Long-term Evaluation of Endothelial Cell Changes in Fuchs Corneal Dystrophy: The Influence of Phacoemulsification and Penetrating Keratoplasty

PURPOSE: To evaluate the natural course of the long-term endothelial cell changes in Fuchs corneal dystrophy (FCD) patients and investigate the effects of phacoemulsification on the annual rate of change in endothelial indices in FCD patients. METHODS: Thirty-four patients diagnosed with FCD at Seoul National University Hospital from 1994 to 2010 were retrospectively reviewed. Sixteen patients who had been followed up for more than 1 year were selected and classified into 3 groups: group A, patients with no ocular surgery; group B, patients who had undergone phacoemulsification only; and group C, patients who had undergone penetrating keratoplasty with cataract surgery. Endothelial cell density, polymegethism, pleomorphism, and pachymetry were measured and the exponential rates of endothelial cell and pachymetry change were analyzed. RESULTS: A non-linear mixed model of non-operated FCD patients showed that only pachymetric data tended to increase with statistical significance (p = 0.001) with a mean follow-up period of 4.15 years. Using an exponential regression analysis fitting curve, the mean rates of annual endothelial cell loss were 0.82%/yr, 20.39%/yr, and 29.27%/yr in groups A, B, and C respectively, and statistical significance was seen only in group C (p < 0.05). CONCLUSIONS: Retrospective long-term follow-up data showed that changes in endothelial density did not significantly decrease over at least 4 years in middle-aged FCD patients. The changes in pachymetric corneal thickness appeared to increase over the same period. Considering that no exponential changes were aggravated after performing cataract surgery alone, cataract surgery would be a preferable option in FCD patients compared to an approach of "wait-and-do" penetrating keratoplasty combined with cataract surgery.

Bilateral dissociated vertical deviation in a case of congenital hereditary endothelial dystrophy.

Dissociated vertical deviation (DVD) is an intermittent anomaly of the non-fixing eye. Although association of DVD with sensory visual deprivation owing to congenital or acquired opacities of the ocular media has been reported, its association with congenital hereditary endothelial dystrophy (CHED) has not been reported hitherto. We report a case having a bilateral asymmetric DVD, in a know case of bilateral CHED.

Central corneal endothelial guttae and age-related macular degeneration: is there an association?

The similarities between the corneal endothelium and retinal pigment epithelium in terms of their embryology, barrier function and predilection to age-related degeneration prompted this investigation into a possible association between central corneal guttae (CCG) and age-related macular degeneration (ARMD). 50 patients with clinically significant CCG were prospectively evaluated for the presence of ARMD. 51 age-matched patients attending for unrelated ailments who did not have CCG were also evaluated for the presence of drusen and other signs of ARMD. Of the 50 patients with CCG, 23 had bilateral ARMD and 4 had unilateral ARMD. In the control group, 9 patients had bilateral and 4 had unilateral ARMD. There was significant difference in the prevalence of ARMD between patients with CCG and those with no CCG (p = 0.017 and p < 0.001 for right and left eyes respectively). We found an association between CCG and ARMD. The presence of CCG in a patient may imply increased risk for the presence of ARMD. In a patient with CCG requiring cataract or corneal surgery, the successful outcome may be compromised by the presence of ARMD.

Ceratocone associado à distrofia endotelial de Fuchs: relato de um caso / Keratoconus associated with Fuchs'endothelial dystrophy: a case report

O ceratocone encontra-se associado a muitas doenças oculares e sistêmicas. Descreve-se caso da paciente de 42 anos de idade, com ceratocone bilateral e distrofia endotelial de Fuchs bilateral. A paciente foi submetida à avaliação corneana através de: biomicroscopia, paquimetria, topografia corneana computadorizada, ceratoscopia, microscopia especular, e ceratoestesiometria, além de outros procedimentos oftalmológicos relevantes. A biomicroscopia mostrou córnea guttata e ectasia centrais. A paquimetria exibiu afinamento corneano central. A topografia corneana demonstrou astigmatismo irregular clássico com encurvamento da meia-periferia inferior. A ceratoestesiometria foi compatível com a diminuição da sensibilidade corneana. A microscopia especular mostrou córnea guttata avançada com áreas com perda completa do padrão do mosaico endotelial. Os achados nesta paciente confirmam observações anteriores de que o ceratocone e a distrofia endotelial de Fuchs podem ser coincidentes, embora não se possa concluir qualquer padrão de herança baseado nesta avaliação limitada.