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Granular corneal dystrophy

MedGen UID:
42290
•Concept ID:
C0018179
•
Disease or Syndrome
Synonyms: Corneal Granular Dystrophies; Corneal Granular Dystrophy; Dystrophy, Corneal Granular; Granular Dystrophy, Corneal
SNOMED CT: Granular corneal dystrophy (45283008)
 
HPO: HP:0007802
Monarch Initiative: MONDO:0001490

Definition

The presence of central, fine, whitish granular lesions in the stroma of the cornea. This type of corneal dystrophy is usually asymptomatic and begins in childhood and shows a slow progression. Later in the course, the corneal epithelium and Bowman's layer may be affected. Histologically, the cornea shows a uniform deposition of hyaline material. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Granular corneal dystrophy

Conditions with this feature

Groenouw corneal dystrophy type I
MedGen UID:
351521
•Concept ID:
C1641846
•
Disease or Syndrome
Groenouw type I, or granular type I, corneal dystrophy (CDGG1) is an autosomal dominant disorder characterized by irregular aggregates of hyaline material in the corneal stroma. These aggregates can cause significant visual disturbance and may require corneal transplantation for restoration of visual acuity or for relief from recurrent corneal erosions (summary by Stone et al., 1994).
See: Condition Record
Groenouw corneal dystrophy type I

Professional guidelines

PubMed

Classic lattice corneal dystrophy: a brief review and summary of treatment modalities.
Milovanova E, Gomon S, Rocha G
Graefes Arch Clin Exp Ophthalmol 2024 Jun;262(6):1667-1681. Epub 2023 Nov 7 doi: 10.1007/s00417-023-06297-6. PMID: 37934291
Mini-Review: Clinical Features and Management of Granular Corneal Dystrophy Type 2.
Chang MS, Jun I, Kim EK
Korean J Ophthalmol 2023 Aug;37(4):340-347. Epub 2023 Jun 19 doi: 10.3341/kjo.2023.0032. PMID: 37336511Free PMC Article
Differential diagnosis between granular corneal dystrophy Groenouw type I and paraproteinemic crystalline keratopathy.
Møller HU, Ehlers N, Bojsen-Møller M, Ridgway AE
Acta Ophthalmol (Copenh) 1993 Aug;71(4):552-5. doi: 10.1111/j.1755-3768.1993.tb04635.x. PMID: 8249591

Recent clinical studies

Etiology

Classic lattice corneal dystrophy: a brief review and summary of treatment modalities.
Milovanova E, Gomon S, Rocha G
Graefes Arch Clin Exp Ophthalmol 2024 Jun;262(6):1667-1681. Epub 2023 Nov 7 doi: 10.1007/s00417-023-06297-6. PMID: 37934291
Granular corneal dystrophy: an enigma resolved.
Sahay P, Agarwal D, Maharana PK, Titiyal JS
Int Ophthalmol 2019 Jul;39(7):1599-1602. Epub 2018 Jun 25 doi: 10.1007/s10792-018-0971-6. PMID: 29943099
Corneal Higher Order Aberrations in Granular, Lattice and Macular Corneal Dystrophies.
Yagi-Yaguchi Y, Yamaguchi T, Okuyama Y, Satake Y, Tsubota K, Shimazaki J
PLoS One 2016;11(8):e0161075. Epub 2016 Aug 18 doi: 10.1371/journal.pone.0161075. PMID: 27536778Free PMC Article
Pathogenesis and treatments of TGFBI corneal dystrophies.
Han KE, Choi SI, Kim TI, Maeng YS, Stulting RD, Ji YW, Kim EK
Prog Retin Eye Res 2016 Jan;50:67-88. Epub 2015 Nov 28 doi: 10.1016/j.preteyeres.2015.11.002. PMID: 26612778
Clinical and genetic aspects of the TGFBI-associated corneal dystrophies.
Lakshminarayanan R, Chaurasia SS, Anandalakshmi V, Chai SM, Murugan E, Vithana EN, Beuerman RW, Mehta JS
Ocul Surf 2014 Oct;12(4):234-51. Epub 2014 Jul 18 doi: 10.1016/j.jtos.2013.12.002. PMID: 25284770

Diagnosis

Mini-Review: Clinical Features and Management of Granular Corneal Dystrophy Type 2.
Chang MS, Jun I, Kim EK
Korean J Ophthalmol 2023 Aug;37(4):340-347. Epub 2023 Jun 19 doi: 10.3341/kjo.2023.0032. PMID: 37336511Free PMC Article
Granular corneal dystrophy: an enigma resolved.
Sahay P, Agarwal D, Maharana PK, Titiyal JS
Int Ophthalmol 2019 Jul;39(7):1599-1602. Epub 2018 Jun 25 doi: 10.1007/s10792-018-0971-6. PMID: 29943099
Corneal Higher Order Aberrations in Granular, Lattice and Macular Corneal Dystrophies.
Yagi-Yaguchi Y, Yamaguchi T, Okuyama Y, Satake Y, Tsubota K, Shimazaki J
PLoS One 2016;11(8):e0161075. Epub 2016 Aug 18 doi: 10.1371/journal.pone.0161075. PMID: 27536778Free PMC Article
A case of concomitant keratoconus and granular corneal dystrophy type II.
Rho CR, Park JH, Jung YH, Kim MS
Cont Lens Anterior Eye 2014 Aug;37(4):314-6. Epub 2014 Feb 28 doi: 10.1016/j.clae.2014.02.001. PMID: 24582869
Granular corneal dystrophy Groenouw type I. Clinical and genetic aspects.
Møller HU
Acta Ophthalmol Suppl (1985) 1991;(198):1-40. PMID: 1651039

Therapy

Mini-Review: Clinical Features and Management of Granular Corneal Dystrophy Type 2.
Chang MS, Jun I, Kim EK
Korean J Ophthalmol 2023 Aug;37(4):340-347. Epub 2023 Jun 19 doi: 10.3341/kjo.2023.0032. PMID: 37336511Free PMC Article
LASIK surgery of granular corneal dystrophy type 2 patients leads to accumulation and differential proteolytic processing of transforming growth factor beta-induced protein (TGFBIp).
Poulsen ET, Nielsen NS, Jensen MM, Nielsen E, Hjortdal J, Kim EK, Enghild JJ
Proteomics 2016 Feb;16(3):539-43. Epub 2016 Jan 3 doi: 10.1002/pmic.201500287. PMID: 26864644
Clinical findings and treatments of granular corneal dystrophy type 2 (avellino corneal dystrophy): a review of the literature.
Han KE, Kim TI, Chung WS, Choi SI, Kim BY, Kim EK
Eye Contact Lens 2010 Sep;36(5):296-9. doi: 10.1097/ICL.0b013e3181ef0da0. PMID: 20724852
Mitomycin C does not inhibit exacerbation of granular corneal dystrophy type II induced by refractive surface ablation.
Ha BJ, Kim TI, Choi SI, Stulting RD, Lee DH, Cho HS, Kim EK
Cornea 2010 May;29(5):490-6. doi: 10.1097/ICO.0b013e3181c3258a. PMID: 20299970
Update on phototherapeutic keratectomy.
Stasi K, Chuck RS
Curr Opin Ophthalmol 2009 Jul;20(4):272-5. doi: 10.1097/ICU.0b013e32832b4f44. PMID: 19381088

Prognosis

Deep anterior lamellar keratoplasty outcomes in macular and granular corneal dystrophy - A comparative cross-sectional study.
Kodavoor SK, Deb B, Ramamurthy D
Indian J Ophthalmol 2019 Nov;67(11):1830-1833. doi: 10.4103/ijo.IJO_1623_18. PMID: 31638043Free PMC Article
Corneal Dystrophy Mutations Drive Pathogenesis by Targeting TGFBIp Stability and Solubility in a Latent Amyloid-forming Domain.
Stenvang M, Schafer NP, Malmos KG, Pérez AW, Niembro O, Sormanni P, Basaiawmoit RV, Christiansen G, Andreasen M, Otzen DE
J Mol Biol 2018 Apr 13;430(8):1116-1140. Epub 2018 Mar 7 doi: 10.1016/j.jmb.2018.03.001. PMID: 29524512
The Oskar Fehr Lecture.
Weiss JS
Klin Monbl Augenheilkd 2016 Jun;233(6):708-12. Epub 2016 Jun 17 doi: 10.1055/s-0042-100735. PMID: 27315290
The Role of the Reactive Oxygen Species and Oxidative Stress in the Pathomechanism of the Age-Related Ocular Diseases and Other Pathologies of the Anterior and Posterior Eye Segments in Adults.
Nita M, Grzybowski A
Oxid Med Cell Longev 2016;2016:3164734. Epub 2016 Jan 10 doi: 10.1155/2016/3164734. PMID: 26881021Free PMC Article
Granular corneal dystrophy Groenouw type I. Clinical aspects and treatment.
Møller HU
Acta Ophthalmol (Copenh) 1990 Aug;68(4):384-9. doi: 10.1111/j.1755-3768.1990.tb01665.x. PMID: 2220354

Clinical prediction guides

Exacerbation of Granular Corneal Dystrophy Type 2 After Small Incision Lenticule Extraction.
Kwak JJ, Yoon SH, Seo KY, Kim TI, Lee HK, Stulting RD, Kim EK
Cornea 2021 Apr;40(4):519-524. doi: 10.1097/ICO.0000000000002655. PMID: 33560675Free PMC Article
Oxidative Stress, Ocular Disease and Diabetes Retinopathy.
Tangvarasittichai O, Tangvarasittichai S
Curr Pharm Des 2018;24(40):4726-4741. doi: 10.2174/1381612825666190115121531. PMID: 30644339
Corneal Dystrophy Mutations Drive Pathogenesis by Targeting TGFBIp Stability and Solubility in a Latent Amyloid-forming Domain.
Stenvang M, Schafer NP, Malmos KG, Pérez AW, Niembro O, Sormanni P, Basaiawmoit RV, Christiansen G, Andreasen M, Otzen DE
J Mol Biol 2018 Apr 13;430(8):1116-1140. Epub 2018 Mar 7 doi: 10.1016/j.jmb.2018.03.001. PMID: 29524512
Pathogenesis and treatments of TGFBI corneal dystrophies.
Han KE, Choi SI, Kim TI, Maeng YS, Stulting RD, Ji YW, Kim EK
Prog Retin Eye Res 2016 Jan;50:67-88. Epub 2015 Nov 28 doi: 10.1016/j.preteyeres.2015.11.002. PMID: 26612778
A unique TGFBI protein in granular corneal dystrophy types 1 and 2.
Han YP, Sim AJ, Vora SC, Huang AJ
Curr Eye Res 2012 Nov;37(11):990-6. Epub 2012 Jun 29 doi: 10.3109/02713683.2012.700752. PMID: 22746317Free PMC Article

Recent systematic reviews

Autophagy modulation in animal models of corneal diseases: a systematic review.
Martínez-Chacón G, Vela FJ, Campos JL, Abellán E, Yakhine-Diop SMS, Ballestín A
Mol Cell Biochem 2020 Nov;474(1-2):41-55. Epub 2020 Jul 24 doi: 10.1007/s11010-020-03832-5. PMID: 32710189

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