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Chandler's Syndrome as a Variant of Essential Iris Atrophy: A Clinicopathologic Study

Chandler's Syndrome as a Variant of Essential Iris Atrophy: A Clinicopathologic Study Abstract • Trabeculectomy and peripheral iridectomy specimens from one male and two female patients with Chandler's syndrome (age, 30 to 42 years) showed that all had unilateral corneal endothelial "dystrophy," corneal edema, mild to moderate iris atrophy without holes, peripheral anterior synechiae, and glaucoma. In one, fluorescein angiography of the iris disclosed a sector filling delay of limbal and conjunctival vessels and pupillary and extrapupillary leakage. Histopathologic examination showed a layer of degenerated corneal endothelium and Descemet's membrane extending across the inner uveal trabeculum. Descemet's membrane displayed irregular, nodular, scroll-like excrescences in some cases, and thinner placoid configurations with abnormal widely spaced collagen (100 nm) in others. Corneal endothelial cells exhibited increased microvilli, widened cellular interdigitations, and occasional shrunken cells with enlarged or disrupted cytoplasmic blebs. Peripheral iris specimens displayed mild to moderate stromal atrophy without vascular occlusions. Pigment epithelium was normal. References 1. Chandler P: Atrophy of the stroma of the iris: Endothelial dystrophy, corneal edema and glaucoma . Am J Ophthalmol 41:607-615, 1956. 2. Cordero-Moreno R: Atrofia mesodermica del iris, distrofia de cornea y glaucoma secundario sindrome de Chandler . Arch Soc Esp Oftal 31:667-675, 1971. 3. Chandler PA, Grant WM: Lectures on Glaucoma . Philadelphia, Lea & Febiger, 1965, pp 283-285. 4. Shields MB, Campbell DG, Simmonds RJ, et al: Iris nodules in essential iris atrophy . Arch Ophthalmol 94:406-410, 1976.Crossref 5. Scheie HG, Yanoff M, Kellogg WT: Essential iris atrophy: Report of a case . Arch Ophthalmol 94:1315-1320, 1976.Crossref 6. Heath P: Essential atrophy of the iris: A histopathologic study . Trans Am Ophthalmol Soc 51:167-192, 1953. 7. Sugar HS: Glaucoma and essential progressive atrophy of the iris . Am J Ophthalmol 28:744-748, 1945. 8. Kaiser-Kupfer M, Kuwabara T, Kupfer C: Progressive bilateral essential iris atrophy . Am J Ophthalmol 83:340-346, 1977. 9. Cogan DG, Reese AB: A syndrome of iris nodules, ectopic Descemet membrane and unilateral glaucoma . Doc Ophthalmol 26:424-433, 1969.Crossref 10. Scheie HG, Yanoff M: Iris nevus (Cogan-Reese) syndrome: A cause of unilateral glaucoma . Arch Ophthalmol 93:963-970, 1975.Crossref 11. Kayes J, Holmberg A: The fine structure of the cornea in Fuchs endothelial dystrophy . Invest Ophthalmol 3:47-67, 1964. 12. Feeney L: Ultrastructure of the nerves in the human trabecular region . Invest Ophthalmol 1:462-473, 1962. 13. Svedbergh B, Bill A: Scanning electron microscopic studies of the corneal endothelium in man and monkeys . Acta Ophthalmol 50:321-336, 1972.Crossref 14. Davies PD, Kirkham JB, Villaneuva S: Surface ultrastructure of human donor corneal endothelium . Trans Ophthal Soc UK 96:96-104, 1976. 15. Pfister RW: The healing of corneal epithelial abrasions in the rabbit: A scanning electron microscope study . Invest Ophthalmol 14:648-661, 1975. 16. Waring GO, Laibson PR, Rodrigues MM: Clinical and pathologic alterations of Descemet membrane with emphasis on endothelial metaplasia . Surv Ophthalmol 18:325-368, 1974. 17. Waring GO, Font RL, Rodrigues MM, et al: Alterations of Descemet membrane in interstitial keratitis . Am J Ophthalmol 81:773-785, 1976. 18. Polack FM: Contributions of electron microscopy to the study of corneal pathology . Surv Ophthalmol 20:375-414, 1976.Crossref 19. Doughman DJ, Van Horn DL, Rodman WP, et al: Human corneal endothelial layer repair during organ culture . Arch Ophthalmol 94:1791-1796, 1976.Crossref 20. Van Horn DL, Hyndiuk RA: Endothelial wound repair in primate cornea . Exp Eye Res 21:113-124, 1975.Crossref 21. Bruun-Jensen J: Fluorescein angiography of the anterior segment . Am J Ophthalmol 67:842-845, 1969. 22. Craadnijk A, Aan de Kerk L: Fluorescein angiography of the iris . Br J Ophthalmol 54:229-232, 1970.Crossref 23. Nieuwenhuis EA: Fluorescein angiography of the iris . Doc Ophthalmol 40:255-268, 1976.Crossref 24. Clements DH, Oberman AE: Iris fluorescein angiography in iris atrophy with glaucoma . Trans Pac Coast Ophthalmol Soc 53:187-197, 1972. 25. Jampol LM, Rosser MJ, Sears ML: Unusual aspects of progressive essential iris atrophy . Am J Ophthalmol 77:353-357, 1974. 26. Cibis GW, Krachmer JH, Phelps CD, et al: Iridocorneal adhesions in posterior polymorphous dystrophy . Trans Am Acad Ophthalmol Otolaryngol 81:770-777, 1976. 27. Chandler PA: Progress in the treatment of glaucoma in my lifetime . Surv Ophthalmol 21:412-428, 1977.Crossref 28. Sugar HS: Endothelial dystrophy and glaucoma in the essential iris atrophy syndrome. Perspect Ophthalmol, to be published. 29. Mills PV: Iridoschisis . Br J Ophthalmol 51:158-164, 1967.Crossref 30. Weatherill JR, Hart CT: Familial hypoplasia of the iris stroma associated with glaucoma . Br J Ophthalmol 53:433-438, 1969.Crossref 31. Walton DS, Grant WM: Distinctive gonioscopic findings in glaucoma due to neurofibromatosis . Arch Ophthalmol 79:127-134, 1968.Crossref 32. Cairns JE, Krasnov MM: Iridocyclo-retraction in glaucoma secondary to essential iris atrophy . Br J Ophthalmol 57:416-417, 1973.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Chandler's Syndrome as a Variant of Essential Iris Atrophy: A Clinicopathologic Study

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1978.03910050339009
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Abstract

Abstract • Trabeculectomy and peripheral iridectomy specimens from one male and two female patients with Chandler's syndrome (age, 30 to 42 years) showed that all had unilateral corneal endothelial "dystrophy," corneal edema, mild to moderate iris atrophy without holes, peripheral anterior synechiae, and glaucoma. In one, fluorescein angiography of the iris disclosed a sector filling delay of limbal and conjunctival vessels and pupillary and extrapupillary leakage. Histopathologic examination showed a layer of degenerated corneal endothelium and Descemet's membrane extending across the inner uveal trabeculum. Descemet's membrane displayed irregular, nodular, scroll-like excrescences in some cases, and thinner placoid configurations with abnormal widely spaced collagen (100 nm) in others. Corneal endothelial cells exhibited increased microvilli, widened cellular interdigitations, and occasional shrunken cells with enlarged or disrupted cytoplasmic blebs. Peripheral iris specimens displayed mild to moderate stromal atrophy without vascular occlusions. Pigment epithelium was normal. References 1. Chandler P: Atrophy of the stroma of the iris: Endothelial dystrophy, corneal edema and glaucoma . Am J Ophthalmol 41:607-615, 1956. 2. Cordero-Moreno R: Atrofia mesodermica del iris, distrofia de cornea y glaucoma secundario sindrome de Chandler . Arch Soc Esp Oftal 31:667-675, 1971. 3. Chandler PA, Grant WM: Lectures on Glaucoma . Philadelphia, Lea & Febiger, 1965, pp 283-285. 4. Shields MB, Campbell DG, Simmonds RJ, et al: Iris nodules in essential iris atrophy . Arch Ophthalmol 94:406-410, 1976.Crossref 5. Scheie HG, Yanoff M, Kellogg WT: Essential iris atrophy: Report of a case . Arch Ophthalmol 94:1315-1320, 1976.Crossref 6. Heath P: Essential atrophy of the iris: A histopathologic study . Trans Am Ophthalmol Soc 51:167-192, 1953. 7. Sugar HS: Glaucoma and essential progressive atrophy of the iris . Am J Ophthalmol 28:744-748, 1945. 8. Kaiser-Kupfer M, Kuwabara T, Kupfer C: Progressive bilateral essential iris atrophy . Am J Ophthalmol 83:340-346, 1977. 9. Cogan DG, Reese AB: A syndrome of iris nodules, ectopic Descemet membrane and unilateral glaucoma . Doc Ophthalmol 26:424-433, 1969.Crossref 10. Scheie HG, Yanoff M: Iris nevus (Cogan-Reese) syndrome: A cause of unilateral glaucoma . Arch Ophthalmol 93:963-970, 1975.Crossref 11. Kayes J, Holmberg A: The fine structure of the cornea in Fuchs endothelial dystrophy . Invest Ophthalmol 3:47-67, 1964. 12. Feeney L: Ultrastructure of the nerves in the human trabecular region . Invest Ophthalmol 1:462-473, 1962. 13. Svedbergh B, Bill A: Scanning electron microscopic studies of the corneal endothelium in man and monkeys . Acta Ophthalmol 50:321-336, 1972.Crossref 14. Davies PD, Kirkham JB, Villaneuva S: Surface ultrastructure of human donor corneal endothelium . Trans Ophthal Soc UK 96:96-104, 1976. 15. Pfister RW: The healing of corneal epithelial abrasions in the rabbit: A scanning electron microscope study . Invest Ophthalmol 14:648-661, 1975. 16. Waring GO, Laibson PR, Rodrigues MM: Clinical and pathologic alterations of Descemet membrane with emphasis on endothelial metaplasia . Surv Ophthalmol 18:325-368, 1974. 17. Waring GO, Font RL, Rodrigues MM, et al: Alterations of Descemet membrane in interstitial keratitis . Am J Ophthalmol 81:773-785, 1976. 18. Polack FM: Contributions of electron microscopy to the study of corneal pathology . Surv Ophthalmol 20:375-414, 1976.Crossref 19. Doughman DJ, Van Horn DL, Rodman WP, et al: Human corneal endothelial layer repair during organ culture . Arch Ophthalmol 94:1791-1796, 1976.Crossref 20. Van Horn DL, Hyndiuk RA: Endothelial wound repair in primate cornea . Exp Eye Res 21:113-124, 1975.Crossref 21. Bruun-Jensen J: Fluorescein angiography of the anterior segment . Am J Ophthalmol 67:842-845, 1969. 22. Craadnijk A, Aan de Kerk L: Fluorescein angiography of the iris . Br J Ophthalmol 54:229-232, 1970.Crossref 23. Nieuwenhuis EA: Fluorescein angiography of the iris . Doc Ophthalmol 40:255-268, 1976.Crossref 24. Clements DH, Oberman AE: Iris fluorescein angiography in iris atrophy with glaucoma . Trans Pac Coast Ophthalmol Soc 53:187-197, 1972. 25. Jampol LM, Rosser MJ, Sears ML: Unusual aspects of progressive essential iris atrophy . Am J Ophthalmol 77:353-357, 1974. 26. Cibis GW, Krachmer JH, Phelps CD, et al: Iridocorneal adhesions in posterior polymorphous dystrophy . Trans Am Acad Ophthalmol Otolaryngol 81:770-777, 1976. 27. Chandler PA: Progress in the treatment of glaucoma in my lifetime . Surv Ophthalmol 21:412-428, 1977.Crossref 28. Sugar HS: Endothelial dystrophy and glaucoma in the essential iris atrophy syndrome. Perspect Ophthalmol, to be published. 29. Mills PV: Iridoschisis . Br J Ophthalmol 51:158-164, 1967.Crossref 30. Weatherill JR, Hart CT: Familial hypoplasia of the iris stroma associated with glaucoma . Br J Ophthalmol 53:433-438, 1969.Crossref 31. Walton DS, Grant WM: Distinctive gonioscopic findings in glaucoma due to neurofibromatosis . Arch Ophthalmol 79:127-134, 1968.Crossref 32. Cairns JE, Krasnov MM: Iridocyclo-retraction in glaucoma secondary to essential iris atrophy . Br J Ophthalmol 57:416-417, 1973.Crossref

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Apr 1, 1978

References