Purpose

To report a case of Orbital Apex Syndrome (OAS) where a chronic, non-invasive fungal ball precipitated an acute, irreversible ischaemic infarction of the orbital apex in a patient with Myelodysplastic Syndrome (MDS).

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Methods

A 76-year old male with high-risk MDS (ASXL1, DDX41 mutations) presented with acute proptosis, complete ophthalmoplegia, and vision loss (PL). CT showed isolated sphenoid opacification. Urgent endoscopic sphenoidotomy revealed fungal debris, but mucosa was macroscopically healthy. Histopathology confirmed Aspergillus fumigatus with no evidence of tissue invasion.
Despite prompt source control and maximalist medical therapy (IV antibiotics and antifungals), vision did not recover. Post-operative imaging confirmed a superior ophthalmic vein (SOV) thrombosis adjacent to a dehiscent clinoid wall.
Therapeutic anticoagulation for the SOV thrombosis was mandated but became impossible due to refractory thrombocytopaenia, however clinical failure was attributed to an established ischaemic infarction occurring at the point of presentation.
Emergent nerve decompression was considered but deferred as the pathology was identified as primarily ischaemic/thrombotic.

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Results

The failure of clinical recovery is attributed to an established primary vascular event at the orbital apex occurring prior to, or at the point of, presentation. Post-operative imaging confirmed SOV thrombosis adjacent to a dehiscent clinoid wall. This suggests a mechanism of either septic thrombophlebitis or reactive inflammatory vasculitis. In the context of the MDS, this “trans-venous” inflammatory spread likely triggered a catastrophic ischaemic infarction, predating surgical source control.

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Conclusion

In the context of the immunocompromised host, OAS may transition rapidly from a compressive phenomenon to a primary vascular event.
Clinicians should recognise that in this specific patient cohort, the window for functional salvage may be dictated by an early “ischaemic switch”, where source control or decompression cannot reverse neurovascular infarction.

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Additional Authors

First name	Last name	Base Hospital / Institution
Issa	Beegun	Imperial College Healthcare NHS Trust

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