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ABSTRACT Purposes: This study aims to assess and compare the postoperative visual and topographic outcomes, complications, and graft survival rates following deep anterior lamellar keratoplasty and penetrating keratoplasty in patients with macular corneal dystrophy. Methods: In this study we enrolled 59 patients (23 male; and 36 female) with macular corneal dystrophy comprising 81 eyes. Out of these, 64 eyes underwent penetrating keratoplasty, while 17 eyes underwent deep anterior lamellar keratoplasty. The two groups were analyzed and compared based on best-corrected visual acuity, corneal tomography parameters, pachymetry, complication rates, and graft survival rates. Results: After 12 months, 70.6% of the patients who underwent deep anterior lamellar keratoplasty (DALK) and 75% of those who had penetrating keratoplasty (PK) achieved a best-corrected visual acuity of 20/40 or better (p=0.712). Following surgery, DALK group showed lower front Kmean (p=0.037), and Q values (p<0.01) compared to the PK group. Postoperative interface opacity was observed in seven eyes (41.2%) in the DALK group. Other topography values and other complications (graft rejection, graft failure, cataract, glaucoma, microbial keratitis, optic atrophy) did not show significant differences between the two groups. The need for regrafting was 9.4% and 11.8% in the PK and DALK groups, respectively (p=0.769). Graft survival rates were 87.5% and 88.2% for PK and DALK; respectively (p=0.88 by Log-rank test). Conclusion: Both PK and DALK are equally effective in treating macular corneal dystrophy, showing similar visual, topographic, and survival outcomes. Although interface opacity occurs more frequently after DALK the visual results were comparable in both groups. Therefore, DALK emerges as a viable surgical choice for patients with macular corneal dystrophy without Descemet membrane involvement is absent.
Résumé
Purpose: To compare the functional and anatomical outcomes (in terms of graft uptake and rejection/failure) of deep anterior lamellar keratoplasty (DALK) in stromal corneal dystrophy (macular and granular). Methods: Sixteen eyes with macular corneal dystrophy (MCD; group A) and 10 eyes with granular corneal dystrophy (GCD; group B) underwent successful DALK by big bubble technique or layer-by-layer dissection. Results: Both groups showed significant improvement in their best-corrected visual acuity postoperatively (postoperative P value in MCD and GCD was 0.00001 and 0.0008, respectively) with no statistically significant differences between the two groups (P = 0.77) at 1 year. Postoperative endothelial count did not drop significantly in group A (MCD, P= 0.1553). Only in seven eyes preoperative endothelial count could be obtained (due to dense stromal corneal opacity), but there was a significant endothelial count difference between preoperative and postoperative count in group B (GCD, P= 0.0405) at the end of 1 year postoperatively which could be because of age and stage of disease (advanced granular dystrophy) and also because of small sample size of GCD compared with MCD. Intergroup comparison between the two groups showed no statistically significant difference (P = 0.6353) with good postoperative outcome in both groups. Conclusion: DALK can be successfully done in both groups and results are comparable. However, long-term outcomes on a large scale need to be further evaluated.
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@#Macular corneal dystrophy(MCD)refers to corneal stromal dystrophy, which can cause progressive visual field defects. MCD belongs to autosomal recessive disease. Mutation of CHST 6 gene is considered to be a pathogenic factor. In general, full-thickness penetrating keratoplasty has become a standard treatment for improving vision. Now more advanced surgical interventions such as deep anterior lamellar keratoplasty(DALK)and phototherapeutic keratectomy(PTK)have also been shown to be important in the treatment of MCD. Some new technologies, such as gene targeting therapy and enzyme replacement therapy, are also being studied as potential permanent solutions for MCD. The pathogenesis, genetic basis, clinical manifestations, treatment and prognosis of this disease are reviewed in this paper.
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Macular corneal dystrophy is autosomal recessive dystrophy characterized by deposits of abnormal glycosaminoglycans in stromal lamellae and within endothelial cells. Deep anterior lamellar keratoplasty is successful in the management of this dystrophy. We herein describe three cases of primary graft failure after uneventful big bubble deep anterior lamellar keratoplasty for macular corneal dystrophy.
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Macular corneal dystrophy is a heredo-familial disease inherited as an autosomal recessive trait and characterized by corneal opacities due to localized mucopolysaccharidosis. One case of macular corneal dystrophy was reported by Cho et al in Korea in 1976, but it was a case without a family history. We report 3 cases of macular corneal dystrophy with a family history including more specific histopathologic findings and the review of available literatures.