Documenta Ophthalmologica

, Volume 129, Issue 3, pp 191–202 | Cite as

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

  • Agnes B. Renner
  • Viola Radeck
  • Ulrich Kellner
  • Herbert Jägle
  • Horst Helbig
Original Research Article



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


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).


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.


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.


Cystoid macular edema Internal limiting membrane Müller cell sheen dystrophy Negative electroretinogram Optical coherence tomography Pars plana vitrectomy 


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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Agnes B. Renner
    • 1
    • 2
  • Viola Radeck
    • 1
  • Ulrich Kellner
    • 2
    • 3
    • 4
  • Herbert Jägle
    • 1
  • Horst Helbig
    • 1
    • 2
  1. 1.Klinik und Poliklinik für AugenheilkundeUniversitätsklinikum RegensburgRegensburgGermany
  2. 2.Klinik und Poliklinik für AugenheilkundeCharité Universitätsmedizin Berlin, Campus Benjamin FranklinBerlinGermany
  3. 3.RetinaScienceBonnGermany
  4. 4.Zentrum für seltene NetzhauterkrankungenAugenZentrum Siegburg, MVZ ADTC Siegburg GmbHSiegburgGermany

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