Exploring Visual Outcomes in Late-Presenting Multisuture Craniosynostosis: A Case Report

Abstract

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.

Figure 1. Clinical photograph of the patient (A) showing a high, peaked forehead (red arrow) and a shortened, pointed head shape characteristic of oxycephaly. Frontal view of the 3D CT reconstruction of the skull (B) highlights a significantly elevated and pointed cranial vault, with blue arrows indicating areas of abnormal suture fusion. Lateral view of the 3D CT reconstruction (C) further emphasizes the vertical elongation and foreshortening of the skull (green arrows).

Figure 2. Fundus photograph showing pale disc with peripapillary infarction in both eyes (A and B).

Figure 3. CT brain demonstrating a copper-beaten appearance of the skull, indicative of chronic raised intracranial pressure (ICP) with prominent impressions (arrows) of brain gyri on the inner table of the skull (A), T2-weighted gadolinium-enhanced MRI (CEMRI) of the brain and orbit showing severe optic nerve sheath oedema (arrows) (B), and Magnetic resonance venography (MRV) revealing collateral venous pathways and venous shunting (arrow), indicative of chronic intracranial hypertension and compensatory venous changes (C).

CT: computed tomography; CEMRI: contrast enhanced magnetic resonance imaging.

Figure 4. 3D reconstructed CT images of the skull following frontal orbital advancement and cranial vault remodeling (red arrows), showing the expanded anterior and posterior cranial vaults with evidence of surgical remodeling and stabilisation (A and B), axial CT image displaying the intracranial view of the cranial vault after surgical intervention, demonstrating craniotomy opening (asterisks) (C), and clinical photograph of the postoperative head showing the healed surgical site with evidence of scalp integrity restoration (yellow arrow) and no significant external deformity (D).

Figure 5. Fundus photographs of both eyes at the one-year follow-up demonstrate a decrease in optic disc pallor with a slight shift toward a pinkish hue, suggesting potential improvement in optic nerve perfusion following surgery (A, B).