Hydrops as the presenting sign and “inverted bell sign” as a diagnostic sign of atypical pellucid marginal degeneration

Abstract

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. A 40-year-old male presented to us with acute pain, redness, and loss of vision. On examination, hydrops was noted in the superior cornea in the right eye and advanced PMD in the left eye. The typical topographic signs of PMD have been described. The conservative management of hydrops in our case resulted in improvement in visual acuity. Early diagnosis and treatment of this condition with regular follow-up are essential for a good visual prognosis in these patients.

Keywords

• atypical pellucid marginal degeneration
• bell sign
• hydrops
• inverted bell sign