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NR 1-2/2004
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Dystrofia plamkowata
rogówki z uwzględnieniem zmian histopatologicznych i
ultrastrukturalnych
Description of macular
corneal dystrophy including histological and ultrastructural
changes
Edward Wylęgała1, Ewa Marta
Wróblewska1, Henryk Grzybek2,
Krzysztof Januszewski3, Mirosława
Przeworowska-Kawala2, Wojciech Bany¶1,
Dariusz Dobrowolski1, Antonina Jurewicz1
1 Z Oddziału Okulistycznego Okręgowego Szpitala
Kolejowego w Katowicach
Ordynator: dr n. med. Edward Wylęgała
2 Z Zakładu Mikroskopii Elektronowej ¦l±skiej
Akademii Medycznej Katowicach
Kierownik: dr hab. n. med. Henryk Grzybek
3 Z Pracowni Anatomopatologicznej Okręgowego Szpitala
Kolejowego w Katowicach
Kierownik: dr n. med. Krzysztof Januszewski |
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| Summary: |
Purpose: The study
reports the results of a histological and
ultrastructural examination of the corneal button,
obtained during penetrating keratoplasty from patient
with clinically recognized macular corneal dystrophy.
Material and methods: 34-year-old male patient
suffering from macular corneal dystrophy (MCD) has been
treated on corneal epithelium defect and photophobia
since his early childhood. Visual acuity was decreased
on the Snellen test chart to 0,02. Slit-lamp examination,
and ultrasonographical measurement of the cornea's
thickness were performed. Removed during penetrating
keratoplasty corneal button was divided into two pieces.
One of them was prepared in standard procedure for
histological examination in the light microscopy after
having been stained with hemotaxylin and eosin, alcian
blue and paS – method. From the other part, slides for
ultrastructural examination in the transmission electron
microscopy were prepared with the use of standard method.
The family history from the patient was also taken, and
available relatives have undergone examination in search
of typical MCD symptoms.
Results: Slit-lamp examination findings revealed
diffuse, from limbus to limbus, stromal opacification.
In measurement by pachymeter cornea's thickness was
reduced. In the light microscopy, in typical stained
slides, delaminations within stroma and deficit of
endothelial cells were observed. After being stained
with alcian blue, dark blue deposits in the places of
delamination became visible. By transmission electron
microscopic examination, intracellular and extracellular
deposits were detected in the stroma, Descemet membrane
and endothelium. Distended keratocytes with enormous
vacuoles containing abnormal material were found.
Pedigree was typical for autosomal recessive inherited
disease.
Conclusions: Histological and ultrastructural
diagnosis is a basis of recognition of macular corneal
dystrophy. Analysis of the pedigree as well as
ultrasonographical measurement of the cornea's thickness
is very helpful to establish the right diagnosis. |
| Słowa kluczowe: |
dystrofia plamkowata
rogówki, ultrastruktura, histopatologia. |
| Key words: |
macular corneal dystrophy,
ultrastructure, histopathology. |
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