![]() ![]() The best-corrected vision in the right eye was 20/200 the left eye was 20/25 with-6Dcylinder at 75° He has been using glasses for the past 2 years. He had no history of eye rubbing or contact lens wear in the past. We present a case of a 40-year-old male with acute hydrops following PMD in an atypical location as the initial presenting sign.Ī 40-year-old male patient presented to us with a sudden onset of pain, redness, and defective vision in the right eye for the past 3 days. Acute hydrops occurs more commonly in keratoconus (KC) than PMD. Rarely, patients present with acute hydrops and/or spontaneous corneal perforation. Patients with PMD typically complain of gradual, progressive loss of vision or long-standing poor visual acuity, due to irregular against the rule astigmatism with onset in the third to fourth decade of life. It mostly involves the inferior quadrant in a crescentic fashion however, literature evidence has shown involvement in the superior,, temporal, and nasal quadrant of the cornea. Pellucid marginal degeneration (PMD) is an idiopathic, ectatic disorder of the peripheral cornea. Hydrops as the presenting sign and “inverted bell sign” as a diagnostic sign of atypical pellucid marginal degeneration. How to cite this URL: Anitha V, Ravindran M, Ghorpade A. How to cite this article: Anitha V, Ravindran M, Ghorpade A. ![]() Keywords: Atypical pellucid marginal degeneration, bell sign, hydrops, inverted bell sign Early diagnosis and treatment of this condition with regular follow-up are essential for a good visual prognosis in these patients. The conservative management of hydrops in our case resulted in improvement in visual acuity. The typical topographic signs of PMD have been described. On examination, hydrops was noted in the superior cornea in the right eye and advanced PMD in the left eye. ![]() A 40-year-old male presented to us with acute pain, redness, and loss of vision. To report a case of, atypical pellucid marginal degeneration (PMD) with hydrops and to describe the inverted bell sign as the diagnostic feature in topography. ![]()
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