The “Silent Brain Syndrome” Creating a Severe Form of the “Giant Fornix Syndrome”

Abstract

To describe the clinical course of a patient requiring ophthalmic care for entropion and fungal keratitis in the setting of undiagnosed enophthalmos after previous ventriculoperitoneal shunting consistent with silent brain syndrome. Case report. A 33-year-old man who had a history of ventriculoperitoneal shunting for an encephalocele during infancy presented because of ocular irritation and entropion, which was presumed to be entirely due to a chemical injury he had sustained 2 years before. He underwent 2 upper eyelid entropion repairs and developed fungal aspergilloma keratitis in the postoperative period. He underwent 2 penetrating keratoplasties and a limbal stem cell transplant but had complications with reinfection of the graft, eventually leading to a prephthisical painful eye. During enucleation, an aspergilloma was found within an enlarged superior fornix. Computed tomographic scan revealed severe enophthalmos with air between the lids and the globe, which was consistent with silent brain syndrome. In patients with silent brain syndrome, the lack of apposition between the eyelids and the globe results in entropion, trichiasis, lagophthalmos, and ocular irritation. This can complicate entropion repair and the severity of infectious keratitis. We also propose that the enlarged fornices seen in silent brain syndrome can also serve as a reservoir for infection, similar to the pathogenesis seen in the giant fornix syndrome.