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Iridocorneal endothelial syndrome

MedGen UID:
242751
•Concept ID:
C1096100
•
Anatomical Abnormality
Synonyms: Endothelial Syndrome, Iridocorneal; ICE Syndrome; Iridocorneal Endothelial Syndrome; Iridocorneal Endothelial Syndromes
SNOMED CT: Iridocorneal endothelial syndrome (129623003); Irido-corneo-endothelial syndrome (129623003); ICE (irido-corneo-endothelial) syndrome (129623003); ICE (iridocorneal endothelial) syndrome (129623003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018988
Orphanet: ORPHA64734

Definition

Iridocorneal endothelial (ICE) syndrome describes a group of progressive corneal proliferative endotheliopathies comprised of Chandler?s syndrome, Cogan-Reese syndrome and essential iris atrophy (see these terms), affecting mainly young adult females and characterized by iris holes and atrophy, papillary distortion, anterior synechiae, corneal edema and often with secondary glaucoma and corneal decompensation as complications [from ORDO]

Professional guidelines

PubMed

Diagnosis and Management of Iridocorneal Endothelial Syndrome.
Sacchetti M, Mantelli F, Marenco M, Macchi I, Ambrosio O, Rama P
Biomed Res Int 2015;2015:763093. Epub 2015 Sep 16 doi: 10.1155/2015/763093. PMID: 26451377Free PMC Article
Differential diagnosis of iridocorneal endothelial syndrome and posterior polymorphous endothelial dystrophy.
Hirst LW
Br J Ophthalmol 1993 Sep;77(9):610. doi: 10.1136/bjo.77.9.610. PMID: 8218068Free PMC Article

Recent clinical studies

Etiology

Iridocorneal Endothelial Syndrome: Case Report of Essential Progressive Iris Atrophy.
Beganovic AP, Vodencarevic AN, Halilbasic M, Medjedovic A
Med Arch 2022 Jun;76(3):224-228. doi: 10.5455/medarh.2022.76.224-228. PMID: 36200117Free PMC Article
Graft Survival After Penetrating and Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome.
Rotenberg M, Downward L, Curnow E, Larkin DF, Tuft SJ; National Health Service Blood and Ocular Tissue Advisory Group and Contributing Ophthalmologists (OTAG Study 27)
Cornea 2020 Jan;39(1):18-22. doi: 10.1097/ICO.0000000000002039. PMID: 31335531
The iridocorneal endothelial syndrome.
Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R
Surv Ophthalmol 2018 Sep-Oct;63(5):665-676. Epub 2018 Jan 11 doi: 10.1016/j.survophthal.2018.01.001. PMID: 29331589
Diagnosis and Management of Iridocorneal Endothelial Syndrome.
Sacchetti M, Mantelli F, Marenco M, Macchi I, Ambrosio O, Rama P
Biomed Res Int 2015;2015:763093. Epub 2015 Sep 16 doi: 10.1155/2015/763093. PMID: 26451377Free PMC Article
Glaucoma drainage implants.
Sidoti PA, Baerveldt G
Curr Opin Ophthalmol 1994 Apr;5(2):85-98. doi: 10.1097/00055735-199404000-00013. PMID: 10172032

Diagnosis

Ultrasound biomicroscopy in glaucoma assessment.
Potop V, Coviltir V, Schmitzer S, Dragosloveanu CDM, Ionescu CI, Burcel MG, Corbu MC, Dăscălescu DMC
Rom J Ophthalmol 2021 Apr-Jun;65(2):114-119. doi: 10.22336/rjo.2021.24. PMID: 34179574Free PMC Article
Clinical and Specular microscopy characteristics and corelation in Iridocorneal endothelial syndrome without corneal edema.
Chaurasia S, Choudhari NS, Mohamed A
Semin Ophthalmol 2021 Oct 3;36(7):561-568. Epub 2021 Mar 22 doi: 10.1080/08820538.2021.1900285. PMID: 33750265
The iridocorneal endothelial syndrome.
Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R
Surv Ophthalmol 2018 Sep-Oct;63(5):665-676. Epub 2018 Jan 11 doi: 10.1016/j.survophthal.2018.01.001. PMID: 29331589
Diagnosis and Management of Iridocorneal Endothelial Syndrome.
Sacchetti M, Mantelli F, Marenco M, Macchi I, Ambrosio O, Rama P
Biomed Res Int 2015;2015:763093. Epub 2015 Sep 16 doi: 10.1155/2015/763093. PMID: 26451377Free PMC Article
Iridocorneal endothelial syndrome.
Saleem AA, Ali M, Akhtar F
J Coll Physicians Surg Pak 2014 May;24 Suppl 2:S112-4. PMID: 24906259

Therapy

Outcomes of the Aurolab aqueous drainage implant and trabeculectomy with mitomycin C in iridocorneal endothelial syndrome.
Senthilkumar VA, Puthuran GV, Tara TD, Nagdev N, Ramesh S, Mani I, Krishnadas SR, Gedde SJ
Graefes Arch Clin Exp Ophthalmol 2023 Feb;261(2):545-554. Epub 2022 Aug 30 doi: 10.1007/s00417-022-05811-6. PMID: 36038686
Outcomes of Ahmed Glaucoma Drainage Implant in Eyes With Glaucoma Secondary to Iridocorneal Endothelial Syndrome.
Gebremichael BG, Mohamed A, Chaurasia S, Senthil S
J Glaucoma 2020 Jul;29(7):567-571. doi: 10.1097/IJG.0000000000001504. PMID: 32251072
Iridocorneal endothelial syndrome.
Saleem AA, Ali M, Akhtar F
J Coll Physicians Surg Pak 2014 May;24 Suppl 2:S112-4. PMID: 24906259
Glaucoma drainage implants.
Sidoti PA, Baerveldt G
Curr Opin Ophthalmol 1994 Apr;5(2):85-98. doi: 10.1097/00055735-199404000-00013. PMID: 10172032
Glaucoma and the iridocorneal endothelial syndrome.
Laganowski HC, Kerr Muir MG, Hitchings RA
Arch Ophthalmol 1992 Mar;110(3):346-50. doi: 10.1001/archopht.1992.01080150044025. PMID: 1543451

Prognosis

Outcomes of Descemet-Stripping Endothelial Keratoplasty in 52 Eyes With Iridocorneal Endothelial Syndrome.
Mohamed A, Chaurasia S, Senthil S, Choudhari NS
Cornea 2022 Feb 1;41(2):159-164. doi: 10.1097/ICO.0000000000002699. PMID: 34351729
Comparison of Descemet Membrane Endothelial Keratoplasty for Iridocorneal Endothelial Syndrome and Fuchs Endothelial Dystrophy.
Wu J, Dong X, Ouyang C, Ji J, Xie L, Hou C, Huang T
Am J Ophthalmol 2021 Jun;226:76-82. Epub 2021 Feb 5 doi: 10.1016/j.ajo.2021.01.029. PMID: 33556383
Graft Survival After Penetrating and Endothelial Keratoplasty in Iridocorneal Endothelial Syndrome.
Rotenberg M, Downward L, Curnow E, Larkin DF, Tuft SJ; National Health Service Blood and Ocular Tissue Advisory Group and Contributing Ophthalmologists (OTAG Study 27)
Cornea 2020 Jan;39(1):18-22. doi: 10.1097/ICO.0000000000002039. PMID: 31335531
Glaucoma drainage implants.
Sidoti PA, Baerveldt G
Curr Opin Ophthalmol 1994 Apr;5(2):85-98. doi: 10.1097/00055735-199404000-00013. PMID: 10172032
Glaucoma and the iridocorneal endothelial syndrome.
Laganowski HC, Kerr Muir MG, Hitchings RA
Arch Ophthalmol 1992 Mar;110(3):346-50. doi: 10.1001/archopht.1992.01080150044025. PMID: 1543451

Clinical prediction guides

Comparison of penetrating and endothelial keratoplasty in patients with iridocorneal endothelial syndrome: A registry study.
Roberts PK, Keane M, Yang G, Chan E, Harkin DG, McKirdy N, Daniell M; all contributors to the Australian Corneal Graft Registry
Clin Exp Ophthalmol 2023 Sep-Oct;51(7):663-672. Epub 2023 Aug 22 doi: 10.1111/ceo.14283. PMID: 37608637
Iridocorneal Endothelial Syndrome: Case Report of Essential Progressive Iris Atrophy.
Beganovic AP, Vodencarevic AN, Halilbasic M, Medjedovic A
Med Arch 2022 Jun;76(3):224-228. doi: 10.5455/medarh.2022.76.224-228. PMID: 36200117Free PMC Article
Clinical and Specular microscopy characteristics and corelation in Iridocorneal endothelial syndrome without corneal edema.
Chaurasia S, Choudhari NS, Mohamed A
Semin Ophthalmol 2021 Oct 3;36(7):561-568. Epub 2021 Mar 22 doi: 10.1080/08820538.2021.1900285. PMID: 33750265
Comparison of Descemet Membrane Endothelial Keratoplasty for Iridocorneal Endothelial Syndrome and Fuchs Endothelial Dystrophy.
Wu J, Dong X, Ouyang C, Ji J, Xie L, Hou C, Huang T
Am J Ophthalmol 2021 Jun;226:76-82. Epub 2021 Feb 5 doi: 10.1016/j.ajo.2021.01.029. PMID: 33556383
Clinical and specular microscopic manifestations of iridocorneal endothelial syndrome.
Liu YK, Wang IJ, Hu FR, Hung PT, Chang HW
Jpn J Ophthalmol 2001 May-Jun;45(3):281-7. doi: 10.1016/s0021-5155(00)00383-x. PMID: 11369379

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