Effect of intravitreal dexamethasone on macular edema in von Hippel-Lindau disease assessed using swept-source optical coherence tomography: A case report

Angelo Maria Minnella, Valeria Pagliei, Martina Maceroni, Matteo Federici, Gloria Gambini, Aldo Caporossi

Research output: Contribution to journalArticle

Abstract

Background: Von Hippel-Lindau disease is a rare hereditary syndrome caused by germinal mutations in a von Hippel-Lindau tumor-suppressing gene. Retinal hemangioblastoma is the ocular hallmark lesion of von Hippel-Lindau disease. Case presentation: A 20-year-old Caucasian woman presented to our institution with painless visual impairment in the right eye. A fundus ophthalmoscopic evaluation and swept-source optical coherence tomographic examination revealed a retinal hemangioblastoma associated with cystoid macular edema. On the basis of the clinical ocular findings and genetic analysis, von Hippel-Lindau disease was diagnosed. Following an intravitreal injection of ranibizumab, off-label administration of intravitreal dexamethasone was considered to reduce the edema. An almost complete resolution of the edema in the macular area was observed 1 week after the injection. Finally, laser photocoagulation and transconjunctival cryotherapy were performed; the patient developed "ablatio fugax" after cryotherapy. Conclusions: In our experience, intravitreal dexamethasone administration has proven to be a useful tool for reducing retinal hemangioblastoma-related macular edema in von Hippel-Lindau disease and may be considered a potentially valuable treatment that can be used in combination with other therapies.

Original languageEnglish
Article number248
JournalJournal of Medical Case Reports
Volume12
Issue number1
DOIs
Publication statusPublished - Sep 6 2018

Keywords

  • En face OCT
  • Innovative biotechnology
  • Macular edema
  • Retinal hemangioblastoma
  • Swept-source OCT
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Medicine(all)

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