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


CONCLUSIONS
The ultrastructure suggested that retinal glial cells had induced a remodeling of the collagenous stroma in the posterior vitreous cortex, resulting in tangential macular traction.
Inoue
Makoto
M
Department of Ophthalmology, Keio University, 35 Shinanomachi, Shinjuku-ku, 160-8582, Tokyo, Japan. inoshin@sc.itc.keio.ac.jp
Yamazaki
Kazuto
K
Shinoda
Kei
K
Ishida
Susumu
S
Shinoda
Hajime
H
Noda
Kousuke
K
Oguchi
Yoshihisa
Y
D001485:Basement Membrane / Q000648:ultrastructure; D019773:Epiretinal Membrane / Q000473:pathology*; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008875:Middle Aged; D012167:Retinal Perforations / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D041623:Tomography, Optical Coherence; D014821:Vitrectomy; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology* / Q000601:surgery