Abstract
P 038
15 year follow-up of a patient with gelatinous drop-like corneal dystrophy
Constantin E. Uhlig1, Markus Groppe2, Holger Busse1, Wolfgang Saeger3
1Klinik und Poliklinik für Augenheilkunde, Universitätsklinikum Münster, Münster; 2Victoria Eye Unit Hereford Hospital, Union Walk, Herefordshire, Hereford, United Kingdom; 3Institut für Pathologie, Marienkrankenhaus Hamburg
Objective
Gelatinous drop-like corneal dystrophy (GDLD) is a bilateral, hereditary autosomal-recessive, primary corneal dystrophy and etiological treatment is still not possible. The amyloid is presumably synthesized in the limbal area and deposits of amyloid frequently are found in the corneal subepithelium and within the stroma. We report of a 13-year old female patient of turkish origin that presented to our hospital with GDLD.
Methods
Ophthalmological examination of the family, chronologic analysis of the visual acuity and the 197 photodocumentations performed over 15 years. Three superficial corneal biopsies and six penetrating keratoplasties were analysed using histological staining, immunocytochemistry and electron microscopy.
Results
Before and after surgical interventions, the drops developed most often at the same locations and preferentially near to limbally accentuated neovascularisations and at the suture points. In the limbal area, they followed a circumferential pattern whereas the changes on the donor graft appeared first at the periphery and then moved to the centre. Gelatinous deposits were found subepithelially as well as in the corneal stroma. Only transient relief was achieved with local treatment, and disease progression could not be stopped with topical administration of antibiotics or local or systemic steroids. A centrally performed excimer laserphotoablation induced continuous corneal intransparency, but six perforating keratoplasties (three left and three right eye) resulted in improvement of visual acuity (maximally 4 to 8 lines) over a period of 16 to 79 months (average 2 years + 8,5 months).
Conclusions
Lamellar or perforating keratoplasties resulted in transient relief and improvement of visual acuity but did not definitely stop disease progression. It is not known how the amyloid synthesizing cells will behave in case of a partial destruction, but a limbokeratoplasty or limbal transplantation might be a more useful option for longer-term treatment of GDLD.
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