The early history of keratoconus prior to Nottingham's landmark 1854 treatise on conical cornea: a review



In an era of scientific method, precision of nomenclature and an electronically accessible publication record, the early history of keratoconus still remains, in parts, as complex and perplexing as the disease itself. Historically, the disease has been somewhat confusingly referred to by several different terms, including hyperkeratosis, ochlodes, conical formed cornea, cornea conica, cornée conique, sugar loaf cornea, prolapses corneae, procidentia corneae, staphyloma transparent de la cornée, staphyloma pellucidum, staphyloma corneae totale conicum pellucidum, staphyloma diaphanum, keratconus and keratoconus. In his major 1854 treatise on conical cornea, John Nottingham is widely cited as the first author to succinctly define keratoconus and its associations; however, for 150 years prior to this landmark publication, others had been slowly deciphering elements of keratoconus and distilling their knowledge in a series of publications obscured by the passage of years. Uncritical re-writing of core information and references without their full verification has also led to confusion in the published literature in the 150 years since Nottingham's comprehensive description of keratoconus. In the light of the preceding limitations in the established literature, the authors conducted an historical survey, based on the analysis of original sources, to more fully establish the chronology of early descriptions of keratoconus prior to 1854, with particular focus on the works of Duddell, Taylor, Mauchart, Scarpa, Wardrop, Lyall, MacKenzie, Lawrence, Schmidt, von Ammon and Pickford. This review attempts to place the observations of these practitioners and others both in the context of contemporary ophthalmic practice and historical precedent.