Abstract
<title>Abstract</title> <p> <bold>Purpose:</bold> To report a case of acute corneal hydrops (ACH) as the initial presenting manifestation of keratoconus (KC) and to systematically review the published literature on this presentation. <bold>Methods:</bold> A new case is reported: a 16-year-old female who developed unilateral ACH as the first manifestation of KC, in the absence of classical risk factors, approximately one year after receiving mild refractive correction. A systematic review was conducted according to PRISMA guidelines. Searches were performed across PubMed/MEDLINE, Scopus, Google Scholar, and Web of Science from database inception to March 2026. Studies were included if ACH was explicitly described as the first clinical manifestation of KC. Due to the heterogeneity of available data and the predominance of case-level evidence, a formal meta-analysis was not feasible; a pooled descriptive analysis was performed. <bold>Results:</bold> After screening, 10 studies met inclusion criteria, comprising 2 observational cohort studies and 8 case reports or series. Presenting hydrops was consistently associated with younger age, advanced KC, and the absence of a prior KC diagnosis. Frequent association with vernal conjunctivitis, allergic disease, syndromic conditions, or developmental vulnerability were recurrent themes. <bold>Conclusion:</bold> ACH may represent the initial clinical manifestation of KC, typically reflecting previously unrecognized ectasia. This presentation may occur even in the absence of classical risk factors. </p>