A Rare Ocular Manifestation of Idiopathic Hypertrophic Cranial Pachymeningitis

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare form of thickening of the dura mater. There are limited reports on the ocular manifestation of IHCP and its treatment. Up to our knowledge, there is no report on bilateral superior ophthalmic veins (SOV) dilatation with IHCP and there are only a few reports on anterior scleritis with IHCP. We report a 62-year-old gentleman with underlying hypertension and chronic headache who presented with fever, headache, and unresolving both eyes redness as manifestations of bilateral anterior scleritis, anterior uveitis, secondary glaucoma, and multiple cranial nerve palsies. Magnetic resonance imaging of the brain showed global thickening and enhancement of the pachymeninges with bilateral SOV dilatations. The diagnosis of IHCP was made after ruling out infective and autoimmune causes. The patient was treated with oral prednisolone, oral azathioprine, topical timolol maleate, topical dexamethasone, and topical moxifloxacin. The patient was successfully treated and was stable throughout two years review. In conclusion, unresolved red eyes with headaches can be an early presentation of IHCP. Pathophysiology and treatment of the ocular manifestations and IHCP were discussed.