J Korean Ophthalmol Soc.  2019 Sep;60(9):909-914. 10.3341/jkos.2019.60.9.909.

Iridocorneal Endothelial Syndrome with Features of Posterior Polymorphous Corneal Dystrophy

• Na JH
• Lee HK

• KMID: 2459139
• DOI: http://doi.org/10.3341/jkos.2019.60.9.909

Abstract

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.

Keyword

Chandler syndrome; ICE cell; Iridocorneal endothelial syndrome; Posterior polymorphous corneal dystrophy; Specular microscopy

MeSH Terms

Cornea
Diagnosis
Endothelial Cells
Female
Humans
Intraocular Pressure
Iridocorneal Endothelial Syndrome*
Iris
Middle Aged
Pupil
Reference Values
Tears
Visual Acuity

Figure

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  Figure 1 Slit-lamp photography of both eyes at initial examination. (A) In the right eye, a single pair of horizontal parallel lines were observed at corneal endothelial layer (arrows). (B) In the left eye, the multiple pairs of parallel lines were observed at endothelial layer. The lines were running obliquely through the center of the cornea (arrowheads).

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  Figure 2 Anterior segment optical coherence tomography of both eyes. The lesions were protruded from the endothelial layer. The lesions were more prominent in the left eye (arrowheads) compared to that of right eye (arrow).

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  Figure 3 Specular microscopy of both eyes at initial examination. (A) In the right eye, the endothelial cells became large and the density of the cells decreased to 668 cells/mm2. (B) In the left eye, the normal hexagonal shape of the endothelial cells disappeared. The multiple abnormal endothelial cells with dark-light reversal were observed (arrows).

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  Figure 4 Gonioscopy of both eyes. The angle of the right eye was open. In contrast, the focal peripheral anterior synechiae were observed in the left eye (arrow).

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