Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
A Moving Black Spot in My Vision Mauricio Vélez, MD; Diana L. Hernández, MD; Sebastián Rojas, MD
Figure 1. Free-floating iris cyst in the anterior chamber.
A 22-year-old man had perceived a moving black spot in the visual field of his right eye and no other symptoms for 1 year. An ophthalmologic examination revealed visual acuity without correction of 20/20 in both eyes, and slitlamp examination findings of the right eye identified a unique lesion that was oval in shape, measured 2 mm in thickness by 2 mm in diameter, Video at jamaophthalmology was translucent and brown, and changed its location .com with head posture. During gonioscopy, we observed a nonpigmented Quiz at jamaophthalmology.com wide angle without other findings that suggested adhesions between the lesion and the structures of the camerular angle. The patient had a clear cornea without pathologic findings, and the intraocular pressure with Goldman tonometer was 10 mm Hg in both eyes. Findings from a dilated fundus examination were unremarkable (Figure 1).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. A computerized tomographic scan B. Anterior segment tomography with Pentacam C. Begin systemic treatment with albendazole D. Surgical removal of the cyst and pathological analysis
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Tufts Univ. Hirsh Health Sciences Library User on 11/24/2015
769
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Free-floating pigmented iris cyst in the anterior chamber
What To Do Next D. Surgical removal of the cyst and pathological analysis Free-floating pigmented iris cysts are benign, rare, unique, and unilateral lesions that originate from the iris pigment epithelium. This type of cyst usually does not interfere with vision and has not been associated with complications such as glaucoma, cataract, uveitis, and corneal decompensation (Video 1). Although expectant observation is the appropriate treatment, in this case, owing to the patient’s constant perception of a moving spot in the visual field of the affected eye, surgical removal of the cyst was performed (Video 2). The histopathology smears revealed a simple wall with pigmented epithelial cells, suggestive of an iris cyst (Figure 2).
Discussion Pigmented iris cysts can be classified as primary or secondary. Primary cysts are uncommon lesions that originate from the iris pigment epithelium, and, in most cases, they can be accurately diagnosed based on the clinical characteristics of each subtype. According to their location and characteristics, primary cysts of the pigment epithelium are classified as central zone, middle zone, peripheral, or free cysts.1 In general, free cysts tend to be unique and located in the vitreous or the anterior chamber, either fixed to the camerular angle or free floating.1,2 It is believed that free cysts originate from central or middle cysts owing to high pigmentation. They can be translucent, suggesting the possible origin of the lesion from peripheral primary cysts.3 Coats4 was the first to report an epithelial cyst in the anterior chamber that changed its position with the movements of the patient’s head. Free pigment iris epithelial cysts are uncommon. Shields et al3 reported 62 cases and found that only 4 of these corresponded to free cysts, most of which were fixed to the angle. Lois et al2 reported in 234 patients with primary iris cysts a 3% incidence of free cysts; only 1 presented with a free-floating cyst in the anterior chamber. Although melanomas and adenomas of the iris generally do not become free-floating cysts in the anterior chamber,5 the possibility of their existence must be considered in a differential diagnosis. Circumscribed melanoma of the iris, unlike free-floating cysts of the epithelium, is usually associated with prominent vascularity spreading in the anterior chamber, increased intraocular pressure, and ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, Universidad Pontificia Bolivariana, Medellín, Colombia. Corresponding Author: Mauricio Vélez, MD, Department of Ophthalmology, Universidad Pontificia Bolivariana, calle 22 sur 44-50, Envigado, Antioquia 574, Colombia ([email protected]). Conflict of Interest Disclosures: None reported. REFERENCES 1. Shields JA. Primary cysts of the iris. Trans Am Ophthalmol Soc. 1981;79:771-809.
770
Figure 2. The histologic smear showing pigmented epithelial cells (hematoxylin-eosin stain, original magnification ×40).
remarkable growth.6 Ultrasound biomicroscopy is very useful for determining the location and size of small uveal tumors. Primary cysts that originate from the stroma tend to occur in children and are characterized by reaching considerable sizes over time, far exceeding the sizes of the free cysts of the iris epithelium.7 After proper identification, the management of this type of cyst can be expectant.1 Rupture of the cyst wall with a yttrium aluminum garnet laser could be attempted; however, owing to the lack of evidence of corneal endothelium cell loss over time and possible visual symptoms, total removal of these lesions and pathological analysis is an effective, easy, and safe approach. The probability of the parasite cysticercus in the anterior chamber, although low, should also be considered in such cases. The main feature that suggests the presence of cysticercus in the anterior chamber is a large translucent cyst that is occasionally associated with increased intraocular pressure due to pupillary block.8 In this case, surgical removal of the cyst and treatment with albendazole and steroids for extraocular cysts would be indicated. In the literature, the origin of secondary cysts has been attributed to surgical and nonsurgical trauma and to the use of topical medications. To date, penetrating keratoplasty, cataract surgery, and radial keratotomy1,9,10 are among the procedures that have been related to the appearance of cysts secondary to surgical trauma. Although the origin is not clear, miotic agents, such as pilocarpine and prostaglandin analogues, specifically latanoprost, have also been related to the presentation of secondary cysts.11
2. Lois N, Shields CL, Shields JA, Mercado G. Primary cysts of the iris pigment epithelium. Ophthalmology. 1998;105(10):1879-1885.
7. Xiao Y, Wang Y-H, Niu G-L, Gao M. Primary iris stromal cyst with rapid growth. Optom Vis Sci. 2009;86(11):1309-1312.
3. Shields JA, Kline MW, Augsburger JJ. Primary iris cysts: a review of the literature and report of 62 cases. Br J Ophthalmol. 1984;68(3):152-166.
8. Chandra A, Singh MK, Singh VP, Rai AK, Chakraborty S, Maurya OPS. A live cysticercosis in anterior chamber leading to glaucoma secondary to pupilary block. J Glaucoma. 2007;16(2):271-273.
4. Coats G. Pigmented cyst lying free in the anterior chamber. Trans Ophthalmol Soc U K. 1912; 32:189-194. 5. Zhou M, Xu G, Bojanowski CM, et al. Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy. Acta Ophthalmol Scand. 2006;84 (1):137-139. 6. Henderson E, Margo CE. Iris melanoma. Arch Pathol Lab Med. 2008;132(2):268-272.
9. Grieser EJ, Dudenhoefer EJ, Waller SG. Long-term follow-up of free-floating epithelial inclusion cyst after radial keratotomy. Cornea. 2007;26(4):512-513. 10. Claoué C, Lewkowicz-Moss S, Easty D. Epithelial cyst in the anterior chamber after penetrating keratoplasty. Br J Ophthalmol. 1988;72(1):36-40. 11. Pruthi S, Kashani S, Ruben S. Bilateral iris cyst secondary to topical latanoprost. Acta Ophthalmol. 2008;86(2):233-234.
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Tufts Univ. Hirsh Health Sciences Library User on 11/24/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
A Moving Black Spot in My Vision Mauricio Vélez, MD; Diana L. Hernández, MD; Sebastián Rojas, MD
Figure 1. Free-floating iris cyst in the anterior chamber.
A 22-year-old man had perceived a moving black spot in the visual field of his right eye and no other symptoms for 1 year. An ophthalmologic examination revealed visual acuity without correction of 20/20 in both eyes, and slitlamp examination findings of the right eye identified a unique lesion that was oval in shape, measured 2 mm in thickness by 2 mm in diameter, Video at jamaophthalmology was translucent and brown, and changed its location .com with head posture. During gonioscopy, we observed a nonpigmented Quiz at jamaophthalmology.com wide angle without other findings that suggested adhesions between the lesion and the structures of the camerular angle. The patient had a clear cornea without pathologic findings, and the intraocular pressure with Goldman tonometer was 10 mm Hg in both eyes. Findings from a dilated fundus examination were unremarkable (Figure 1).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. A computerized tomographic scan B. Anterior segment tomography with Pentacam C. Begin systemic treatment with albendazole D. Surgical removal of the cyst and pathological analysis
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Tufts Univ. Hirsh Health Sciences Library User on 11/24/2015
769
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Free-floating pigmented iris cyst in the anterior chamber
What To Do Next D. Surgical removal of the cyst and pathological analysis Free-floating pigmented iris cysts are benign, rare, unique, and unilateral lesions that originate from the iris pigment epithelium. This type of cyst usually does not interfere with vision and has not been associated with complications such as glaucoma, cataract, uveitis, and corneal decompensation (Video 1). Although expectant observation is the appropriate treatment, in this case, owing to the patient’s constant perception of a moving spot in the visual field of the affected eye, surgical removal of the cyst was performed (Video 2). The histopathology smears revealed a simple wall with pigmented epithelial cells, suggestive of an iris cyst (Figure 2).
Discussion Pigmented iris cysts can be classified as primary or secondary. Primary cysts are uncommon lesions that originate from the iris pigment epithelium, and, in most cases, they can be accurately diagnosed based on the clinical characteristics of each subtype. According to their location and characteristics, primary cysts of the pigment epithelium are classified as central zone, middle zone, peripheral, or free cysts.1 In general, free cysts tend to be unique and located in the vitreous or the anterior chamber, either fixed to the camerular angle or free floating.1,2 It is believed that free cysts originate from central or middle cysts owing to high pigmentation. They can be translucent, suggesting the possible origin of the lesion from peripheral primary cysts.3 Coats4 was the first to report an epithelial cyst in the anterior chamber that changed its position with the movements of the patient’s head. Free pigment iris epithelial cysts are uncommon. Shields et al3 reported 62 cases and found that only 4 of these corresponded to free cysts, most of which were fixed to the angle. Lois et al2 reported in 234 patients with primary iris cysts a 3% incidence of free cysts; only 1 presented with a free-floating cyst in the anterior chamber. Although melanomas and adenomas of the iris generally do not become free-floating cysts in the anterior chamber,5 the possibility of their existence must be considered in a differential diagnosis. Circumscribed melanoma of the iris, unlike free-floating cysts of the epithelium, is usually associated with prominent vascularity spreading in the anterior chamber, increased intraocular pressure, and ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, Universidad Pontificia Bolivariana, Medellín, Colombia. Corresponding Author: Mauricio Vélez, MD, Department of Ophthalmology, Universidad Pontificia Bolivariana, calle 22 sur 44-50, Envigado, Antioquia 574, Colombia ([email protected]). Conflict of Interest Disclosures: None reported. REFERENCES 1. Shields JA. Primary cysts of the iris. Trans Am Ophthalmol Soc. 1981;79:771-809.
770
Figure 2. The histologic smear showing pigmented epithelial cells (hematoxylin-eosin stain, original magnification ×40).
remarkable growth.6 Ultrasound biomicroscopy is very useful for determining the location and size of small uveal tumors. Primary cysts that originate from the stroma tend to occur in children and are characterized by reaching considerable sizes over time, far exceeding the sizes of the free cysts of the iris epithelium.7 After proper identification, the management of this type of cyst can be expectant.1 Rupture of the cyst wall with a yttrium aluminum garnet laser could be attempted; however, owing to the lack of evidence of corneal endothelium cell loss over time and possible visual symptoms, total removal of these lesions and pathological analysis is an effective, easy, and safe approach. The probability of the parasite cysticercus in the anterior chamber, although low, should also be considered in such cases. The main feature that suggests the presence of cysticercus in the anterior chamber is a large translucent cyst that is occasionally associated with increased intraocular pressure due to pupillary block.8 In this case, surgical removal of the cyst and treatment with albendazole and steroids for extraocular cysts would be indicated. In the literature, the origin of secondary cysts has been attributed to surgical and nonsurgical trauma and to the use of topical medications. To date, penetrating keratoplasty, cataract surgery, and radial keratotomy1,9,10 are among the procedures that have been related to the appearance of cysts secondary to surgical trauma. Although the origin is not clear, miotic agents, such as pilocarpine and prostaglandin analogues, specifically latanoprost, have also been related to the presentation of secondary cysts.11
2. Lois N, Shields CL, Shields JA, Mercado G. Primary cysts of the iris pigment epithelium. Ophthalmology. 1998;105(10):1879-1885.
7. Xiao Y, Wang Y-H, Niu G-L, Gao M. Primary iris stromal cyst with rapid growth. Optom Vis Sci. 2009;86(11):1309-1312.
3. Shields JA, Kline MW, Augsburger JJ. Primary iris cysts: a review of the literature and report of 62 cases. Br J Ophthalmol. 1984;68(3):152-166.
8. Chandra A, Singh MK, Singh VP, Rai AK, Chakraborty S, Maurya OPS. A live cysticercosis in anterior chamber leading to glaucoma secondary to pupilary block. J Glaucoma. 2007;16(2):271-273.
4. Coats G. Pigmented cyst lying free in the anterior chamber. Trans Ophthalmol Soc U K. 1912; 32:189-194. 5. Zhou M, Xu G, Bojanowski CM, et al. Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy. Acta Ophthalmol Scand. 2006;84 (1):137-139. 6. Henderson E, Margo CE. Iris melanoma. Arch Pathol Lab Med. 2008;132(2):268-272.
9. Grieser EJ, Dudenhoefer EJ, Waller SG. Long-term follow-up of free-floating epithelial inclusion cyst after radial keratotomy. Cornea. 2007;26(4):512-513. 10. Claoué C, Lewkowicz-Moss S, Easty D. Epithelial cyst in the anterior chamber after penetrating keratoplasty. Br J Ophthalmol. 1988;72(1):36-40. 11. Pruthi S, Kashani S, Ruben S. Bilateral iris cyst secondary to topical latanoprost. Acta Ophthalmol. 2008;86(2):233-234.
JAMA Ophthalmology June 2014 Volume 132, Number 6
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Tufts Univ. Hirsh Health Sciences Library User on 11/24/2015
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