CASE REPORTS AND SMALL CASE SERIES ophthalmic medications. The pa- netic hemochromatosis, intestinal Corneal Findings tient received multiple phlebotomy iron absorption is significantly in- in Hemochromatosis treatments in 1997. creased, and causes deposition of At the time of our initial oph- iron in the liver, skin, pancreas, Corneal pigmentation deposition thalmic examination in March 1996, joints, and heart. The organs in- has been reported in many sys- the best-corrected visual acuity was volved become impaired with con- temic diseases. These include the 20/40 OD and 20/40 OS. The pa- tinued accumulation. lysosomal diseases, Wilson disease, tient’s eyelids were not abnormal in Acquired hemochromatosis oc- amyloidosis, multiple myeloma, color; the lenses had a mild degree curs from hemolysis (usually from cystinosis, and hemochromatosis. of nuclear sclerosis. The conjunc- thalassemia or sideroblastic ane- These underlying conditions must tiva and cornea of both eyes showed mia) or multiple transfusions. Un- be considered in the differential small fine brown pigmentary depos- like primary hemochromatosis, iron diagnosis of corneal deposits, as its. The diffuse corneal pigment was deposition occurs first in reticulo- they might be treatable. We focus slightly greater inferiorly than su- endothelial cells. As the process con- on an unusual case of corneal iron periorly, with a clear zone of ap- tinues, the reticuloendothelial sys- deposition in a patient with proximately 1.5 mm between the tem becomes saturated, and acquired hemochromatosis. pigment and the limbus, in the in- parenchymal cell deposition also oc- traepithelial and anterior third of the curs, leading to many of the same Report of a Case. A 70-year-old Rus- stroma (Figure 1 and Figure 2). systemic manifestations as genetic sian woman immigrated to the The conjunctival pigment was ex- hemochromatosis. United States in 1995. She was seen tremely fine, diffuse, and interpal- Ocular hemosiderosis is iron by the ophthalmology department pebral. The extremities revealed no toxicity confined to the eye due to because of gradually worsening vi- abnormal pigmentation. a retained intraocular iron foreign sion since 1990. Her medical his- Laboratory test results since body or persistent intraocular hem- tory was notable for type II diabe- 1995 have revealed serum ferritin orrhage. The iron deposition oc- tes, hypertension, arthritis, anemia, levels between 793 and 1173 µg/L curs in most parts of the eye, but es- gastritis, and liver dysfunction. She (reference range, 10-291 µg/L), pecially involves the posterior had been diagnosed with anemia in with a normal iron-binding capac- segment. Severe visual loss can re- 1988, and received weekly intrave- ity, and abnormal liver function. A sult from vitreal degeneration with nous iron injections between 1988 liver biopsy specimen in 1996 formation of contraction bands and and 1989 after daily injections for 3 revealed iron pigment in paren- retinal degeneration.1 weeks. The dosage of each injec- chymal cells and cirrhotic changes The ophthalmic manifesta- tion and the type of anemia that was consistent with hemochromatosis. tions of genetic or acquired hemo- present could not be ascertained. She chromatosis are quite different denied alcohol abuse, previous ocu- Comment. Hemochromatosis can be from the effects of ocular hemo- lar trauma, or the use of any topical genetic (primary) or acquired. In ge- siderosis. In genetic or acquired Figure 1. Low-power view revealing diffuse pattern of pigment in this quiet un- Figure 2. High-power view demonstrating fine brown corneal pigment seen inflamed eye (cilia and eyelid pigment is artifact ) (original magnification 37.5). on direct and indirect retroillumination (original magnification 330). ARCH OPHTHALMOL / VOL 116, NOV 1998 1531 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 disease, the deposition is generally limited to the sclera and ciliary Retained Nuclear Fragment body.1,2 Hudson3 analyzed 5 post- in the Anterior Segment mortem cases of biopsy-proven hemochromatosis and observed Several studies have examined the no abnormalities other than con- management of retained nuclear frag- junctival aneurysms and fundus ments in the posterior segment of the changes similar to early diabetic eye.1-2 Bohigian and Wexler3 re- retinopathy (2 of the 5 patients cently treated 2 patients with nuclear had diabetes). In fact, Hudson fragments in the anterior chamber of stated that “no other ocular find- their eyes after phacoemulsifica- Figure 1. Slitlamp view showing a ings characteristic of hemochro- tion. We also treated a patient with wedge-shaped area of corneal edema localized matosis have been observed.”3 a retained nuclear fragment in the an- to the inferior cornea. Our patient is, to our knowl- terior segment of the eye. edge, the first reported case of cor- neal iron deposition in acquired Report of a Case. An 86-year-old hemochromatosis. Only 1 previous woman underwent a phacoemul- report in the literature has de- sification cataract extraction with pos- scribed a similar clinical picture. terior chamber intraocular lens im- Urrets-Zavalia and Katz4 noted cor- plantation in the right eye. Her visual neal iron deposition and termed it acuity improved to 20/25 OD 1 month “corneal hemochromatosis,” postoperatively. A persistent ante- because there was no systemic iron rior chamber reaction was noted 6 overload. They described a “multi- weeks postoperatively following ta- tude of minute refringent dots in pering of 1% prednisolone acetate. Figure 2. Gonioscopic view of inferior angle the anterior third of the corneal She had a sudden decrease in vision demonstrates a retained lens fragment. stroma,” an appearance similar to 10 weeks later. Visual acuity had di- our patient. Both eyes required minished to 20/200 OD. Slitlamp ex- ferior corneal edema. A similar pat- penetrating keratoplasty due to amination showed a wedge-shaped tern of migration of intraocular corneal opacity. Davies et al2 area of corneal edema (Figure 1). foreign bodies into the anterior cham- reported conjunctival pigmenta- Trace cell and flare were present in the ber angle has been reported previ- tion encroaching onto the limbus anterior chamber. Gonioscopy re- ously.4 Like intraocular foreign bod- in their series of patients with vealed a small lens fragment in the in- ies in the anterior segment, nuclear hemochromatosis. No treatment ferior angle (Figure 2). fragments can incite a prolonged in- was advised for our patient, as her Hourly, 1% prednisolone ac- flammatory reaction or mechani- visual acuity was good. Further- etate was administered. There was no cally damage the endothelium, lead- more, as it may require 2 to 3 years improvement after 1 week, and she ing to localized corneal edema.4 for phlebotomy to reduce substan- underwent removal of the lens frag- There are similarities between tially the total body iron stores, the ment through a temporal incision. our patient and the 2 patients previ- intracorneal iron theoretically may We used 2% pilocarpine hydrochlo- ously described by Bohigian and also clear with the passage of time. ride preoperatively for pupillary con- Wexler.3 Persistent corneal edema striction, and intraoperative gonios- and mild inflammation character- Douglas R. Lazzaro, MD copy was performed to localize the ized all eyes with retained nuclear Ken Lin, MD fragment. Histopathologic examina- fragments in the anterior segment. As Jonathan A. Stevens, OD tion revealed eosinophilic tissue ar- with the prior cases, topical cortico- Brooklyn, NY ranged in lamellae consistent with steroid therapy was ineffective in our lens nucleus. The most recent fol- patient and surgical removal of the low-up examination, 2 months after nuclear fragment was required to im- removal of the fragment, showed im- prove the corneal edema. Because re- Reprints: Douglas R. Lazzaro, MD, provement of the corneal edema, and tained lens fragments can migrate 7901 Fourth Ave, Brooklyn, NY the patient’s visual acuity had im- when changes in posture occur, we 11209. proved to 20/30 OD. recommend preoperative treatment 1. Roth AM, Foos RY. Ocular pathologic changes with pilocarpine to constrict the pu- in primary hemochromatosis. Arch Ophthal- Comment. We hypothesize that the pil and decrease the likelihood of mi- mol. 1972;87:507-514. lens fragment in our patient re- 2. Davies G, Dymock I, Harry J, Williams R. Depo- gration behind the iris. Intraopera- sition of melanin and iron in ocular structures mained sequestered behind the iris tive gonioscopy before removal is also in hemochromatosis. Br J Ophthalmol. 1972;56: for several months after cataract sur- crucial for accurate localization. 338-342. 3. Hudson JR. Ocular findings in haemochroma- gery and produced a chronic, low- tosis. Br J Ophthalmol. 1953;37:242-246. grade inflammation. It later mi- Steven J. Gedde, MD 4. Urrets-Zavalia A Jr, Katz C. Corneal hemochro- grated into the anterior chamber, Carol L. Karp, MD matosis: a unique type of corneal dystrophy in- volving the anterior stroma and both limiting gravitating to the inferior angle and Donald L. Budenz, MD membranes. Am J Ophthalmol. 1971;72:88-96. resulting in the acute onset of in- Miami, Fla ARCH OPHTHALMOL / VOL 116, NOV 1998 1532 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Reprints: Steven J. Gedde, MD, Bas- Though the patient experi- phonuclear cells (2+). The aqueous com Palmer Eye Institute, 900 NW enced persistent intense pain and specimen contained polymorpho- 17th St, Miami, FL 33136 (e-mail: redness of the left eye that began the nuclear cells (1+) but no organ- [email protected]). night of the surgery, he did not re- isms. Microbiological cultures sub- turn for follow-up examination un- sequently grew C indologen from 1. Blodi BA, Flynn HW, Blodi CF, Folk JC, Daily MJ. Retained nuclei after cataract surgery. Oph- til 7 days later, when he was re- both specimens. thalmology.