Inferior Spear-like Lens Opacity as a Sign of Keratoconus

PURPOSE: To report 21 cases of typical inferior feather-shape lens opacity associated with keratoconus. METHODS: In this cross-sectional study, we evaluated the association of keratoconus with inferior feather-shape lens opacity in refractive surgery candidates. Visual acuity, demographic, refractive, and topographic characteristics of 26 eyes of 21 patients with inferior feather-shape lens opacity were evaluated in detail. Pedigree analysis was also performed to assess possible inheritance. RESULTS: Overall, 2122 out of 33,368 cases (6.4%) without lens opacity had keratoconus, while 20 out of 21 patients (95.2%) with peculiar lens opacity had definite keratoconus (P [Formula: see text] 0.001). Lens opacity was bilateral in 5 cases (24%), and keratoconus was bilateral in all 20 patients with lens opacity. Nine eyes out of thirty-six with a complete data record (25%) had a severe keratoconus and underwent deep lamellar keratoplasty, while 11 (31%) had forme fruste keratoconus. Pedigrees were drawn for eight patients, most families of whom suggested an X-linked recessive inheritance. CONCLUSION: The present study was the first to investigate patients with a peculiar inferior feather-shape lens opacity accompanied by bilateral keratoconus, which was observed in 95% of the patients. This finding should raise awareness as to the possibility of diagnosing keratoconus in the eyes of the patients with these characteristics.