Abstract
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Background: Spontaneous cerebrospinal fluid (CSF) leakage into the orbit is exceptionally rare, particularly when no identifiable structural defect is present. Its clinical manifestations and potential routes of CSF passage remain incompletely understood. We describe an unusual presentation associated with idiopathic intracranial hypertension (IIH) and explore a possible peri-optic anatomical pathway for orbital CSF accumulation.
Case presentation: A 48-year-old woman with IIH presented with unilateral conjunctival chemosis, proptosis and choroidal detachment. Analysis of subconjunctival fluid was positive for β-2 transferrin, confirming the presence of CSF. Extensive neuroimaging identified no osseous or meningeal defect, vascular malformation or mass lesion to explain the fluid accumulation. Clinical improvement followed conservative management with intracranial pressure-lowering therapy.
Discussion: Spontaneous CSF leaks are generally considered in the context of an identifiable communication or defect, yet none was demonstrated in this patient. We propose that chronically elevated intracranial pressure may contribute to focal weakening or microscopic disruption of peri-optic meningeal or adjacent connective tissues. This could permit CSF to track along the path of least resistance via the optic nerve sheath into the orbital and sub-Tenon’s spaces. The proposed pathway is hypothetical and cannot be confirmed by the available imaging; nevertheless, it offers a potential explanation for the unusual combination of orbital and ocular findings. To our knowledge, this constellation of manifestations without a demonstrable structural defect has not previously been described, and the proposed soft-tissue pathway remains largely unexplored in the literature.
Conclusion: This case highlights an unusual ocular presentation of IIH-associated orbital CSF leakage and suggests that an occult peri-optic communication should be considered when unexplained orbital fluid accumulation occurs despite negative structural imaging.
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