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Essential iris atrophy

MedGen UID:
543177
•Concept ID:
C0271111
•
Disease or Syndrome
Synonyms: essential iris atrophy; Progressive iris atrophy
SNOMED CT: Essential iris atrophy (25913001); Progressive iris atrophy (25913001)
 
Monarch Initiative: MONDO:0020371
Orphanet: ORPHA98981

Definition

A clinical variant of iridocorneal endothelial (ICE) syndrome, characterized by progressive iris atrophy and holes present on the surface of the iris, corneal edema, corectopia, uveal ectropion and anterior synechiae. Secondary glaucoma is also a common complication of the disease. [from ORDO]

Professional guidelines

PubMed

The differential diagnosis of malignant melanoma of the iris. A clinical study of 200 patients.
Shields JA, Sanborn GE, Augsburger JJ
Ophthalmology 1983 Jun;90(6):716-20. doi: 10.1016/s0161-6420(83)34500-0. PMID: 6888862
Treatment of glaucoma associated with progressive essential iris atrophy and iridoschisis.
FORSIUS H, TARKKANEN
Acta Ophthalmol (Copenh) 1961;39:356-66. doi: 10.1111/j.1755-3768.1961.tb00862.x. PMID: 13700860

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
ICE SYNDROME--CASE REPORT.
Coviltir V, Valentin D
Rom J Ophthalmol 2015 Apr-Jun;59(2):119-22. PMID: 26978875Free PMC Article
Boston Type 1 Keratoprosthesis for Iridocorneal Endothelial Syndromes.
Phillips DL, Goins KM, Greiner MA, Alward WL, Kwon YH, Wagoner MD
Cornea 2015 Nov;34(11):1383-6. doi: 10.1097/ICO.0000000000000616. PMID: 26398156
Progression of essential iris atrophy studied with confocal microscopy and ultrasound biomicroscopy: a 5-year case report.
Pezzi PP, Marenco M, Cosimi P, Mannino G, Iannetti L
Cornea 2009 Jan;28(1):99-102. doi: 10.1097/ICO.0b013e3181822579. PMID: 19092417
Proliferative endotheliopathy with iris abnormalities. The iridocorneal endothelial syndrome.
Eagle RC Jr, Font RL, Yanoff M, Fine BS
Arch Ophthalmol 1979 Nov;97(11):2104-11. doi: 10.1001/archopht.1979.01020020422002. PMID: 508177

Diagnosis

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
Iridocorneal endothelial syndrome in a patient with keratoconus - a case report.
De Maria M, Iannetta D, Moramarco A, Fontana L
BMC Ophthalmol 2019 Nov 11;19(1):221. doi: 10.1186/s12886-019-1215-x. PMID: 31711443Free PMC Article
ICE SYNDROME--CASE REPORT.
Coviltir V, Valentin D
Rom J Ophthalmol 2015 Apr-Jun;59(2):119-22. PMID: 26978875Free PMC Article
Progression of essential iris atrophy studied with confocal microscopy and ultrasound biomicroscopy: a 5-year case report.
Pezzi PP, Marenco M, Cosimi P, Mannino G, Iannetti L
Cornea 2009 Jan;28(1):99-102. doi: 10.1097/ICO.0b013e3181822579. PMID: 19092417
The differential diagnosis of malignant melanoma of the iris. A clinical study of 200 patients.
Shields JA, Sanborn GE, Augsburger JJ
Ophthalmology 1983 Jun;90(6):716-20. doi: 10.1016/s0161-6420(83)34500-0. PMID: 6888862

Therapy

'Progressive peripheral anterior synechiae in iridocorneoendothelial syndrome- a crawling disaster'.
D'cruz RP, Rao A
Eur J Ophthalmol 2023 May;33(3):NP40-NP44. Epub 2021 Dec 29 doi: 10.1177/11206721211070095. PMID: 34964381
Femtosecond-assisted keratopigmentation for functional and cosmetic restoration in essential iris atrophy.
Alió JL, Rodriguez AE, Toffaha BT, Piñero DP, Moreno LJ
J Cataract Refract Surg 2011 Oct;37(10):1744-7. Epub 2011 Aug 23 doi: 10.1016/j.jcrs.2011.08.003. PMID: 21865008
A case of essential iris atrophy in a male patient.
Bandyopadhyay S, Biswas G, Biswas J, Das D
J Indian Med Assoc 2010 Sep;108(9):609-10. PMID: 21510538
Bullous keratopathy accompanying essential iris atrophy: response to lowered intraocular pressure.
Choplin NT, Harris S, Rahn EK
Ann Ophthalmol 1980 Sep;12(9):1052-3. PMID: 6927215

Prognosis

Boston Type 1 Keratoprosthesis for Iridocorneal Endothelial Syndromes.
Phillips DL, Goins KM, Greiner MA, Alward WL, Kwon YH, Wagoner MD
Cornea 2015 Nov;34(11):1383-6. doi: 10.1097/ICO.0000000000000616. PMID: 26398156
A comparison of the clinical variations of the iridocorneal endothelial syndrome.
Wilson MC, Shields MB
Arch Ophthalmol 1989 Oct;107(10):1465-8. doi: 10.1001/archopht.1989.01070020539035. PMID: 2803093
Surgical results in iridocorneal endothelial syndrome.
Kidd M, Hetherington J, Magee S
Arch Ophthalmol 1988 Feb;106(2):199-201. doi: 10.1001/archopht.1988.01060130209027. PMID: 3341974
The essential iris atrophies.
Shields MB, Campbell DG, Simmons RJ
Am J Ophthalmol 1978 Jun;85(6):749-59. doi: 10.1016/s0002-9394(14)78101-2. PMID: 677202
Iris nodules in essential iris atrophy.
Shields MB, Campbell DG, Simmons RJ, Hutchinson BT
Arch Ophthalmol 1976 Mar;94(3):406-10. doi: 10.1001/archopht.1976.03910030194004. PMID: 818989

Clinical prediction guides

'Progressive peripheral anterior synechiae in iridocorneoendothelial syndrome- a crawling disaster'.
D'cruz RP, Rao A
Eur J Ophthalmol 2023 May;33(3):NP40-NP44. Epub 2021 Dec 29 doi: 10.1177/11206721211070095. PMID: 34964381
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
Progression of essential iris atrophy studied with confocal microscopy and ultrasound biomicroscopy: a 5-year case report.
Pezzi PP, Marenco M, Cosimi P, Mannino G, Iannetti L
Cornea 2009 Jan;28(1):99-102. doi: 10.1097/ICO.0b013e3181822579. PMID: 19092417
Essential iris atrophy. A clinical, immunohistologic, and electron microscopic study in an enucleated eye.
Rodrigues MM, Jester JV, Richards R, Rajagopalan S, Stevens G
Ophthalmology 1988 Jan;95(1):69-78. doi: 10.1016/s0161-6420(88)33234-3. PMID: 2449642
Specular microscopy of irido-corneal endothelial syndrome.
Hirst LW, Quigley HA, Stark WJ, Shields NB
Aust J Ophthalmol 1980 May;8(2):139-46. doi: 10.1111/j.1442-9071.1980.tb01672.x. PMID: 7447799

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