title: Photophobia and corneal crystal density in nephropathic cystinosis: an in vivo confocal microscopy and anterior-segment optical coherence tomography study.
abstract: OBJECTIVE
  To analyze the correlation between photophobia and corneal crystal density in nephropathic cystinosis using in vivo confocal microscopy (IVCM) and anterior-segment optical coherence tomography (AS-OCT).
  
  
  METHODS
  Forty eyes of 20 patients with nephropathic cystinosis aged 7 to 37 years were included in this study. Ophthalmologic investigations included clinician-assessed and self-assessed evaluations of photophobia, slit-lamp biomicroscopy analysis, the depth of crystal deposition (DCD) and the central corneal thickness (CCT) in the central cornea measured with AS-OCT, and IVCM analysis of the crystal density score (IVCM-CysS), inflammatory cell density (IVCM-inf), and nerve damage (IVCM-N). Age, sex, intraleukocyte cystine concentrations (ICC), and the need for renal transplantation were also recorded.
  
  
  RESULTS
  The average subjective and objective photophobia scores were 2.10 ± 1.28 and 1.70 ± 1.41, respectively. Using AS-OCT, the average percentage of crystal infiltration (OCT-CysP) and was 49.56 ± 27.31% (range, 11.45%-95.81%). The mean IVCM-CysS was 8.84 ± 4.34, the mean density of inflammatory cells (IVCM-inf) was 178.28 ± 173.00 cells/mm2, and the mean IVCM-N score was 3.11 ± 2.11. Clinician- and self-assessed estimations of photophobia were correlated (R2 = 0.61). No significant correlation was observed between clinician- and self-assessed photophobia scores and ICC or sex. There were significant correlations between clinician- and self-assessed photophobia scores and age, OCT-CysP, IVCM-CysS, IVCM-inf, and IVCM-N. The IVCM-CysS was also correlated with OCT-CysP (R2 = 0.27), IVCM-inf (R2 = 0.37), and IVCM-N (R2 = 0.56).
  
  
  CONCLUSIONS
  In vivo confocal microscopy and AS-OCT are reliable tools to quantify cystinosis corneal crystals. In patients with nephropathic cystinosis, the intensity of photophobia is associated with the density of crystals, infiltration of inflammatory cells, and nerve damage within the cornea.
authors.affiliation: Department of Ophthalmology III Quinze-Vingts National Ophthalmology Hospital, Paris, France 2Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, DHU View Maintain, Institut national de la santé et de la recherche médicale (INSERM)-DHOS CIC 142.
authors.forename: Hong
authors.initials: H
authors.lastname: Liang
authors.affiliation: Department of Ophthalmology III Quinze-Vingts National Ophthalmology Hospital, Paris, France 2Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, DHU View Maintain, Institut national de la santé et de la recherche médicale (INSERM)-DHOS CIC 142.
authors.forename: Christophe
authors.initials: C
authors.lastname: Baudouin
authors.affiliation: Department of Ophthalmology III Quinze-Vingts National Ophthalmology Hospital, Paris, France 2Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, DHU View Maintain, Institut national de la santé et de la recherche médicale (INSERM)-DHOS CIC 142.
authors.forename: Rachid
authors.initials: R
authors.lastname: Tahiri Joutei Hassani
authors.affiliation: INSERM, U968, Paris, France 4Sorbonne Universités, UPMC University Paris 06, UMR_S 968, Institut de la Vision, Paris, France 5Centre national de la recherche scientifique (CNRS), UMR_7210, Paris, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Brignole-Baudouin
authors.affiliation: Department of Ophthalmology III Quinze-Vingts National Ophthalmology Hospital, Paris, France 2Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, DHU View Maintain, Institut national de la santé et de la recherche médicale (INSERM)-DHOS CIC 142.
authors.forename: Antoine
authors.initials: A
authors.lastname: Labbe
mesh_terms: D000293:Adolescent; D000328:Adult; D000869:Anterior Eye Segment / Q000473:pathology*; D002648:Child; D003316:Corneal Diseases / Q000209:etiology / Q000473:pathology*; D003460:Crystallization; D003554:Cystinosis / Q000150:complications*; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000150:complications*; D008297:Male; D018613:Microscopy, Confocal / Q000379:methods*; D020795:Photophobia / Q000209:etiology / Q000473:pathology*; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity; D055815:Young Adult