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Sympathetic Ophthalmitis
SYMPATHETIC O P H T H A L M I T I S G. E. BRAUNINGER, M.D.,
AND F. M.
POLACK,
M.D.
Gainesville, Florida
Sympathetic ophthalmitis is generally con sidered to be a relentless ocular inflamma tion that leads to bilateral loss of vision in 60 to 70% of cases.1 In fact, however, dur ing a single course of therapy, the disease often remits, does not recur when therapy is discontinued,2'3 and leaves no permanent visual loss in its wake. Such a case of sympathetic ophthalmitis is reported here. This patient's non-trauma tized eye responded to corticosteroid therapy and there was no permanent loss of vision. No evidence of recurrence was noted over a two-year period of follow-up observation.
Fig. 1 (Brauninger and Polack). Granulomatous inflammation in the choroid. The epithelial cells contain numerous pigment granules (arrow) and a giant cell is present (hematoxylin-eosin, X200).
CASE REPORT
On April 28, 1969, a 37-year-old woman was seen with a blind, painful left eye following a pene trating injury one month earlier. The laceration had involved the cornea, sclera, and ciliary body and had been surgically repaired. Ocular examination of the right eye showed this eye to be normal with best corrected visual acuity of 20/25. The blind left eye was soft to palpation. Exami nation revealed corneal stroma and epithelial edema with folds in Descemet's membrane. The anterior chamber was shallow and revealed marked cells and flare. A dense cataract was noted behind the eccen tric and unreactive pupil. The retina was not visible. The eye was enucleated on April 29, 1969. Pathologic examination disclosed sympathetic ophthalmitis (Fig. 1). On May 23, 1969, the patient complained of pho tophobia and decreased vision in her remaining eye. Best corrected visual acuity was 20/40 and a mod erate number of cells and flare were noted in the anterior chamber. There were no keratic precipi tates and the retina was normal. She was treated with a corticosteroid (Prednisone) 80 mg daily, and the eye improved rapidly. Two weeks later, ocular examination was normal and visual acuity was correctible to 20/25. Cortico steroid therapy was gradually tapered; after six weeks of therapy it was discontinued. The patient experienced no further difficulty during the follow ing 24 months.
PATHOLOGY
From the Department of Ophthalmology, College of Medicine, University of Florida. This study was supported in part by Public Health Service Grant EY-00033 from the National Eye Institute. Reprint request to Frank M. Polack, M.D., De partment of Ophthalmology, College of Medicine, University of Florida, Gainesville, Florida 32601.
Examination of the enucleated eye re vealed a 9 mm laceration, surgically re paired, which had extended through the pe ripheral cornea and adjacent sclera, and in volved the trabecular meshwork and ciliary body. The laceration was filled with fibroblastic tissue which was patchily infiltrated with small round cells. There was early neovascularization of the corneal stroma, with edema and infiltration of lymphocytes and polymorphonuclear leukocytes. In one quad rant the angle was obliterated by the iris root which was displaced forward against the cornea. The trabecular meshwork and iris root contained numerous scattered lympho cytes and plasma cells. Throughout the cili ary body and choroid there was a diffuse in filtrate of lymphocytes and scattering of eosinophils. Throughout the choroid there were nodular accumulations of epithelioid cells which contained fine pigment granules within their cytoplasm. These granulomatous foci of choroidal inflammation did not in volve the adjacent choriocapillaris. The retina was totally detached and folded upon itself; however, the nuclear layers were well de fined and without inflammatory involvement.
967
968
AMERICAN JOURNAL OF OPHTHALMOLOGY
An organizing vitreous hemorrhage was present and the lens was cataractous but there were no inflammatory cells in or on the lens surface. Leukocytes and eosinophils were seen in the, sclera along the ciliary nerves and emissary vessels. DISCUSSION
Sympathetic ophthalmitis is characterized in its early stages by an insidious uveitis, an terior, posterior, or both, with disproportion ate and early decrease in visual acuity in an eye that does not seem to be particularly red or painful.2'4 Cells and flare in the anterior chamber are prerequisites for the clinical di agnosis, while keratic precipitates, swelling in the iris, papillitis or scattered yellowish spots in the fundus may occur early, all eventually leading to panuveitis.2-4 The pro cess in the "exciting" eye usually begins two weeks after injury and 80% of cases occur within three months.1 The "sympathizing eye" becomes involved at the same time or shortly thereafter. The incidence of sympathetic ophthalmitis has declined with modern surgical technique and postoperative care.5 Nevertheless, the disease is a cause of great concern to the cli nician who surgically repairs an injured eye and later finds both globes involved in an in flammatory process that threatens vision. Sympathetic ophthalmitis may spontane ously remit without therapy, disappear under a course of systemic corticosteroids, or follow a complicated course characterized by remissions and exacerbations with eventual irreversible eye damage,2"4 At present, prog nosis is uncertain and not well understood, partly because those cases in which the course of the disease is mild are generally not reported in the literature. Further, the clinician who treats an apparent case of sym pathetic ophthalmitis that responds quickly
NOVEMBER, 1971
and completely is then inclined to doubt his own initial diagnosis, and pathologic confir mation is not usually available in mild cases. Nevertheless, Makley and Liebold5 have in dicated that a favorable visual result can be obtained with systemic corticosteroid ther apy in 75% of cases. The case of sympathetic ophthalmitis re ported here emphasizes that this disease may present as a mild iritis which disappears dur ing a course of corticosteroid therapy and does not recur or result in permanent clinical damage to the eye. Early detection of this disease process, combined with adequate cor ticosteroid therapy can provide a better vis ual result for the patient.3 SUMMARY
A pathologically documented case of sym pathetic ophthalmitis in a 37-year-old woman began as a mild iritis which responded rap idly to systemic corticosteroid therapy and did not recur or result in permanent eye damage. This case is reported in support of the modern clinical impression that many cases of sympathetic ophthalmitis, with early recognition, adequate systemic corticosteroid therapy and careful patient follow-up, do not recur or cause permanent eye damage. ACKNOWLEDGMENT
We thank Ian C. Hood, M.D., for his study of this case, and R. W. Irwin for his photography. REFERENCES
1. DeVoe, A. G.: Sympathetic ophthalmitis. A case treated by prolonged use of corticotropin and adrenal steroids. Tr. Am. Ophth. Soc. 58:75, 1960. 2. Schlaegel, T. T., Jr.: Essentials of Uveitis. Boston, Little, Brown, 1969, p. 123. 3. Makley, T. A., Jr., and Leibold, J. E.: Modern therapy of sympathetic ophthalmia. Arch. Ophth. 64:809, 1960. 4. Duke Elder, S.: System Ophthalmology, vol. 9. St. Louis, Mosby, 1966, p. 558. 5. Allen, J.: Sympathetic ophthalmitis. A disap pearing disease. J.A.M.A. 209:1090, 1969.
AND F. M.
POLACK,
M.D.
Gainesville, Florida
Sympathetic ophthalmitis is generally con sidered to be a relentless ocular inflamma tion that leads to bilateral loss of vision in 60 to 70% of cases.1 In fact, however, dur ing a single course of therapy, the disease often remits, does not recur when therapy is discontinued,2'3 and leaves no permanent visual loss in its wake. Such a case of sympathetic ophthalmitis is reported here. This patient's non-trauma tized eye responded to corticosteroid therapy and there was no permanent loss of vision. No evidence of recurrence was noted over a two-year period of follow-up observation.
Fig. 1 (Brauninger and Polack). Granulomatous inflammation in the choroid. The epithelial cells contain numerous pigment granules (arrow) and a giant cell is present (hematoxylin-eosin, X200).
CASE REPORT
On April 28, 1969, a 37-year-old woman was seen with a blind, painful left eye following a pene trating injury one month earlier. The laceration had involved the cornea, sclera, and ciliary body and had been surgically repaired. Ocular examination of the right eye showed this eye to be normal with best corrected visual acuity of 20/25. The blind left eye was soft to palpation. Exami nation revealed corneal stroma and epithelial edema with folds in Descemet's membrane. The anterior chamber was shallow and revealed marked cells and flare. A dense cataract was noted behind the eccen tric and unreactive pupil. The retina was not visible. The eye was enucleated on April 29, 1969. Pathologic examination disclosed sympathetic ophthalmitis (Fig. 1). On May 23, 1969, the patient complained of pho tophobia and decreased vision in her remaining eye. Best corrected visual acuity was 20/40 and a mod erate number of cells and flare were noted in the anterior chamber. There were no keratic precipi tates and the retina was normal. She was treated with a corticosteroid (Prednisone) 80 mg daily, and the eye improved rapidly. Two weeks later, ocular examination was normal and visual acuity was correctible to 20/25. Cortico steroid therapy was gradually tapered; after six weeks of therapy it was discontinued. The patient experienced no further difficulty during the follow ing 24 months.
PATHOLOGY
From the Department of Ophthalmology, College of Medicine, University of Florida. This study was supported in part by Public Health Service Grant EY-00033 from the National Eye Institute. Reprint request to Frank M. Polack, M.D., De partment of Ophthalmology, College of Medicine, University of Florida, Gainesville, Florida 32601.
Examination of the enucleated eye re vealed a 9 mm laceration, surgically re paired, which had extended through the pe ripheral cornea and adjacent sclera, and in volved the trabecular meshwork and ciliary body. The laceration was filled with fibroblastic tissue which was patchily infiltrated with small round cells. There was early neovascularization of the corneal stroma, with edema and infiltration of lymphocytes and polymorphonuclear leukocytes. In one quad rant the angle was obliterated by the iris root which was displaced forward against the cornea. The trabecular meshwork and iris root contained numerous scattered lympho cytes and plasma cells. Throughout the cili ary body and choroid there was a diffuse in filtrate of lymphocytes and scattering of eosinophils. Throughout the choroid there were nodular accumulations of epithelioid cells which contained fine pigment granules within their cytoplasm. These granulomatous foci of choroidal inflammation did not in volve the adjacent choriocapillaris. The retina was totally detached and folded upon itself; however, the nuclear layers were well de fined and without inflammatory involvement.
967
968
AMERICAN JOURNAL OF OPHTHALMOLOGY
An organizing vitreous hemorrhage was present and the lens was cataractous but there were no inflammatory cells in or on the lens surface. Leukocytes and eosinophils were seen in the, sclera along the ciliary nerves and emissary vessels. DISCUSSION
Sympathetic ophthalmitis is characterized in its early stages by an insidious uveitis, an terior, posterior, or both, with disproportion ate and early decrease in visual acuity in an eye that does not seem to be particularly red or painful.2'4 Cells and flare in the anterior chamber are prerequisites for the clinical di agnosis, while keratic precipitates, swelling in the iris, papillitis or scattered yellowish spots in the fundus may occur early, all eventually leading to panuveitis.2-4 The pro cess in the "exciting" eye usually begins two weeks after injury and 80% of cases occur within three months.1 The "sympathizing eye" becomes involved at the same time or shortly thereafter. The incidence of sympathetic ophthalmitis has declined with modern surgical technique and postoperative care.5 Nevertheless, the disease is a cause of great concern to the cli nician who surgically repairs an injured eye and later finds both globes involved in an in flammatory process that threatens vision. Sympathetic ophthalmitis may spontane ously remit without therapy, disappear under a course of systemic corticosteroids, or follow a complicated course characterized by remissions and exacerbations with eventual irreversible eye damage,2"4 At present, prog nosis is uncertain and not well understood, partly because those cases in which the course of the disease is mild are generally not reported in the literature. Further, the clinician who treats an apparent case of sym pathetic ophthalmitis that responds quickly
NOVEMBER, 1971
and completely is then inclined to doubt his own initial diagnosis, and pathologic confir mation is not usually available in mild cases. Nevertheless, Makley and Liebold5 have in dicated that a favorable visual result can be obtained with systemic corticosteroid ther apy in 75% of cases. The case of sympathetic ophthalmitis re ported here emphasizes that this disease may present as a mild iritis which disappears dur ing a course of corticosteroid therapy and does not recur or result in permanent clinical damage to the eye. Early detection of this disease process, combined with adequate cor ticosteroid therapy can provide a better vis ual result for the patient.3 SUMMARY
A pathologically documented case of sym pathetic ophthalmitis in a 37-year-old woman began as a mild iritis which responded rap idly to systemic corticosteroid therapy and did not recur or result in permanent eye damage. This case is reported in support of the modern clinical impression that many cases of sympathetic ophthalmitis, with early recognition, adequate systemic corticosteroid therapy and careful patient follow-up, do not recur or cause permanent eye damage. ACKNOWLEDGMENT
We thank Ian C. Hood, M.D., for his study of this case, and R. W. Irwin for his photography. REFERENCES
1. DeVoe, A. G.: Sympathetic ophthalmitis. A case treated by prolonged use of corticotropin and adrenal steroids. Tr. Am. Ophth. Soc. 58:75, 1960. 2. Schlaegel, T. T., Jr.: Essentials of Uveitis. Boston, Little, Brown, 1969, p. 123. 3. Makley, T. A., Jr., and Leibold, J. E.: Modern therapy of sympathetic ophthalmia. Arch. Ophth. 64:809, 1960. 4. Duke Elder, S.: System Ophthalmology, vol. 9. St. Louis, Mosby, 1966, p. 558. 5. Allen, J.: Sympathetic ophthalmitis. A disap pearing disease. J.A.M.A. 209:1090, 1969.