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Case Reports
. 2011 Sep-Oct;59(5):398-400.
doi: 10.4103/0301-4738.83624.

Late occurrence of granular dystrophy in bilateral keratoconus: penetrating keratoplasty and long-term follow-up

Varsha M Rathi  1 , Geeta K Vemuganti, Virender S Sangwan, Chitra Kannabiran
Affiliations
Case Reports

Late occurrence of granular dystrophy in bilateral keratoconus: penetrating keratoplasty and long-term follow-up

Varsha M Rathi et al. Indian J Ophthalmol. 2011 Sep-Oct.

Abstract

We report a rare case of keratoconus with granular dystrophy with a follow-up of two decades, documenting the sequential presentation of two diseases confirmed by histology and genetic studies. A 13-year-old boy was diagnosed in 1988 with keratoconus in both eyes (BE) based on slit-lamp biomicroscopy findings of corneal ectasia in BE accompanied by Fleischer's ring, Vogt's striae, a small, old, healed hydrops. The left eye (LE) had central corneal thinning and scar in the central area involving the mid and posterior stroma secondary to healed hydrops. Penetrating keratoplasty (PKP) was advised. The boy was lost to follow-up till 1991 and presented with white, dot-like opacities in the central cornea in the RE only, suggestive of granular corneal dystrophy. Similar findings of white, dot-like opacities were noted in the LE in 1995 and the patient subsequently underwent PKP in BE. Histopathology of corneal buttons confirmed the presence of patchy, crystal-like orange deposits, which stained bright red with Masson's trichrome. Mutational analysis of the TGFBI gene in patient's DNA revealed a heterozygous mutation corresponding to a change in Arg555Trp in the keratoepithelin protein. Granular dystrophy recurred after 8 years in the RE.

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Figures

Figure 1
Figure 1
Right eye – the diffuse photomicrograph shows the Fleischer's ring and multiple flakes in the central cornea within the cone with clear areas in between and limbus is spared. This photograph was taken at the age of 17 years
Figure 2
Figure 2
The flakes typical of granular corneal dystrophy as seen in the left eye of the patient with a dense scar posthydrops. This photograph was taken at the age of 21 years
Figure 3
Figure 3
The flakes can be seen in the center of the graft with clear areas in between suggestive of the recurrence of granular corneal dystrophy. Note the buried sutures
Figure 4
Figure 4
(a) Section of the cornea shows bright-red crystalline deposits in the stroma characteristic of granular dystrophy. (b) The basal cells show intracytoplasmic Prussian blue reaction with Perl's stain, confirming the presence of iron deposits, a characteristic feature of keratoconus
See this image and copyright information in PMC

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