, uveitis, and arthritis in children less than 4 years of age. In older children, it could be associated with generalized lymphadenopathy and eyes, skin, liver, and lung involvement. In the developing world where tuberculosis is endemic, the occurrence of generalized lymphadenopathy is usually mistaken for tuberculosis and many of the children are on antitubercular therapy. Childhood sarcoidosis may also be misdiagnosed as juvenile chronic arthritis [2].

Ocular Sarcoidosis

Patients may be asymptomatic or present with blurred vision, floaters, redness, or discomfort. Ocular involvement is usually a chronic disease and has an insidious onset. It can involve the lacrimal glands, orbit, eyelids, conjunctiva, uveal tract, and optic nerve (Figs. 14.2, 14.3, 14.4, 14.5, 14.6, 14.7, 14.8, 14.9, 14.10, and 14.11). The ocular findings in sarcoidosis are summarized in Table 14.1 [2, 49, 1420].


Fig. 14.2
Slit-lamp photograph showing the conjunctival granulomas in a patient with thoracic sarcoid


Fig. 14.3
Slit-lamp photograph showing the scleral nodule in a patient with sarcoidosis


Fig. 14.4
Slit-lamp photograph showing the granulomatous anterior uveitis with large koeppe nodules


Fig. 14.5
Fundus photograph showing vitritis with snowball opacities in the peripheral retina


Fig. 14.6
Fundus photograph showing the perivasculitis


Fig. 14.7
Fundus photograph showing the disk edema


Fig. 14.8
Fundus photograph showing the optic nerve granuloma


Fig. 14.9
Fundus photograph showing the choroidal granuloma


Fig. 14.10
Fundus photograph showing the peripheral chorioretinal punched-out scars in a patient with sarcoidosis


Fig. 14.11
Fundus photograph showing the optic nerve head anastomosis in a patient with cutaneous sarcoidosis

Table 14.1
Ocular findings in sarcoidosis


Eye lid and conjunctival granulomas

Lacrimal gland involvement

Keratoconjunctivitis sicca

Nongranulomatous anterior uveitis

Mutton fat keratic precipitates

Iris and pupillary nodules/iris mass

Increase in IOP

Tent shaped PAS

Nodules in trabecular meshwork

Intermediate uveitis

Snowballs/strings of pearls vitreous opacities

Multifocal peripheral chorioretinal lesions (active and atrophic)

Nodular and/or segmental periphlebitis (with or without candle wax exudate)

Retinal macroaneurysm

Choroidal granuloma

Hemorrhagic retinopathy with branch or central retinal venous occlusions

Acute posterior multifocal placoid pigment epitheliopathy and retinal pigment epithelial detachments

Optic disk nodules/granuloma/optociliary shunts/dilated collateral veins on the optic nerve head

Neurological manifestations: cranial nerve palsies, encephalopathy, chiasmal syndromes, motility disorders, disorders of the hypothalamus and pituitary gland, optic atrophy either due to direct sarcoid tissue infiltration, or compression by cerebral mass

Lacrimal Gland and Orbits

Lacrimal gland involvement is common and may present as a dry eye. Severe keratoconjunctivitis sicca is not uncommon. Reports of sarcoid-induced myositis have been reported. Sarcoidosis coexisting with Grave’s disease has been reported.

Eyelids and Conjunctiva

Conjunctival granulomas are millet-shaped to large cream to brown nodular lesions, which can occur in sarcoidosis. They are usually asymptomatic. However large conjunctival granulomas can cause diplopia. They respond generally to topical steroids. Eyelid granulomas are also seen in sarcoidosis. Long-standing uveitis may lead to corneal band degeneration especially in children.


It is typically a chronic bilateral uveitis and characterized by granulomatous inflammatory reaction in the anterior segment which includes mutton fat keratic precipitates, iris nodules, trabecular meshwork nodules, and tent-shaped peripheral anterior synechiae; snowball and/or “string of pearls” opacities in the vitreous; and, in the posterior segment, nodular periphlebitis, multiple peripheral active or atrophic chorioretinal lesions, optic disk nodules/granuloma, and choroidal nodules. Reports of scleral involvement have also been described in sarcoidosis. In acute-onset sarcoidosis, non-granulomatous anterior uveitis may be seen. The 1st international workshop on ocular sarcoidosis in Tokyo in 2006 laid down diagnostic criteria for ocular sarcoidosis, consisting of 7 ocular signs (Table 14.2), 5 laboratory investigations (Table 14.3), and diagnostic criteria based on a combination of ophthalmic signs and laboratory investigations (Table 14.4) [17].

Table 14.2
Clinical signs suggestive of ocular sarcoidosis


Mutton fat keratic precipitates and/or iris nodules at papillary margin or on stroma


Trabecular meshwork nodules and/or tent-shaped peripheral anterior synechiae


Snowballs/strings of pearls vitreous opacities


Multifocal peripheral chorioretinal lesions (active and atrophic)


Nodular and/or segmental periphlebitis (with or without candle wax exudate) and/or macroaneurysm


Optic disk nodules/granuloma and/or solitary choroidal nodule


Bilateral inflammation (evident on clinical examination or on investigational imaging)

Table 14.3
Laboratory investigations in suspected ocular sarcoidosis


Negative tuberculin test in a patient who either had BCG vaccination or previously had a positive tuberculin test


Elevated serum angiotensin-converting enzyme and/or elevated serum lysozymea


Chest X-ray: bilateral hilar lymphadenopathy


Abnormal liver enzyme tests (any 2: of alkaline phosphatase, aspartate transaminase, alanine transaminase


Chest CT scan in patients with normal chest X-ray

aLysozyme is required in patients treated with ACE inhibitors

Oct 28, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Ocular Sarcoidosis

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