Introduction: We present a case of a patient with osteogenesis imperfecta (OI) and keratoglobus (KG) who had a near-total rupture of Descemet's membrane followed by spontaneous corneal clearing. This case is unique in that it demonstrates the potentially excellent outcome of conservative treatment for Descemet's rupture in patients with KG and illustrates the impressive migratory potential of healthy endothelial cells.
Case presentation: An 11-year-old girl with OI and KG who had rupture and near-total detachment of Descemet's membrane presented for evaluation. This was managed conservatively and resulted in the eventual spontaneous clearing of the cornea. A similar process happened in the fellow eye some years later. Given the result of conservative management originally, the patient was once again treated conservatively, with significant improvement in corneal edema and visual acuity.
Conclusion: Given the size of the ruptures, this case highlights the dynamic nature of the corneal endothelium and provides an extreme example of the migratory potential of corneal endothelial cells.
Keywords: Corneal ectasia; Descemet rupture; Hydrops; Keratoglobus; Osteogenesis imperfecta.
© 2024 The Author(s). Published by S. Karger AG, Basel.