title: Prevalence and histopathological characteristics of corneal stromal dystrophies in Saudi Arabia.
abstract: OBJECTIVE
  The aim was to determine the frequency and describe the main histopathologic features of corneal stromal dystrophy in Saudi Arabia.
  
  
  METHODS
  A single-center, retrospective analysis of 193 corneal specimens diagnosed with stromal dystrophy. All samples were retrieved from the Histopathology Department at King Khaled Eye Specialist Hospital over a 10-year period (2002 to December 31, 2011). Cases of stromal dystrophy undergoing keratoplasty were included in the study. Routine histopathologic stains and specific stains were used to determine a diagnosis. The corresponding demographic data and basic clinical/surgical information were collected via chart review.
  
  
  RESULTS
  The study sample was comprised of 193 eyes. The final diagnoses were macular corneal dystrophy (MCD) in 180 (93.26%) eyes, granular corneal dystrophy (GCD) in 9 (4.66%) and lattice corneal dystrophy (LCD) in 4 (2.07%) eyes. The mean age at presentation was 27.03 years for MCD, 26.33 years for GCD and 53.75 years for LCD. The interval between diagnosis and surgical intervention was not statistically different between the macular and granular groups (P = 0.141). There was a positive family history for the MCD (37.22%) and GCD (44.44%) groups. All eyes underwent penetrating keratoplasty (PKP) except 10 MCD cases that underwent lamellar keratoplasty. Diffuse stromal deposits were present in 87.2% of MCD corneas and 66.67% of GCD corneas. Seventeen eyes with MCD were misdiagnosed as GCD. None of the LCD cases were clinically identified since all of these cases were diagnosed as corneal scarring. In eyes with MCD that underwent PKP, there was diffuse stromal involvement (in 87.22% eyes) and changes in Descemet's membrane (in 53.5% eyes).
  
  
  CONCLUSIONS
  This pathological study suggested that MCD was the most common corneal stromal dystrophy that required keratoplasty in Saudi Arabia. Patient with MCD and GCD presented at a significantly younger age than LCD. The clinical diagnosis of MCD is not achieved in all cases likely due to a more severe phenotype in the Saudi population or the presence of corneal scarring that is associated with previous trachoma, which obscures the classical appearance of LCD. We believe that PKP is first-line surgical treatment, especially for MCD because it involves all corneal layers. However, deep stromal involvement and changes in Descemet's membrane in MCD should be considered when selecting the surgical procedure.
authors.affiliation: Ophthalmology Department, College of Medicine, Al-Qassim University, Riyadh, Saudi Arabia.
authors.forename: Sultan
authors.initials: S
authors.lastname: Alzuhairy
authors.affiliation: Department of Pathology and Laboratory Medicine, KKESH, Riyadh, Saudi Arabia.
authors.forename: Hind M
authors.initials: HM
authors.lastname: Alkatan
authors.affiliation: College of Medicine, Al-Faisal University and Al-Hokama Eye Specialist Center, Riyadh, Saudi Arabia.
authors.forename: Ali A
authors.initials: AA
authors.lastname: Al-Rajhi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003317:Corneal Dystrophies, Hereditary / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D016039:Corneal Transplantation / Q000379:methods; D005260:Female; D006801:Humans; D015948:Keratoplasty, Penetrating; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012529:Saudi Arabia / Q000453:epidemiology; D014792:Visual Acuity / Q000502:physiology
keywords: Corneal Stromal Dystrophy; Histopathology; Keratoplasty; Saudi Arabia