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01.12.2014 | Original Research Article | Ausgabe 3/2014

Documenta Ophthalmologica 3/2014

Ten-year follow-up of two unrelated patients with Müller cell sheen dystrophy and first report of successful vitrectomy

Zeitschrift:
Documenta Ophthalmologica > Ausgabe 3/2014
Autoren:
Agnes B. Renner, Viola Radeck, Ulrich Kellner, Herbert Jägle, Horst Helbig

Abstract

Purpose

To describe clinical characteristics of Müller cell sheen dystrophy (MCSD) in two unrelated patients followed for 10 years.

Methods

Best-corrected visual acuity (BCVA), kinetic perimetry, biomicroscopy, ophthalmoscopy, fundus photography, fluorescein angiography, fundus autofluorescence, near-infrared reflectance, optical coherence tomography (OCT), and electroretinography (ERG).

Results

Case 1: A 61-year-old woman showed internal limiting membrane (ILM) folds at the posterior pole (OU), and cystoid macular edema (CME) in OD. During follow-up, BCVA decreased from 0.2 to 0.06 (OD) and from 0.7 to hand movements (OS). Fundus presented fluctuant CME and subretinal fluid, and an increase in ILM folds and intraretinal schisis cavities. ERG was negative in OD and initially normal in OS. Case 2: A 60-year-old man was first diagnosed with epiretinal membrane before MCSD with ILM folds was detected. OCT showed schisis cavities in all retinal layers. After vitrectomy with ILM peeling in OD because of visual loss and massive CME, BCVA recovered from 0.05 to 0.4. BCVA in OS remained at 0.6. OD developed negative ERG.

Conclusions

MCSD presents with late onset, ILM folds, intraretinal schisis cavities, and negative ERG. Visual loss is accompanied by CME and subretinal fluid. Vitrectomy with ILM peeling led to BCVA increase and anatomic improvement.

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