A five-year-old female came with a history of frequent rubbing of the right eye and noticed prolonged elevation of her head since birth, informed by her mother. On ocular examination, the best corrected visual acuity shown in the right eye was 1/60, and the left eye was 6/6, with proptosis in both eyes. Fundus examination showed both eyes having pale discs. General assessment of the patient shows a high, peaked forehead and a shortened, pointed head shape suggestive of oxycephaly. Immediate neuroimaging revealed premature closure of skull bones with narrowing of orbit apex leading to bilateral compressive optic neuropathy, which is suggestive of craniosynostosis. Frontal advancement surgery was done for oxycephaly to relieve compression to the optic nerve. Post-surgery vision improved to 6/24 in the right eye. Recent advancements in surgical techniques and a collaborative team-based approach have significantly enhanced the safety and outcomes of this disease.
Keywords: compressive optic neuropathy; craniosynostosis; frontal advancement surgery; optic atrophy; oxycephaly; proptosis.
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