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A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives

Year 2025, Volume: 42 Issue: 2, 212 - 218, 30.06.2025

Abstract

Chandler's Syndrome, a rare variant of iridocorneal endothelial (ICE) syndrome, is characterized by corneal endothelial abnormalities, iris atrophy, and secondary glaucoma. This review highlights this progressive condition's clinical presentation, pathophysiology, diagnosis, and management. Chandler's Syndrome primarily affects middle-aged Caucasian women and presents with unilateral corneal edema, visual disturbances, and subtle iris changes. Endothelial cell proliferation and migration lead to peripheral anterior synechiae (PAS), elevated intraocular pressure (IOP), and corneal decompensation. While the exact etiology remains unclear, viral infections such as herpes simplex (HSV) and Epstein-Barr (EBV) are hypothesized contributors. Morphological changes, including metaplasia of endothelial cells and ectopic membrane formation, further compromise aqueous outflow and corneal integrity. Diagnosis relies on slit-lamp examination, specular and confocal microscopy, and ultrasound biomicroscopy (UBM) to detect endothelial abnormalities and anterior segment changes. Differential diagnosis with conditions like posterior polymorphous corneal dystrophy (PPCD) and Fuchs' dystrophy is critical for accurate assessment. Management includes medical therapy to control IOP and surgical interventions like trabeculectomy, glaucoma drainage implants, and corneal procedures such as Descemet stripping endothelial keratoplasty (DSEK) or penetrating keratoplasty (PK). Long-term follow-up is essential to monitor disease progression and preserve visual function. Future research into the viral pathogenesis and mechanisms of endothelial dysfunction is vital to advance targeted therapies.

Ethical Statement

There is no need of ethics committee approval as it is a review article

References

  • Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R. The iridocorneal endothelial syndrome. Surv Ophthalmol. 2018;63(5):665-676.
  • Chandler PA. Atrophy of the stroma of the iris; endothelial dystrophy, corneal edema, and glaucoma. Am J Ophthalmol. 1956;41(4):607-615.
  • Cogan DG, Reese AB. A syndrome of iris nodules, ectopic Descemet's membrane, and unilateral glaucoma. Doc Ophthalmol. 1969;26:424-433.
  • Morris RW, Dunbar MT. Atypical presentation and review of the ICE syndrome. Optometry. 2004;75(1):13-25.
  • Heath P. Essential atrophy of the iris: a histopathologic study. Trans Am Ophthalmol Soc. 1953;51:167-192.
  • Chandler PA, Grant WM: Lectures on Glaucoma. Philadelphia, Lea & Febiger.1965:276-295.
  • Saleem AA, Ali M, Akhtar F. Iridocorneal endothelial syndrome. J Coll Physicians Surg Pak. 2014;24 Suppl 2:S112-S114.
  • Rasquinha, M. (n.d.). Irido-corneal endothelial syndrome: An overview [Internet]. Available at [cited 02 December 2024]: https://www.eyenews.uk.com/media/26755/eye-onex-jan22 rasquinha-final.pdf
  • Alvarado JA, Underwood JL, Green WR, et al. Detection of herpes simplex viral DNA in the iridocorneal endothelial syndrome. Arch Ophthalmol.1994;112(12):1601-1609.
  • Rodrigues MM, Phelps CD, Krachmer JH, Cibis GW, Weingeist TA. Glaucoma due to endothelialization of the anterior chamber angle. A comparison of posterior polymorphous dystrophy of the cornea and Chandler's syndrome. Arch Ophthalmol. 1980;98(4):688-696.
  • Levy SG, McCartney AC, Baghai MH, Barrett MC, Moss J. Pathology of the iridocorneal-endothelial syndrome. The ICE-cell. Invest Ophthalmol Vis Sci. 1995;36(13):2592-2601.
  • Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Senthil S. Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS One. 2017;12(3):e0171884. Published 2017 Mar 10.
  • Des Marchais B, Simmons RB, Simmons RJ, Shields MB. Bilateral Chandler syndrome. J Glaucoma. 1999;8(4):276- 277.
  • Huna R, Barak A, Melamed S. Bilateral iridocorneal endothelial syndrome presented as Cogan-Reese and Chandler's syndrome. J Glaucoma. 1996;5(1):60-62.
  • Hemady RK, Patel A, Blum S, Nirankari VS. Bilateral iridocorneal endothelial syndrome: case report and review of the literature. Cornea. 1994;13(4):368-372.
  • Blum JV, Allen JH, Holland MG. Familial bilateral essential iris atrophy (Group 2). Trans Am Acad Ophthalmol Otolaryngol. 1962;66:493-500.
  • Kaiser-Kupfer M, Kuwabara T, Kupfer C. Progressive bilateral essential iris atrophy. Am J Ophthalmol. 1977;83(3):340-346.
  • Teekhasaenee C, Ritch R. Iridocorneal endothelial syndrome in Thai patients: clinical variations. Arch Ophthalmol. 2000;118(2):187-192.
  • Kupfer C, Kaiser-Kupfer MI, Datiles M, McCain L. The contralateral eye in the iridocorneal endothelial (ICE) syndrome. Ophthalmology. 1983;90(11):1343-1350.
  • Lucas-Glass TC, Baratz KH, Nelson LR, Hodge DO, Bourne WM. The contralateral corneal endothelium in the iridocorneal endothelial syndrome. Arch Ophthalmol. 1997;115(1):40-44.
  • Zhang M, Chen J, Liang L, Laties AM, Liu Z. Ultrasound biomicroscopy of Chinese eyes with iridocorneal endothelial syndrome. Br J Ophthalmol. 2006;90(1):64-69.
  • Wang N, Wu H, Fan Z. Primary angle closure glaucoma in Chinese and Western populations. Chin Med J (Engl). 2002;115(11):1706-1715.
  • Foster PJ, Oen FT, Machin D, et al. The prevalence of glaucoma in Chinese residents of Singapore: a cross- sectional population survey of the Tanjong Pagar district. Arch Ophthalmol. 2000;118(8):1105-1111.
  • Bourne RR, Sukudom P, Foster PJ, et al. Prevalence of glaucoma in Thailand: a population-based survey in Rom Klao District, Bangkok. Br J Ophthalmol. 2003;87(9):1069-1074.
  • Foster PJ, Baasanhu J, Alsbirk PH, Munkhbayar D, Uranchimeg D, Johnson GJ. Glaucoma in Mongolia. A population- based survey in Hövsgöl province, northern Mongolia. Arch Ophthalmol. 1996;114(10):1235-1241.
  • Alvim PT, Cohen EJ, Rapuano CJ, et al. Penetrating keratoplasty in iridocorneal endothelial syndrome. Cornea. 2001;20(2):134-140.
  • Islam F, Azad N, Khan A. Bilateral iridocorneal endothelial (ICE) syndrome with microspherophakia. J Coll Physicians Surg Pak. 2011;21(6):374-375.
  • Gupta V, Kumar R, Gupta R, Srinivasan G, Sihota R. Bilateral iridocorneal endothelial syndrome in a young girl with Down's syndrome. Indian J Ophthalmol. 2009;57(1):61-63.
  • Estacia CT, Gameiro Filho AR, Faccenda PG, Negri RV, Chianello DT, Alves MAS. Iridocorneal Endothelial Syndrome: Case Report of Chandler’s Variant. Revista Brasileira de Oftalmologia. 2017;76(4).
  • Malhotra C, Seth NG, Pandav SS, et al. Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort [published correction appears in Indian J Ophthalmol. 2020 Mar;68(3):544. doi: 10.4103/0301-4738.278338]. Indian J Ophthalmol. 2019;67(5):604-610.
  • Joko T, Suzuki T, Inoue T, et al. Coincidence of Varicella-Zoster Virus Anterior Uveitis in a Patient with Chandler's Syndrome. Case Rep Ophthalmol. 2013;4(3):274-278. Published 2013 Nov 28.
  • Pan XH, Tao YF, Yang MM, Shen XL, Si J, Li M, Fan N. Bilateral iridocorneal endothelial syndrome-Chandler's syndrome: a case report and literature review. Int J Ophthalmol. 2024 Apr 18;17(4):773-776.
  • Salim S, Aref AA, Akkara JD, Hossain K, Koretz Z, Owaidah OA, et al. Iridocorneal Endothelial Syndrome and Secondary Glaucoma - EyeWiki [Internet]. Eyewiki.org. American Academy of Ophthalmology; 2025. Available from: https://eyewiki.org/Iridocorneal_Endothelial_Syndrome_and_Secondary_Glaucoma
  • De Maria M, Iannetta D, Moramarco A, Fontana L. Iridocorneal endothelial syndrome in a patient with keratoconus - a case report. BMC Ophthalmol. 2019 Nov 11;19(1):221.
  • Beganovic AP, Vodencarevic AN, Halilbasic M, Medjedovic A. Iridocorneal Endothelial Syndrome: Case Report of Essential Progressive Iris Atrophy. Med Arch. 2022 Jun;76(3):224-228.
  • Phillips DL, Alward WLM. Chandler Syndrome: A subtle presentation [Internet]. Uiowa.edu. 2015. Available from: https://webeye.ophth.uiowa.edu/eyeforum/cases/203-chandlers.htm#gsc.tab=0
  • Loya-Garcia D, Hernandez-Camarena JC, Valdez-Garcia JE, Rodríguez-Garcia A. Cogan-Reese syndrome: image analysis with specular microscopy, optical coherence tomography, and ultrasound biomicroscopy. Digit J Ophthalmol. 2019 May 31;25(2):26-29.
  • Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Senthil S. Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS One. 2017;12(3):e0171884.
  • Ichhpujani P, Kaushik S, Gupta A, Pandav SS. Bilateral Chandler's syndrome: Uncommon entity diagnosed by ultrasound biomicroscopy and confocal microscopy. Indian J Ophthalmol. 2020;68(3):528-529.
  • Sacchetti M, Mantelli F, Marenco M, Macchi I, Ambrosio O, Rama P. Diagnosis and Management of Iridocorneal Endothelial Syndrome. BioMed Research International. 2015;2015:1–9.
  • Patel A, Kenyon KR, Hirst LW, Quigley HA, Stark WJ, Meyer RF, Green WR. Clinicopathologic features of Chandler's syndrome. Surv Ophthalmol. 1983;27(5):327-44.
  • Alvarado JA, Murphy CG, Juster RP, Hetherington J. Pathogenesis of Chandler’s syndrome, essential iris atrophy and the Cogan-Reese syndrome. II. Estimated age at disease onset. Investigative Ophthalmology & Visual Science.1986;27(6):873–82. https://iovs.arvojournals.org/article.aspx?articleid=2160011
  • Walkden A, Au L. Iridocorneal endothelial syndrome: clinical perspectives. Clin Ophthalmol. 2018;12:657-664.
  • Shields MBruce. Progressive essential iris atrophy, Chandler’s syndrome, and the iris nevus (Cogan-Reese) syndrome: A spectrum of disease. Survey of Ophthalmology. 1979 Jul;24(1):3–20.
  • Rochat GF, Mulder W. on progressive atrophy of the iris with formation of holes and glaucoma. Br J Ophthalmol. 1924;8(8):362-366.
  • Campbell DG, Shields MB, Smith TR. The corneal endothelium and the spectrum of essential iris atrophy. Am J Ophthalmol. 1978;86(3):317-324.
  • Polack FM, Bourne WM, Forstot SL, Yamaguchi T. Scanning electron microscopy of posterior polymorphous corneal dystrophy. Am J Ophthalmol. 1980;89(4):575-584.
  • Cooper D, Schermer A, Sun TT. Classification of human epithelia and their neoplasms using monoclonal antibodies to keratins: strategies, applications, and limitations. Lab Invest. 1985;52(3):243-256.
  • Weinreb RN, Ryder MI. In situ localization of cytoskeletal elements in the human trabecular meshwork and cornea. Invest Ophthalmol Vis Sci. 1990;31(9):1839-1847.
  • Rodrigues MM, Stulting RD, Waring GO 3rd. Clinical, electron microscopic, and immunohistochemical study of the corneal endothelium and Descemet's membrane in the iridocorneal endothelial syndrome. Am J Ophthalmol. 1986;101(1):16-27.
  • Bourne WM, Brubaker RF. Decreased endothelial permeability in the iridocorneal endothelial syndrome. Ophthalmology. 1982;89(6):591-595.
  • Kayes J, Holmberg A. The fine structure of the cornea in fuchs' endothelial dystrophy. Invest Ophthalmol. 1964;3:47- 67.
  • Iwamoto T, DeVoe AG. Electron microscopic studies on Fuchs'combined dystrophy. I. Posterior portion of the cornea. Invest Ophthalmol. 1971;10(1):9-28.
  • Richardson TM. Corneal decompensation in Chandler's syndrome. A scanning and transmission electron microscopic study. Arch Ophthalmol. 1979;97(11):2112-2119.
  • Waring GO, Laibson PR, Rodrigues M. Clinical and pathologic alterations of Descemet's membrane: With emphasis on endothelial metaplasia. Surv Ophthalmol. 1974;18:325-368.
  • Quigley HA, Forster RF. Histopathology of cornea and iris in Chandler's syndrome. ArchOphthalmol.1978;96(10):1878-1882.
  • Snip RC, Kenyon KR, Green WR. Retrocorneal fibrous membrane in the vitreous touch syndrome. Am J Ophthalmol. 1975;79(2):233-244.
  • Cordero-Moreno R. Atrofia mesodermia del iris, distrofia de córnea y glaucoma secundario: síndrome de Chandler. Arch Soc Esp Oftalmol. 1971;31:667-675.
  • Rodrigues MM, Streeten BW, Spaeth GL. Chandler's syndrome as a variant of essential iris atrophy. A clinicopathologic study. Arch Ophthalmol. 1978;96(4):643-652.
  • Li F, Liu Y, Sun Y, Zhang X. Etiological mechanism of iridocorneal endothelial (ICE) syndrome may involve infection of herpes simplex virus (HSV) and integration of viral genes into human genome. Med Hypotheses. 2018;110:50-52.
  • Joko T, Suzuki T, Inoue T, Kikuchi M, Hara Y, Shiraishi A, Ohashi Y. Coincidence of Varicella-Zoster Virus Anterior Uveitis in a Patient with Chandler's Syndrome. Case Rep Ophthalmol. 2013;4(3):274-8.
  • Ismail AA, El-Ruby SA. Bilateral Chandler Syndrome, Nanophthalmos, and Angle Closure Glaucoma: A Complex Presentation, Challenging Diagnosis, and Pathological Insight-A Case Report. J Curr Glaucoma Pract. 2024 Apr- Jun;18(2):68-73.
  • Agarwal M, Dhiman R, Subramanian K. Atypical Case of Bilateral Chandler Syndrome With Recurrent Band Keratopathy. Cornea. 2021 Oct 1;40(10):1353-1356.
  • Sheppard JD Jr, Lattanzio FA Jr, Williams PB, Mitrev PV, Allen RC. Confocal microscopy used as the definitive, early diagnostic method in Chandler syndrome. Cornea. 2005 Mar;24(2):227-9.
  • Zhang M, Chen J, Liang L, Laties AM, Liu Z. Ultrasound biomicroscopy of Chinese eyes with iridocorneal endothelial syndrome. Br J Ophthalmol. 2006 Jan;90(1):64-9.
  • Loya-Garcia D, Hernandez-Camarena JC, Valdez-Garcia JE, Rodríguez-Garcia A. Cogan-Reese syndrome: image analysis with specular microscopy, optical coherence tomography, and ultrasound biomicroscopy. Digit J Ophthalmol. 2019 May 31;25(2):26-29.
  • Hillard JG. Case Report: Iridocorneal Endothelial Syndrome Progression Documented by Anterior Segment Optical Coherence Tomography. Optom Vis Sci. 2019 Apr;96(4):309-313.
  • Sidoti PA, Schulhof J. Managing Glaucoma with ICE Syndrome. Rev Opth. 2016. https://www.reviewofophthalmology.com/article/managing-glaucoma-with-ice-syndrome.
  • Chaurasia S, Ramappa M, Garg P, Murthy SI, Senthil S, Sangwan VS. Endothelial keratoplasty in the management of irido-corneal endothelial syndrome. Eye. 2013 Jan 25;27(4):564–6.
  • Mahalingam K, Yadav M, Venkatesh Nathiya, Dewang Angmo. A novel presentation of atypical bilateral Chandler syndrome with an insight into management. Indian journal of ophthalmology Case reports. 2021;1(1):114-114.
  • Nongpiur ME, Singhal S, Stewart S, et al. The Impact of Lens Vault on Visual Acuity and Refractive Error: Implications for Management of Primary Angle-closure Glaucoma. J Glaucoma. 2016;25(3):e236-e240.
  • Selvan H, Angmo D, Tomar AS, Yadav S, Sharma A, Dada T. Changes in Intraocular Pressure and Angle Status After Phacoemulsification in Primary Angle Closure Hypertension. J Glaucoma. 2019;28(2):105-110.
There are 72 citations in total.

Details

Primary Language English
Subjects Ophthalmology
Journal Section Review Articles
Authors

Gorle Urmıla 0000-0002-8852-5279

Vinodkumar Mugada 0000-0002-9364-9874

Aishwarya Lakshmi Rajana 0009-0003-5931-2615

Leela Kumari Kilaparthi 0009-0007-2332-2351

Srinivasa Rao Yarguntla 0000-0002-8803-9668

Publication Date June 30, 2025
Submission Date December 17, 2024
Acceptance Date June 27, 2025
Published in Issue Year 2025 Volume: 42 Issue: 2

Cite

APA Urmıla, G., Mugada, V., Rajana, A. L., Kilaparthi, L. K., et al. (2025). A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives. Deneysel Ve Klinik Tıp Dergisi, 42(2), 212-218.
AMA Urmıla G, Mugada V, Rajana AL, Kilaparthi LK, Yarguntla SR. A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives. J. Exp. Clin. Med. June 2025;42(2):212-218.
Chicago Urmıla, Gorle, Vinodkumar Mugada, Aishwarya Lakshmi Rajana, Leela Kumari Kilaparthi, and Srinivasa Rao Yarguntla. “A Comprehensive Review on Chandler’s Syndrome: Pathophysiology, Diagnosis, Management, and Future Perspectives”. Deneysel Ve Klinik Tıp Dergisi 42, no. 2 (June 2025): 212-18.
EndNote Urmıla G, Mugada V, Rajana AL, Kilaparthi LK, Yarguntla SR (June 1, 2025) A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives. Deneysel ve Klinik Tıp Dergisi 42 2 212–218.
IEEE G. Urmıla, V. Mugada, A. L. Rajana, L. K. Kilaparthi, and S. R. Yarguntla, “A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives”, J. Exp. Clin. Med., vol. 42, no. 2, pp. 212–218, 2025.
ISNAD Urmıla, Gorle et al. “A Comprehensive Review on Chandler’s Syndrome: Pathophysiology, Diagnosis, Management, and Future Perspectives”. Deneysel ve Klinik Tıp Dergisi 42/2 (June 2025), 212-218.
JAMA Urmıla G, Mugada V, Rajana AL, Kilaparthi LK, Yarguntla SR. A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives. J. Exp. Clin. Med. 2025;42:212–218.
MLA Urmıla, Gorle et al. “A Comprehensive Review on Chandler’s Syndrome: Pathophysiology, Diagnosis, Management, and Future Perspectives”. Deneysel Ve Klinik Tıp Dergisi, vol. 42, no. 2, 2025, pp. 212-8.
Vancouver Urmıla G, Mugada V, Rajana AL, Kilaparthi LK, Yarguntla SR. A comprehensive review on Chandler’s syndrome: Pathophysiology, diagnosis, management, and future perspectives. J. Exp. Clin. Med. 2025;42(2):212-8.