- Email: [email protected]
Atypical case of ocular hemosiderosis: Leopard cataract
CASE REPORT
Atypical case of ocular hemosiderosis: Leopard cataract Samuel Masket, MD, Basak Bostanci Ceran, MD
We present an interventional case report of an 83-year-old woman who developed ocular hemosiderosis secondary to massive retinal and intravitreal bleeding associated with a choroidal neovascular membrane as a result of age-related macular degeneration. Anterior segment manifestations included low-grade inflammation, posterior synechiae, reversible hyperchromic heterochromia, and a mature cataract with “leopard spots.” The longstanding vitreous hemorrhage was thought to be the etiology of these findings. At the request of the vitreoretinal surgeon, cataract surgery was performed to provide visualization of the posterior segment. However, the patient’s visual potential was limited by her underlying retinal pathology. Financial Disclosure: Neither author has a financial or proprietary interest in any material or method mentioned. Additional disclosure is found in the footnotes. J Cataract Refract Surg 2011; 37:1902–1904 Q 2011 ASCRS and ESCRS Online Video
Hemosiderosis bulbi, or ocular hemosiderosis, is induced by iron toxicity due to the accumulation of iron from the breakdown of hemoglobin after extensive intraocular hemorrhage.1 We report a case of atypical ocular hemosiderosis characterized by accumulation of pigmented spots in a leopard-spot pattern under the anterior lens capsule and anterior subcapsular lens cortex. Additionally, the patient had iris heterochromia with the involved right eye darker than the fellow eye (hyperchromia). CASE REPORT An 83-year-old woman was referred for management of a mature cataract involving the right eye. The medical history was significant for breast cancer and hypercholesterolemia. Ten years earlier, she was treated by one of us (S.M.) for a post-vitrectomy cataract in the fellow left eye following
Submitted: May 18, 2011. Accepted: June 2, 2011. From a private practice (Masket, Ceran), Century City, and the David Geffen School of Medicine (Masket), University of California, Los Angeles, California, USA. Additional financial disclosure: Dr. Masket is a consultant to Alcon Laboratories, Inc. Corresponding author: Samuel Masket, MD, Suite 911, 2080 Century Park East, Los Angeles, California 90067, USA. E-mail: [email protected].
1902
Q 2011 ASCRS and ESCRS Published by Elsevier Inc.
macular hole surgery. Both surgeries were uneventful. Postoperatively, the findings included corrected distance visual acuity (CDVA) of 20/20 in the right eye and 20/25 in the left eye. A modest nuclear cataract was noted in the right eye. Fundus examination revealed nonexudative agerelated macular degeneration bilaterally, and the patient was returned to the vitreoretinal surgeon's care. Under the care of the vitreoretinal surgeon, the patient later developed a subretinal hemorrhage from a choroidal neovascular membrane associated with exudative macular degeneration. According to the standard of care at that time, she was treated with an intravitreal triamcinolone injection and photodynamic therapy. Two years later, the patient was referred again to one of us (S.M.) for surgical management of a mature cataract in the right eye that precluded visualization and inhibited therapy of a suspected retinal detachment. At that time, anterior segment examination of the right eye revealed a clear cornea, 1C cell and flare anterior chamber reaction, and a mature cataract with brown pigmentation involving the anterior lens in a leopard-spot pattern, and posterior synechiae (Figure 1). In addition, the iris in the right eye appeared to be darker than that in the left eye. This was not noted during her earlier care for cataract surgery in the left eye. B-scan ultrasonography was performed due to the limited view of the retina and showed a chronic inferotemporal choroidal and retinal detachment with dense vitreous hemorrhage and debris. Cataract extraction was performed uneventfully, aided by trypan blue capsule staining, using phaco-chop nuclear emulsification. In performing the capsulorhexis, it became evident that the pigmented spots were subcapsular on the anterior lens cortex, as noted in Figure 2 and the Video (available at http://jcrsjournal.org). Four months postoperatively, the CDVA in the right eye improved to counting fingers at 2 feet with eccentric fixation. 0886-3350/$ - see front matter doi:10.1016/j.jcrs.2011.07.024
CASE REPORT: LEOPARD CATARACT
1903
Figure 1. Intraoperative appearance of the involved eye. A mature cataract with brown pigmentation in a leopard-spot pattern and posterior synechiae can be seen. A paracentesis is generated.
Figure 2. During capsulorhexis, it is evident that the pigmented spots are subcapsular.
The patient was lost to follow-up for 4 years. By this time, all intraocular bleeding had resolved, the hemorrhage had absorbed, and iris heterochromia was not noted.
incorporated in phagolysosomes, with eventual cellular degeneration.3 Prussian-blue tissue staining confirms that the iron pigment is located beneath the anterior capsule in the subcapsular lens epithelial cells.2 This is in keeping with the clinical pattern noted in our surgical case. Acquired hyperchromic heterochromia of the iris can result from the stromal deposition of iron pigment, and there have been reports of reversible heterochromia in siderosis after foreign-body removal.4,5 Similarly, the current case demonstrated reversal of heterochromia after resolution of the extensive posterior segment hemorrhage. In summary, we present a unique case of ocular hemosiderosis induced by massive posterior segment hemorrhage. The patient demonstrated a leopardpattern mature cataract, chronic low-grade inflammation, and hyperchromic heterochromia. Cataract surgery was uneventful, and the iris returned to normal coloration after resolution of the posterior segment hemorrhage.
DISCUSSION Extensive and long-term intraocular hemorrhage can allow breakdown of red blood cells with oxidation of hemoglobin into hemosiderin.2 The hemosiderin becomes engulfed in macrophages that accumulate in various parts of the eye. This pattern is quite similar to ocular siderosis that occurs as a result of a ferrous intraocular foreign body. The accumulation of hemosiderin in macrophages results in the constellation of findings that are consistent with ocular hemosiderosis. As noted in the current case report, the patient displayed certain characteristics of that condition. The involved eye demonstrated low-grade anterior chamber inflammation, iris darkening with hyperchromic heterochromia, and spotty, leopard-like pigmentation of the anterior lens. Although these findings may be associated with ocular siderosis, there was no history of or evidence for an iron-containing foreign body. The cataract of siderosis is unique and begins as small brown dots over the anterior surface beneath the lens capsule. The iron appears to bind to enzymes within lens epithelial cells, becoming insoluble and
REFERENCES 1. He X, Hahn P, Iacovelli J, Wong R, King C, Bhisitkul R, MassaroGiordano M, Dunaief JL. Iron homeostasis and toxicity in retinal degeneration. Prog Retin Eye Res 2007; 26:649–673. Available
J CATARACT REFRACT SURG - VOL 37, OCTOBER 2011
1904
CASE REPORT: LEOPARD CATARACT
at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2093950/pdf/ nihms34094.pdf. Accessed June 9, 2011 2. Yanoff M, Sassani JW. Ocular Pathology, 6th ed. St. Louis, MO, Mosby Elsevier, 2009; 24–25, 148–149 3. Hogan MJ, Zimmerman LE. Ophthalmic Pathology: an Atlas and Textbook. Philadelphia, PA, Saunders, 1962; 159–162 4. Welch RB. Two remarkable events in the field of intraocular foreign body: (1) the reversal of siderosis bulbi. (2) the spontaneous extrusion of an intraocular copper foreign body. Trans Am Ophthalmol Soc 1975; 73:187–199; discussion 199–203. Available at: http://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1311452/pdf/taos00026-0207.pdf. Accessed June 9, 2011
5. Pollack A, Oliver M. Reversal of siderosis. Arch Ophthalmol 1998; 116:678–679. Available at: http://archopht.ama-assn.org/cgi/ reprint/116/5/678. Accessed June 9, 2011
J CATARACT REFRACT SURG - VOL 37, OCTOBER 2011
First author: Samuel Masket, MD David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, USA
Atypical case of ocular hemosiderosis: Leopard cataract Samuel Masket, MD, Basak Bostanci Ceran, MD
We present an interventional case report of an 83-year-old woman who developed ocular hemosiderosis secondary to massive retinal and intravitreal bleeding associated with a choroidal neovascular membrane as a result of age-related macular degeneration. Anterior segment manifestations included low-grade inflammation, posterior synechiae, reversible hyperchromic heterochromia, and a mature cataract with “leopard spots.” The longstanding vitreous hemorrhage was thought to be the etiology of these findings. At the request of the vitreoretinal surgeon, cataract surgery was performed to provide visualization of the posterior segment. However, the patient’s visual potential was limited by her underlying retinal pathology. Financial Disclosure: Neither author has a financial or proprietary interest in any material or method mentioned. Additional disclosure is found in the footnotes. J Cataract Refract Surg 2011; 37:1902–1904 Q 2011 ASCRS and ESCRS Online Video
Hemosiderosis bulbi, or ocular hemosiderosis, is induced by iron toxicity due to the accumulation of iron from the breakdown of hemoglobin after extensive intraocular hemorrhage.1 We report a case of atypical ocular hemosiderosis characterized by accumulation of pigmented spots in a leopard-spot pattern under the anterior lens capsule and anterior subcapsular lens cortex. Additionally, the patient had iris heterochromia with the involved right eye darker than the fellow eye (hyperchromia). CASE REPORT An 83-year-old woman was referred for management of a mature cataract involving the right eye. The medical history was significant for breast cancer and hypercholesterolemia. Ten years earlier, she was treated by one of us (S.M.) for a post-vitrectomy cataract in the fellow left eye following
Submitted: May 18, 2011. Accepted: June 2, 2011. From a private practice (Masket, Ceran), Century City, and the David Geffen School of Medicine (Masket), University of California, Los Angeles, California, USA. Additional financial disclosure: Dr. Masket is a consultant to Alcon Laboratories, Inc. Corresponding author: Samuel Masket, MD, Suite 911, 2080 Century Park East, Los Angeles, California 90067, USA. E-mail: [email protected].
1902
Q 2011 ASCRS and ESCRS Published by Elsevier Inc.
macular hole surgery. Both surgeries were uneventful. Postoperatively, the findings included corrected distance visual acuity (CDVA) of 20/20 in the right eye and 20/25 in the left eye. A modest nuclear cataract was noted in the right eye. Fundus examination revealed nonexudative agerelated macular degeneration bilaterally, and the patient was returned to the vitreoretinal surgeon's care. Under the care of the vitreoretinal surgeon, the patient later developed a subretinal hemorrhage from a choroidal neovascular membrane associated with exudative macular degeneration. According to the standard of care at that time, she was treated with an intravitreal triamcinolone injection and photodynamic therapy. Two years later, the patient was referred again to one of us (S.M.) for surgical management of a mature cataract in the right eye that precluded visualization and inhibited therapy of a suspected retinal detachment. At that time, anterior segment examination of the right eye revealed a clear cornea, 1C cell and flare anterior chamber reaction, and a mature cataract with brown pigmentation involving the anterior lens in a leopard-spot pattern, and posterior synechiae (Figure 1). In addition, the iris in the right eye appeared to be darker than that in the left eye. This was not noted during her earlier care for cataract surgery in the left eye. B-scan ultrasonography was performed due to the limited view of the retina and showed a chronic inferotemporal choroidal and retinal detachment with dense vitreous hemorrhage and debris. Cataract extraction was performed uneventfully, aided by trypan blue capsule staining, using phaco-chop nuclear emulsification. In performing the capsulorhexis, it became evident that the pigmented spots were subcapsular on the anterior lens cortex, as noted in Figure 2 and the Video (available at http://jcrsjournal.org). Four months postoperatively, the CDVA in the right eye improved to counting fingers at 2 feet with eccentric fixation. 0886-3350/$ - see front matter doi:10.1016/j.jcrs.2011.07.024
CASE REPORT: LEOPARD CATARACT
1903
Figure 1. Intraoperative appearance of the involved eye. A mature cataract with brown pigmentation in a leopard-spot pattern and posterior synechiae can be seen. A paracentesis is generated.
Figure 2. During capsulorhexis, it is evident that the pigmented spots are subcapsular.
The patient was lost to follow-up for 4 years. By this time, all intraocular bleeding had resolved, the hemorrhage had absorbed, and iris heterochromia was not noted.
incorporated in phagolysosomes, with eventual cellular degeneration.3 Prussian-blue tissue staining confirms that the iron pigment is located beneath the anterior capsule in the subcapsular lens epithelial cells.2 This is in keeping with the clinical pattern noted in our surgical case. Acquired hyperchromic heterochromia of the iris can result from the stromal deposition of iron pigment, and there have been reports of reversible heterochromia in siderosis after foreign-body removal.4,5 Similarly, the current case demonstrated reversal of heterochromia after resolution of the extensive posterior segment hemorrhage. In summary, we present a unique case of ocular hemosiderosis induced by massive posterior segment hemorrhage. The patient demonstrated a leopardpattern mature cataract, chronic low-grade inflammation, and hyperchromic heterochromia. Cataract surgery was uneventful, and the iris returned to normal coloration after resolution of the posterior segment hemorrhage.
DISCUSSION Extensive and long-term intraocular hemorrhage can allow breakdown of red blood cells with oxidation of hemoglobin into hemosiderin.2 The hemosiderin becomes engulfed in macrophages that accumulate in various parts of the eye. This pattern is quite similar to ocular siderosis that occurs as a result of a ferrous intraocular foreign body. The accumulation of hemosiderin in macrophages results in the constellation of findings that are consistent with ocular hemosiderosis. As noted in the current case report, the patient displayed certain characteristics of that condition. The involved eye demonstrated low-grade anterior chamber inflammation, iris darkening with hyperchromic heterochromia, and spotty, leopard-like pigmentation of the anterior lens. Although these findings may be associated with ocular siderosis, there was no history of or evidence for an iron-containing foreign body. The cataract of siderosis is unique and begins as small brown dots over the anterior surface beneath the lens capsule. The iron appears to bind to enzymes within lens epithelial cells, becoming insoluble and
REFERENCES 1. He X, Hahn P, Iacovelli J, Wong R, King C, Bhisitkul R, MassaroGiordano M, Dunaief JL. Iron homeostasis and toxicity in retinal degeneration. Prog Retin Eye Res 2007; 26:649–673. Available
J CATARACT REFRACT SURG - VOL 37, OCTOBER 2011
1904
CASE REPORT: LEOPARD CATARACT
at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2093950/pdf/ nihms34094.pdf. Accessed June 9, 2011 2. Yanoff M, Sassani JW. Ocular Pathology, 6th ed. St. Louis, MO, Mosby Elsevier, 2009; 24–25, 148–149 3. Hogan MJ, Zimmerman LE. Ophthalmic Pathology: an Atlas and Textbook. Philadelphia, PA, Saunders, 1962; 159–162 4. Welch RB. Two remarkable events in the field of intraocular foreign body: (1) the reversal of siderosis bulbi. (2) the spontaneous extrusion of an intraocular copper foreign body. Trans Am Ophthalmol Soc 1975; 73:187–199; discussion 199–203. Available at: http://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1311452/pdf/taos00026-0207.pdf. Accessed June 9, 2011
5. Pollack A, Oliver M. Reversal of siderosis. Arch Ophthalmol 1998; 116:678–679. Available at: http://archopht.ama-assn.org/cgi/ reprint/116/5/678. Accessed June 9, 2011
J CATARACT REFRACT SURG - VOL 37, OCTOBER 2011
First author: Samuel Masket, MD David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, USA