Inverse Bell Phenomenon and Superior Keratoconus

To report 6 eyes of 4 patients with superior keratoconus and its occurrence in conjunction with inverse Bell phenomenon in which the eye rotates downward on eye closure.

A retrospective review and analysis was performed on all patients with keratoconus who presented to our cornea service between August 2022 and December 2025. Patient demographics, clinical examination findings, and Pentacam anterior segment tomography imaging (Oculus, Arlington, WA) were reviewed.

All patients with superior keratoconus had an inverse Bell phenomenon. A 27-year-old man (patient 1), a 15-year-old boy (patient 2), a 60-year-old man (patient 3), and a 29-year-old woman (patient 4) with clinical and topographic findings of keratoconus with a superior cone were identified. Patients 1 and 3 had unilateral superior keratoconus, and patients 2 and 4 had bilateral superior keratoconus. All patients initially denied eye rubbing, atopy, and family history of keratoconus; however, all patients slept on their side or face with their hands pressed against their eyes, and patients 1 and 3 had floppy eyelid syndrome.

Superior keratoconus and inverse Bell phenomenon are both rare ophthalmic findings alone and co-occur in these patients. The inverse Bell phenomenon exposes the superior corneal to mechanical pressure on lid closure and further supports the role of mechanical pressure on the development of keratoconus.