Letters

Figure 2. Optical Coherence Tomographic Images A

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. Funding/Support: This study was supported by grant R01-EY15569 from the National Eye Institute and by the Foundation Fighting Blindness, the Stephen A. Wynn Foundation, and the Howard Hughes Medical Institute. Role of the Funder/Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

200 µm

1. Alzial C, Dufier JL, Brasnu C, Aicardi J, de Grouchy J. “True” microcephaly with dominant-inheritance chorioretinal dysplasia [in French]. Ann Genet. 1980;23 (2):91-94.

200 µm

B

2. Ostergaard P, Simpson MA, Mendola A, et al. Mutations in KIF11 cause autosomal-dominant microcephaly variably associated with congenital lymphedema and chorioretinopathy. Am J Hum Genet. 2012;90(2):356-362. 3. Opitz JM. On congenital lymphedema. Am J Med Genet. 1986;24(1):127-129. 4. Myers KA, Baas PW. Kinesin-5 regulates the growth of the axon by acting as a brake on its microtubule array. J Cell Biol. 2007;178(6):1081-1091.

200 µm

200 µm

Pigmented Limbal Nodule Consistent With a Ciliary Body Nevus in an Organ Donor

C

200 µm

With the exception of melanocytomas, ciliary body nevi are rare. Previous case series have reported episcleral nodular extension of ciliary body nevi leading to their diagnosis1 or alternatively ciliary body nevi with involvement of the iris root, resulting in their detection using slitlamp biomicroscopy.2 Herein, we report a pigmented limbal nodule consistent with a ciliary body nevus diagnosed post mortem in a donor eye as an incidental finding during corneoscleral disc preparation for transplantation.

200 µm

D

200 µm

200 µm

Horizontal (A and C) and vertical (B and D) optical coherence tomographic images of the right (A and B) and left (C and D) eyes showing thinning of the retina and loss of the ellipsoid zone in the perifoveal area. Green lines indicate the direction of the scan.

these other disorders, the mechanism involved in disease associated with KIF11 mutation is likely not to be related to intraflagellar transport. Katrina Mears, MD Benjamin Bakall, MD, PhD Lisa A. Harney, BA Jessica A. Penticoff, BA Edwin M. Stone, MD, PhD Author Affiliations: Department of Ophthalmology and Visual Sciences, Carver College of Medicine, University of Iowa, Iowa City (Mears, Bakall, Harney, Penticoff, Stone); Stephen A. Wynn Institute for Vision Research, University of Iowa, Iowa City (Mears, Bakall, Harney, Penticoff, Stone); Howard Hughes Medical Institute, University of Iowa, Iowa City (Stone). Corresponding Author: Edwin M. Stone, MD, PhD, Stephen A. Wynn Institute for Vision Research, University of Iowa, 375 Newton Rd, 4111 MERF, Iowa City, IA 52242 ([email protected]). Published Online: March 12, 2015. doi:10.1001/jamaophthalmol.2015.199. jamaophthalmology.com

Report of a Case | We received an enucleated eye from a male organ donor in his early 50s. It had a separated corneoscleral disc and a single well-circumscribed brown-pigmented limbal nodule. The donor kidneys had already been transplanted, and hence there was the concern of potential malignancy of this lesion while preparing the cornea for storage in the eye bank. The requested histomorphological examination of the remaining enucleated eye showed autolytic changes only, without any evidence of a choroidal melanoma. The corneoscleral disc contained anterior ciliary body muscle, trabecular meshwork, and cornea. A small flat tumor embedded in the ciliary body was observed, composed of homogeneous spindle cells that were pigmented with ovoid nuclei and without any cellular atypia (Figure 1). These cells extended along transscleral blood vessels into the episclera, where they formed a 2 × 0.5-mm nodule. Immunohistochemistry demonstrated melan A– and S-100 protein–positive spindle cells, with a Ki-67 proliferation rate less than 1%. Molecular genetic analysis by multiplex ligation-dependent probe amplification of both the intraocular and extraocular components of the lesion showed normal patterns for chromosomes 1p, 3, 6, and 8 (Figure 2); no GNAQ or GNA11 mutations were detected by sequencing. Taken together, these results were consistent with the histomorphological diagnosis of a ciliary body nevus with involvement of the episcleral conjunctiva. Discussion | This case is rare. To our knowledge, there has been no previous description of a ciliary body nevus in a donor eye with transscleral extension. (Reprinted) JAMA Ophthalmology June 2015 Volume 133, Number 6

Copyright 2015 American Medical Association. All rights reserved.

Downloaded From: http://archopht.jamanetwork.com/ by a Cornell University User on 09/26/2016

721

Letters

Figure 1. Histopathological Analysis of the Ciliary Body Nevus A

B

C

D

A, Corneoscleral disc showing ciliary body nevus with transscleral extension into the episclera (original magnification ×3.4, hematoxylin-eosin). The area outlined by the box is shown in B. B, Intrascleral extension of bland nevus cells (original magnification ×40, hematoxylin-eosin). C, Melan A–positive nevus cells in the episcleral nodule (original magnification ×3.4, 3,3'-diaminobenzidine immunostaining). D, S-100 protein–positive nevus cells (original magnification ×40, 3,3'-diaminobenzidine immunostaining).

Figure 2. Results of Multiplex Ligation-Dependent Probe Amplification of the Ciliary Body Nevus 3.0

Control probes

Chromosome 1p

Chromosome 3

Chromosome 6

Chromosome 8

2.5

Peak Ratio

2.0

1.5

1.0

0.5

C9 C1 0 NO C12 TC H M 2 FN GJ 2 RP B3 E6 NB 5 PT L1 A M FR UT Y M H LH 1 M FHI I R T1 12 8CH 2 BA L1 P1 P (i) C3 PAR OR G F1 FH 0 IT RB 2 M VH 5 RO L1 BO V 1 CT HL2 NN B1 BA XPC P1 (ii M ) M CA E PR SR OS OP 1 RU A1 NX E 2 CD C12 KN DC 1A DC IG 2 F2 CT R G LZ F TS NR 1 M G1 Y M C(i) YC (ii ) R AS P 1 AP 1

C7 C8

C6

C4

C3

0

Gene

722

Results of multiplex ligation-dependent probe amplification of the ciliary body nevus. The loci examined are indicated by their gene names on the x-axis. The parallel dashed lines indicate the range within which the loci are considered to

be normal for uveal melanoma. The overall status for chromosomes 1p, 3, 6, and 8 was considered to be normal for this case.

The differential diagnoses of a single well-circumscribed episcleral brown-pigmented nodule include conjunctival melanocytic neoplasias, conjunctival pigmented squamous tumors, traumatic pigment inclusions, extraocular extension of a uveal nevus (the most common pseudomelanoma3), or a uveal melanoma. The dominance of the small tumor in this case within the ciliary body and its associated bland histomorphological features (also seen in the extraocular component) spoke for the earlier-mentioned diagnosis and against the other differential diagnoses. This was supported by the results of the molecular genetic analysis of the tumor for chromosomes 1p, 3, 6, and 8, which proved to be consistent with a benign me-

lanocytic tumor. The unusual transscleral extension observed is reminiscent of that seen in hyperpigmented magnocellular nevi (also termed melanocytomas) 4 and also occasionally in cases of bilateral diffuse uveal melanocytic proliferation. Because the kidneys of this organ donor had already been transplanted into 2 patients prior to the preparation of both donor eyes for corneal transplantation, there was considerable concern that the donor had an undiagnosed ocular melanoma that had potentially already metastasized and could be a danger for the organ recipients, as previously described.5 As a result of our histomorphological and molecular genetic find-

JAMA Ophthalmology June 2015 Volume 133, Number 6 (Reprinted)

Copyright 2015 American Medical Association. All rights reserved.

Downloaded From: http://archopht.jamanetwork.com/ by a Cornell University User on 09/26/2016

jamaophthalmology.com

Letters

ings, we were confident that the transplanted kidneys did not require explantation, although special follow-up of the recipients was recommended. Eye donor selection guidelines in Europe and the United States allow systemic malignant neoplasms (except hematological) to be present provided there is no ocular involvement, but ocular malignant neoplasms are contraindications to corneal transplantation. 6 Our case suggests that thorough posterior segment examination of all donor eyes also be considered. In conclusion, this case presents an unusual finding in an eye from an organ donor and highlights the need for careful examination of all donor eyes prior to corneal transplantation. Ebba Nissen, MD John Armitage, PhD Sophie Thornton, BSc Sarah E. Coupland, MBBS, PhD, FRCPath

Figure 1. BrightOcular Cosmetic Iris Implant in the Eye

Slitlamp color photograph of the cosmetic iris implant in the right eye.

Figure 2. Anterior Segment Optical Coherence Tomographic Image of the Cosmetic Iris Implant

Author Affiliations: Liverpool Ocular Oncology Research Group, Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, England (Nissen, Thornton, Coupland); Department of Ophthalmology, CTS Eye Bank, Bristol, England (Armitage). Corresponding Author: Sarah E. Coupland, MBBS, PhD, FRCPath, Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Sixth Floor, Duncan Bldg, Daulby Street, Liverpool L69 3GA, England ([email protected]). Published Online: March 19, 2015. doi:10.1001/jamaophthalmol.2015.214. Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. 1. Taban M, Sears JE, Singh AD. Ciliary body naevus. Eye (Lond). 2007;21(12): 1528-1530. 2. Jakobiec FA, Gragoudas ES, Colby KA. Biopsy of an anterior episcleral nodule as an aid in managing a ciliary body melanocytic tumor. Cornea. 2013;32(8): 1174-1177. 3. Shields CL, Manalac J, Das C, Ferguson K, Shields JA. Choroidal melanoma: clinical features, classification, and top 10 pseudomelanomas. Curr Opin Ophthalmol. 2014;25(3):177-185. 4. Esmaili DD, Mukai S, Jakobiec FA, Kim IK, Gragoudas ES. Ocular melanocytoma. Int Ophthalmol Clin. 2009;49(1):165-175. 5. Bilal M, Eason JD, Das K, Sylvestre PB, Dean AG, Vanatta JM. Donor-derived metastatic melanoma in a liver transplant recipient established by DNA fingerprinting. Exp Clin Transplant. 2013;11(5):458-463. 6. European Eye Bank Association. Minimum medical standards: donor medical assessment. http://www.eeba.eu/downloads/EEBA%20Minimum%20Medical %20Standards%20Rev%201%20-%202013Agreed.pdf. Accessed October 23, 2014.

Corneal Endothelial Cell Loss and Iritis Associated With a New Cosmetic Iris Implant Anterior chamber cosmetic iris implants permanently alter the apparent iris color. Previous reports of these implants highlight adverse outcomes with severe ocular morbidity, including glaucoma, corneal endothelial decompensation, iris damage, and uveitis, which may result in vision loss.1-3 These reports concern the NewColorIris implant (Kahn Medical Devices). We report a case involving a new cosmetic iris implant, BrightOcular (Stellar Devices LLC), causing iris damage, uveitis, and corneal endothelial damage. jamaophthalmology.com

Anterior segment optical coherence tomographic image showing the cosmetic iris implant with iris apposition and iris tuck in the nasal angle of the left eye (arrowhead).

Report of a Case | A woman in her mid-30s had bilateral eye redness and irritation. She reported having surgery in Lebanon 1 week prior to change the color of her iris with a BrightOcular implant. The patient’s best-corrected visual acuity was 20/30 OU and the intraocular pressure was 11 mm Hg OU. Slitlamp examination revealed bilateral ciliary flush, bilateral small pigmented keratic precipitates, and bilateral 2+ pigmented cell in the anterior chamber. The anterior chamber cosmetic iris implants appeared to be immobile, with the patient’s natural iris visible posterior to the implant (Figure 1). Anterior segment optic al coherence tomography (Visante; Carl Zeiss Meditec) and gonioscopy demonstrated bilateral apposition of the implant to the iris and angle structures. In the left eye, the nasal iris was tucked into the angle by the implant (Figure 2). Specular microscopy (Noncon Robo P; Konan Medical) demonstrated a markedly reduced endothelial cell count, polymegathism, and pleomorphism of the right cornea. The left eye demonstrated normal endothelial cell morphology. The patient was managed medically with topical dexamethasone, moxifloxacin, and dorzolamide hydrochloride/ timolol. Bilateral cosmetic iris implant removal surgery was performed. The implants were thin, flat, and composed of a flexible (Reprinted) JAMA Ophthalmology June 2015 Volume 133, Number 6

Copyright 2015 American Medical Association. All rights reserved.

Downloaded From: http://archopht.jamanetwork.com/ by a Cornell University User on 09/26/2016

723

Pigmented Limbal Nodule Consistent With a Ciliary Body Nevus in an Organ Donor.

Pigmented Limbal Nodule Consistent With a Ciliary Body Nevus in an Organ Donor. - PDF Download Free
447KB Sizes 0 Downloads 6 Views