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Iridocorneal Endothelial Syndrome with Features of Posterior Polymorphous Corneal Dystrophy
후부다형각막이상증의 특징을 보이는 홍채각막내피증후군
JKOS 2019 Sep;60(9):909-14
Published online September 15, 2019;
Copyright © 2019 The Korean Ophthalmological Society.
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Jeong Ho Na, MD, Hyo Kyung Lee, MD
나정호 · 이효경

Department of Ophthalmology, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea
인제대학교 의과대학 해운대백병원 안과학교실
Received March 14, 2019; Revised April 21, 2019; Accepted August 16, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Purpose: To report a case of iridocorneal endothelial syndrome, which overlapped with some of the features of posterior polymorphous corneal dystrophy.
Case summary: A 61-year-old female presented with tearing pain and blurred vision in her left eye, which was aggravated in the morning. The symptom started approximately 1 year prior to her visit. At the initial visit, the visual acuities were 1.0 in both eyes and the intraocular pressures were normal. On slit-lamp examination, a single pair of horizontal parallel lines was observed at the central corneal endothelial layer in the right eye. In contrast, multiple pairs of oblique parallel lines were observed in the left eye. The lines of the lesions were more prominent and wavier in the left eye than those of the right eye. The overlying cornea was clear, and the corneal thicknesses were in the normal range in both eyes. Using a gonioscopic examination, localized peripheral anterior synechiae were observed only in the left eye. The pupil and iris were normal in both eyes. On specular microscopic examination, the corneal endothelial cell size in the right eye increased and the corneal endothelial density decreased to 668 cells/mm2. In the left eye, multiple abnormal endothelial cells with dark-light reversal were observed. In conclusion, the patient was subsequently diagnosed with iridocorneal syndrome, rather than posterior polymorphous corneal dystrophy.
Conclusions: Posterior polymorphous corneal dystrophy and iridocorneal endothelial syndrome may present with many similarities. Therefore, in cases of uncertain diagnosis, an understanding of the clinical features is important for proper diagnosis.
Keywords : Chandler syndrome, ICE cell, Iridocorneal endothelial syndrome, Posterior polymorphous corneal dystrophy, Specular microscopy


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