A clinicopathologic case report on macular hole associated with von Hippel-Lindau disease: A novel ultrastructural finding of wormlike, wavy tangles of filaments

Makoto Inoue, Kazuto Yamazaki, Kei Shinoda, Susumu Ishida, Hajime Shinoda, Kousuke Noda, Yoshihisa Oguchi

Research output: Contribution to journalArticle

9 Citations (Scopus)


Background: We describe a novel ultrastructural finding observed in an epiretinal membrane excised from a patient with von Hippel-Lindau disease. Methods: This interventional case report presents a 45-year-ol d woman who noted decreased vision in her right eye and was referred for treatment of a macular hole secondary to von Hippel-Lindau disease. Blindness had occurred previously in the left eye because of exudative retinal detachment. Funduscopic examination revealed a macular hole associated with a delicate epiretinal membrane, multiple retinal angiomas, and multiple old laser scars. Vitrectomy was performed in her right eye. The epiretinal membrane was peeled off and processed for light and electron microscopy. Results: Postoperatively the macular hole was successfully closed, and vision recovered to 20/20 from 20/40. In the excised epiretinal membrane, flattened glial cells and extracellular matrix containing fibrous elements formed a multilayered pattern. Ultrastructurally, the fibrous elements appeared as wormlike, wavy tangles of filaments (WWTF) with an orientation parallel to the surface of the epiretinal membrane. Just above the WWTF, flattened glial cells frequently extended thin cytoplasmic processes with pinocytotic vesicles. Conclusion: The ultras tructure suggested that retinal glial cells had induced a remodeling of the collagenous stroma in the posterior vitreous cortex, resulting in tangential macular traction.

Original languageEnglish
Pages (from-to)881-886
Number of pages6
JournalGraefe's Archive for Clinical and Experimental Ophthalmology
Issue number10
Publication statusPublished - 2004 Oct 1


ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this