Fuchs endothelial corneal dystrophy: clinical characteristics of surgical and nonsurgical patients

Abstract

PurposeTo review the patient and clinical characteristics of patients with Fuchs endothelial corneal dystrophy (FECD).MethodsReview of records for every patient who presented to the Bascom Palmer Eye Institute between 2003 and 2009 whose visit was coded for endothelial corneal dystrophy (International Classification of Diseases, Ninth Revision [ICD9] 371.57), bullous keratopathy (ICD9 371.23), or who underwent a corneal surgery with or without cataract extraction. Demographic, clinical, and ancillary testing data were collected from the time of presentation, diagnosis, and follow-up, and the use, timing, and type of surgical interventions was documented, with 6-month and final visual acuities recorded.ResultsA total of 2,370 charts were included in this study, of which 966 patients had a diagnosis of FECD. Of these, 197 patients (21%) received a corneal transplantation procedure. The surgery most often performed was penetrating keratoplasty with or without cataract extraction (66%), followed by endothelial keratoplasty with or without cataract extraction (34%). The risk factors for surgery include worse visual acuity at presentation (20/60 Snellen visual acuity in surgical patients versus 20/40 Snellen visual acuity in nonsurgical patients, P<0.001), greater average central corneal thickness (635 μm versus 592 μm, P<0.001), loss of visual acuity over time (two lines lost versus zero lines lost, P<0.001), increasing age (P<0.001), and male sex (P=0.008). Over half of patients (52%) did not receive surgery despite poor vision.ConclusionDuring this time period, FECD did not have a consistent pattern for management or treatment, and despite advances in surgical techniques, most patients were still managed without surgery.

Superficial keratectomy as a treatment for non‐healing corneal ulceration associated with primary corneal endothelial degeneration

“…Some studies assessing a relatively small study population have shown a sex predilection in female dogs; however, this finding has not been consistent across all studies . Women are reported to be at higher risk than men for developing Fuchs’ endothelial dystrophy and, as this human disorder shares many similarities to canine PCED, affected dogs have been suggested as a potential study model for the human disease . The sex distribution in our study involving 238 PCED‐affected dogs failed to show a significant sex predisposition with 40.8% female dogs and 59.2% male dogs.…”

“…7,10 Women are reported to be at higher risk than men for developing Fuchs' endothelial dystrophy and, as this human disorder shares many similarities to canine PCED, affected dogs have been suggested as a potential study model for the human disease. 6,7,39 The sex distribution in our study involving 238 PCED-affected dogs failed to show a significant sex predisposition with 40.8% female dogs and 59.2% male dogs. The age of diagnosis of 11.4 years in this study is largely consistent with previous studies showing that PCED is generally a disease of older dogs.…”

In Vivo Imaging of Corneal Endothelial Dystrophy in Boston Terriers: A Spontaneous, Canine Model for Fuchs' Endothelial Corneal Dystrophy

“…By contrast, human patients with endothelial decompensation from FECD typically exhibit more subtle increases in CCT of approximately 10% to 50% versus normal individuals. 33 This difference in CCT between canine CED and human FECD affected patients is likely multifactorial. First, key differences in corneal structure exist between humans and dogs.…”

“… 35 – 37 Second, it is likely that human patients with mild corneal edema exhibit decreases in visual acuity that prompt rapid clinical evaluation and subsequent surgical intervention. 33 By contrast, owners of dogs with canine CED are less likely to seek consultation with a veterinary ophthalmologist or choose a surgical intervention until late in the disease course due to blindness or ocular discomfort from recurrent corneal ulceration. 9 , 38 …”

Long-term Quantitative and Qualitative Assessment of the Corneal Endothelium after Anterior Radial Keratotomy

“…2. Единичные (А), множественные (B) и сливные (C) темные зоны, обнаруженные при эндотелиальной микроскопии роговицы пациентов после кератотомии Cornea guttata является предшественницей эпителиально-эндотелиальной дистрофии роговицы, которая на начальной стадии протекает бессимптомно [16,17]. Однако эндотелиальные нарушения (снижение плотности эндотелиальных клеток, полимегатизм и плеоморфизм) могут быть выявлены за несколько лет до появления у пациента развернутой клинической картины дистрофии [15][16][17].…”