Confocal Microscopic Findings of Avellino Corneal Dystrophy According to Disease Severity

Young Min Park; Ho Yun Kim; Jong Soo Lee

doi:10.3341/jkos.2014.55.3.361

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Park, Kim, and Lee: Confocal Microscopic Findings of Avellino Corneal Dystrophy According to Disease Severity

Original Article

J Korean Ophthalmol Soc 2014;55(3):361-367.

Published online: 7 September 2014

DOI: https://doi.org/10.3341/jkos.2014.55.3.361

Confocal Microscopic Findings of Avellino Corneal Dystrophy According to Disease Severity

Young Min Park, MD, Ho Yun Kim, MD, Jong Soo Lee, MD, PhD

Department of Ophthalmology, Pusan National University Hospital, Pusan National University & Medical Research Institute, School of Medicine, Busan, Korea

Address reprint requests to Jong Soo Lee, MD, PhD Department of Ophthalmology, Pusan National University Hospital, #179 Gudeok-ro, Seo-gu, Busan 602-739, Korea, Tel: 82-51-240-7323, Fax: 82-51-242-7341, E-mail: jongsool@pusan.ac.kr

Received 12 October 201311 December 2013 Accepted 17 February 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

In this study we analyzed and objectified the characteristics of the Avellino corneal dystrophy patients consider-ing disease severity using in vivo confocal microscopy (IVCM).

Methods

Each corneal layer of 36 eyes in 18 patients with Avellino corneal dystrophy was examined using IVCM (ConfoScan 4.0, NIDEK, Co. Ltd., Albignasego, Italy). Patients were classified into 3 groups based on disease severity (mild, moderate, or severe).

Results

In the mild group, hyper-reflective granular deposits without dark shadows were observed in the anterior stroma. As the disease progressed, corneal deposits were also found at the posterior stroma and epithelium, and clusters of hyper-reflective corneal deposits resembling stromal opacity were noted. The range of corneal deposits measured using Z-scan optical pachymeter was 111.14 ± 30.95 um in the mild group, 157.47 ± 25.00 um in the moderate group, and 193.42 ± 52.23 um in the severe group (p < 0.05).

Conclusions

The origin of the corneal deposits in Avellino corneal dystrophy may be related to corneal stromal cells and distributed from the corneal epithelial layer to the stromal layer. IVCM might be useful for the standardization of disease severity by digitalizing the range of deposits.

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Keywords: Avellino corneal dystrophy, Confocal microscopy, Z-scan optical pachymeter

References

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Figure 1.

(Case 1) Corneal images obtained from left eye of a “mild” Avellino corneal dystrophy patient by confocal microscopy. (A) Slit lamp photograph reveals mild granular corneal deposits. (B) Normal wing cell layer of the corneal epithelium. (C) Deposition of hyper-reflective materials in the anterior stroma (black arrows). (D) Some hyper-reflective deposits in the mid-stroma (black arrow). (E) The range of corneal deposits measured by Z-scan optical pachymeter is 98 um.

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Figure 2.

(Case 2) Corneal images obtained from left eye of a “moderate” Avellino corneal dystrophy patient by confocal microscopy. (A) Slit lamp photograph reveals round white corneal deposits and scattered stellate opacities. (B) Hyper-reflective deposits are found in the area of epithelium (black arrow). (C) Hyper-reflective materials deposit in the anterior stroma (white arrow). (D) Cluster of hyper-reflective deposits in the posterior stroma (black arrow). (E) The range of corneal deposits measured by Z-scan optical pachymeter is 128 um.

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Figure 3.

(Case 3) Corneal images obtained from the left eye of a “severe” Avellino corneal dystrophy patient by confocal microscopy. (A) Slit lamp photograph reveals multiple round white-gray corneal deposits. (B) Hyper-reflective corneal deposits in the wing cell layer of corneal epithelium. (C) Cluster of hyper-reflective deposits in the anterior stroma, resembling stromal scar. (D) Linear shaped hyper-reflective deposits are observed in the posterior stroma (black arrows). (E) The range of corneal deposits measured by Z-scan optical pachymeter is 201 um.

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Table 1.

Baseline information of the Avellino corneal dystrophy patients

Patient number	Age (years)	Laterality	Sex	BCVA (Snellen)	IOP	Severity	Rangge of corneal deposit (um)
1	69	OD	M	0.5	12	Moderate	139
2	69	OS	M	0.5	10	Moderate	167
3	56	OD	F	0.5	15	Moderate	125
4	56	OS	F	0.8	12	Mild	199
5	43	OD	F	0.8	11	Mild	131
6	43	OS	F	0.8	11	Mild	123
7	69	OD	F	0.2	11	Severe	179
8	69	OS	F	0.16	10	Severe	129
9	55	OD	F	0.4	11	Moderate	162
10	55	OS	F	0.25	9	Severe	239
11	52	OD	F	1	10	Mild	88
12	52	OS	F	0.8	10	Mild	109
13	67	OD	F	0.4	9	Moderate	110
14	67	OS	F	0.5	13	Moderate	178
15	32	OD	M	0.32	15	Severe	263
16	32	OS	M	0.56	10	Moderate	184
17	72	OD	F	1	14	Mild	93
18	72	OS	F	0.4	17	Moderate	179
19	33	OD	F	0.8	13	Mild	135
20	33	OS	F	0.8	10	Mild	129
21	52	OD	F	0.8	15	Mild	97
22	52	OS	F	0.8	13	Mild	89
23	68	OD	M	0.5	8	Moderate	184
24	68	OS	M	0.63	7	Moderate	179
25	53	OD	F	1	11	Mild	92
26	53	OS	F	0.8	12	Mild	98
27	46	OD	F	1	10	Mild	86
28	46	OS	F	1	11	Mild	87
29	54	OD	F	0.5	15	Moderate	128
30	54	OS	F	0.5	13	Moderate	139
31	28	OD	F	0.5	12	Moderate	143
32	28	OS	F	0.5	15	Moderate	159
33	43	OD	F	0.32	12	Severe	136
34	43	OS	F	0.1	13	Severe	232
35	28	OD	F	0.32	10	Severe	176
36	28	OS	F	0.4	10	Moderate	186

BCVA = best corrected visual acuity; IOP = intraocular pressure.

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