The EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip-palate) is a rare disorder inherited as an autosomal dominant trait or can occur sporadically. We describe a case of this syndrome with chronic ocular surface disorder secondary to abnormalities of ocular adnexia.
A 25-year old primigravida at 11-weeks period of amenorrhoea presented with bilateral optic neuritis following Varicella Zoster viral (VZV) infection. She was serologically positive for systemic lupus erythematosus but negative for virus. The exact pathogenesis of the patient's severe optic neuritis, adduction and neurological deficit was unknown. The initiation of high dose steroids for optic neuritis was a big clinical dilemma in a pregnant patient with viral infection. The patient was treated with high dose steroids after three days of commencement of antiviral treatment. At 6 months after presentation, her visual acuity in the right eye was 6/36 with perception to light in the left.