Orbital Sarcoidosis

Description of Ophthalmic-Orbital Sarcoidosis

Pathophysiology of Ophthalmic-Orbital Sarcoidosis

Symptoms and Signs of Ophthalmic-Orbital Sarcoidosis

Neuro-ophthalmic involvement

Diplopia: Due to cranial nerve palsies

Reduced vision with or without scotoma: Due to oedema or infiltration of the optic nerve

Visual symptoms: Due to optic nerve damage caused by raised intraocular pressure

Orbital structure involvement

Uveitis results in blurring of vision, photophobia, floaters, scotomata and painful red eye.

Periocular lesions present with dry eye symptoms and disfigurement of adjacent structures

Mass lesion within the orbit causes bulging of the eyeballs and double vision

If it involves the orbital and adnexal structures, it can produce symptoms related to the lacrimal gland, lacrimal sac, eyelid and orbit.

Diagnosis of Ophthalmic-Orbital Sarcoidosis

Diagnosis of ophthalmic or orbital sarcoidosis is established through meticulous clinical examination and investigations.

Clinical examination reveals various manifestations of sarcoidosis in five different areas, namely:

  1. Neuro-ophthalmic manifestations: Rare cases of diplopia and ptosis occur due to involvement of the cranial nerves – oculomotor, trochlear, or abducens nerve. 5-38% of patients with neurosarcoid affect the optic nerve with ensuing edema, increased vascularity, nerve head elevation, haemorrhage, or optic atrophy. Retrobulbar optic nerve involvement might present as a mass lesion requiring biopsy to confirm the diagnosis.
  2. Ocular manifestations: 25-54% of patients with sarcoidosis present with ocular manifestations while almost all cases of ophthalmic sarcoidosis have systemic involvement.

Heerfordt Syndrome: Uveitis, parotid enlargement and occasionally papilledema.

Löfgren syndrome: Erythema nodosum, bilateral hilar adenopathy, arthralgias and uveitis.

Painless, bilateral, palpable swelling of the lacrimal gland is seen in some patients causing moderate to severe keratitis sicca.

Posterior segment disease: Seen in 25-30% of patients with sarcoidosis. Findings include:

Orbital disease: 20% of patients with ophthalmic manifestations of sarcoidosis involve the orbit or the lacrimal gland presenting as a mass lesion with bulging of the eyes, drooping of eyelids, enlarged lacrimal gland or ophthalmoplegia.

Investigations form the basis of confirming a diagnosis of sarcoidosis due to the wide variety of eye disorders that mimic its manifestations. The various investigations that could lead to a diagnosis of sarcoidosis include:

  1. Neuroimaging: Magnetic Resonance Imaging (MRI) is more useful than Computed Tomography (CT). Lesions detected on MRI are multiple white matter lesions, meningeal enhancement, optic nerve enhancement and mass lesions in the brain parenchyma.
  2. Nuclear Studies: Gallium-67 citrate scanning helps in identifying potential biopsy sites to aid tissue diagnosis.
  3. Vitreous Fluid Analysis: Tapping of vitreous for immunological analysis provides supportive evidence for a diagnosis of sarcoidosis.
  4. Biopsy: Most cases of sarcoidosis require a biopsy from the most accessible tissues to confirm the diagnosis.

Treatment of Ophthalmic-Orbital Sarcoidosis

Oophthalmicor orbital sarcoidosis is treated using both medications and surgery.

Pharmacologic Therapy might include topical or systemic steroids, cycloplegics, topical nonsteroidal anti-inflammatory agents and corticosteroid-sparing agents.

Surgical treatment includes cataract surgery, vitrectomy or trabeculectomy.