- Email: [email protected]
Bilateral Diffuse Choroiditis and Exudative Retinal Detachments with Evidence of Lyme Disease
Vol. 105, No. 4
Letters to the Journal
419
Bilateral Diffuse Choroiditis and Exudative Retinal Detachments With Evidence of Lyme Disease
at 36 mg/day. This dosage was continued throughout the clinical and laboratory examinations. Results of slit-lamp examination disclosed bilateral inflammatory cells in the anterior Alexander A. Bialasiewicz, M . D . , chamber and vitreous. Exudative retinal deKlaus W. Ruprecht, M . D . , tachments that extended peripherally between Gottfried O. H. N a u m a n n , M . D . , the 4 o'clock and 8 o'clock meridians were and Holger Blenk, M . D . noted in both eyes (Fig. 1). Cystoid maculopUniversity Eye Hospital Erlangen-Ntirnberg (A.A.B., athy in the central fundus regions was also K.W.R., and G.O.H.N.) and the Institute for Microobserved. Results of Octopus and Goldmann biology (H.B.). perimetry showed paracentral scotomas. CorInquiries to Alexander A. Bialasiewicz, M.D., University neal sensitivity was normal and no exophthalEye Hospital, Schwabachanlage 6, 8520 Erlangen, West mos was present. Intraocular pressure was Germany. 12 mm Hg by applanation tonometry. Fluorescein angiography was repeated and disclosed Tick-borne Borrelia infections are worldwide choroidal lesions (Fig. 2). A thickening of the in distribution. Ocular symptoms include hemchoroid was noted on B-scan ultrasonography. orrhagic conjunctivitis, dendritiform keratitis, iritis, iridocyclitis, retinal vasculitis and hemorResults of a complete physical examination rhages, optic disk edema, and retrobulbar neuruled out both neoplasms and sarcoidosis. A ritis.1"5 slight hypacusis (familial by history) and signs of meningitis were found. Results of cerebroA 32-year-old woman was admitted with a 14-day history of bilateral blurred vision, metaspinal fluid analysis showed a lymphocytic plemorphopsia, and ocular pain. These symptoms ocytosis with 91 cells and a protein level of had been preceded by a "flu-like" illness. The 52 mg/dl. referring ophthalmologist had made the diagDifferential blood cell count showed a slight nosis of Vogt-Koyanagi-Harada disease and leukocytosis of 11,900/mm 3 with a lymphocytohad prescribed systemic oral corticosteroids sis of 54%. Serologic markers for sarcoidosis (72 mg/day of prednylidene). At that time, visual acuity was 20/40 in both eyes. The patient observed further blurring, however, and was admitted with a visual acuity of R.E.: 20/100 and L.E.: 20/66. Prednylidene intake was
Fig. 1 (Bialasiewicz and associates). Diffuse choroidal infiltration with exudative retinal detachment, disk edema, and cystoid macular edema in the left eye. Visual acuity is 20/100.
Fig. 2 (Bialasiewicz and associates). Hyperfluorescence at the optic disk in the left eye. Note the disseminated choroidal lesions with the mottled appearance of the retinal pigment epithelium.
420
AMERICAN JOURNAL OF OPHTHALMOLOGY
were negative. This supported the clinical and x-ray findings. Results of hemoglobin electrophoresis were normal, and the HLA pattern was not suggestive for Vogt-Koyanagi-Harada disease. Results of serologic tests for human immunodeficiency virus, herpes simplex virus, rubella, coxsackie, (para)influenza, measles, Epstein-Barr virus, Yersinia, Salmonella, Treponema pallidum, and Q fever were unremarkable. Tumor markers carcinoembryonic antigen, CA 19-9, and CA 125 were also unremarkable. The results of a Lyme-immunofluorescent test were 1:640 (normal, <1:80), and a Lyme-IgMimmunofluorescent test, 1:40 (normal, <1:10); the results of a Lyme-immunofluorescent test in the cerebrospinal fluid were marginal. When the results of the Lyme-immunofluorescent test were obtained, 100 mg of doxycycline twice daily was started orally for seven days. Meningeal symptoms resolved, and visual acuity was rapidly restored. Seven days after therapy, visual acuity was 20/25 in both eyes. Although a slight bilateral disk edema remained, the exudative retinal detachments had almost disappeared. Control titers at the end of therapy were as follows: Lyme-immunofluorescent test, 1:320; Lyme-IgM-immunofluorescent test, <1:20; and Lyme-immunofluorescent test in cerebrospinal fluid, <1:10. The number of cells in the cerebrospinal fluid was 15/dl. This study indicates a strong correlation between choroidal lesions, optic disk edema, bilateral central exudative retinal detachments, and lymphocytic pleocytosis in the cerebrospinal fluid, and positive Lyme serologic findings. The clinical course of the disease (worsening of symptoms with corticosteroids, rapid resolutions with doxycycline) and the decreasing serum Lyme-IgM-immunofluorescent test (as well as the Lyme-IgG-immunofluorescent test) after successful therapy strongly suggest an infection with Borrelia species. In selected diffuse choroidal lesions, we propose that a thorough laboratory examination should be performed, including tests for Lyme disease.
References 1. Steere, A. C , Duray, P. H., Kauffmann, D. J. H., and Wormser, G. P.: Unilateral blindness caused bv infection with the Lyme disease spirochete. Ann. Intern. Med. 103:382, 1985.
April, 1988
2. Schechter, S. L.: Lyme disease associated with optic neuropathy. Am. J. Med. 81:143, 1986. 3. Wu, G., Lincoff, A., Ellsworth, R. ML, and Haik, B. G.: Optic disk edema and Lyme disease. Ann. Ophthalmol. 18:252, 1986. 4. Schmidt, R., and Ackermann, R.: Durch Zecken iibertragene Meningo-Polyneuritis. Fortschr. Neurol. Psychiatr. 53:145, 1985. 5. Salih, S. Y., and Rich, L. F.: Relapsing fever. In Fraunfelder, F. T., Roy, F. H., and Meyer, S. M. (eds.): Current Ocular Therapy, ed. 2, Philadelphia, W. B. Saunders Co., 1985, pp. 24-25.
Venous Stasis Retinopathy and Gaisbock's Syndrome Laurence R. A v i n s , M . D . , and Thomas K. Krummenacher, M . D . Department of Ophthalmology, St. Louis University. Inquiries to Laurence R. Avins, M.D., 816 University Club Tower, 1034 S. Brentwood Blvd., St. Louis, MO 63117. In many previous studies central retinal vein occlusion has been related to generalized vascular disease. 1 Venous occlusive disease in the eye has also been reported as a complication of hyperviscosity (primary and secondary polycythemia, myeloma, and acute dehydration), coagulation disorders, systemic vasculitis (systemic lupus erythematosus and scleroderma), and abnormalities of the optic disk (drusen and sarcoid). We recently studied a case of nonischemic central retinal vein occlusion associated with Gaisbock's syndrome. A 56-year-old man in apparent good health reported a one-month history of decreased vision in the left eye. Best corrected visual acuity was R.E.: 20/20 and L.E.: 20/200 + . There was sludging of blood in the conjunctival capillaries. A subtle left afferent pupillary defect was noted. Intraocular pressure was 18 mm Hg in both eyes. Slit-lamp biomicroscopy demonstrated a few vitreous cells in the left eye, and both disks were hyperemic and congested. In the right eye there was moderate retinal venous dilation and tortuosity, with scattered small inner retinal hemorrhages in all four quadrants. The macula was not edematous on clinical examination. Retinal venous congestion and hemorrhage in the left eye were more marked, with peripapillary cotton-wool spots and advanced cystoid macular edema. There
Letters to the Journal
419
Bilateral Diffuse Choroiditis and Exudative Retinal Detachments With Evidence of Lyme Disease
at 36 mg/day. This dosage was continued throughout the clinical and laboratory examinations. Results of slit-lamp examination disclosed bilateral inflammatory cells in the anterior Alexander A. Bialasiewicz, M . D . , chamber and vitreous. Exudative retinal deKlaus W. Ruprecht, M . D . , tachments that extended peripherally between Gottfried O. H. N a u m a n n , M . D . , the 4 o'clock and 8 o'clock meridians were and Holger Blenk, M . D . noted in both eyes (Fig. 1). Cystoid maculopUniversity Eye Hospital Erlangen-Ntirnberg (A.A.B., athy in the central fundus regions was also K.W.R., and G.O.H.N.) and the Institute for Microobserved. Results of Octopus and Goldmann biology (H.B.). perimetry showed paracentral scotomas. CorInquiries to Alexander A. Bialasiewicz, M.D., University neal sensitivity was normal and no exophthalEye Hospital, Schwabachanlage 6, 8520 Erlangen, West mos was present. Intraocular pressure was Germany. 12 mm Hg by applanation tonometry. Fluorescein angiography was repeated and disclosed Tick-borne Borrelia infections are worldwide choroidal lesions (Fig. 2). A thickening of the in distribution. Ocular symptoms include hemchoroid was noted on B-scan ultrasonography. orrhagic conjunctivitis, dendritiform keratitis, iritis, iridocyclitis, retinal vasculitis and hemorResults of a complete physical examination rhages, optic disk edema, and retrobulbar neuruled out both neoplasms and sarcoidosis. A ritis.1"5 slight hypacusis (familial by history) and signs of meningitis were found. Results of cerebroA 32-year-old woman was admitted with a 14-day history of bilateral blurred vision, metaspinal fluid analysis showed a lymphocytic plemorphopsia, and ocular pain. These symptoms ocytosis with 91 cells and a protein level of had been preceded by a "flu-like" illness. The 52 mg/dl. referring ophthalmologist had made the diagDifferential blood cell count showed a slight nosis of Vogt-Koyanagi-Harada disease and leukocytosis of 11,900/mm 3 with a lymphocytohad prescribed systemic oral corticosteroids sis of 54%. Serologic markers for sarcoidosis (72 mg/day of prednylidene). At that time, visual acuity was 20/40 in both eyes. The patient observed further blurring, however, and was admitted with a visual acuity of R.E.: 20/100 and L.E.: 20/66. Prednylidene intake was
Fig. 1 (Bialasiewicz and associates). Diffuse choroidal infiltration with exudative retinal detachment, disk edema, and cystoid macular edema in the left eye. Visual acuity is 20/100.
Fig. 2 (Bialasiewicz and associates). Hyperfluorescence at the optic disk in the left eye. Note the disseminated choroidal lesions with the mottled appearance of the retinal pigment epithelium.
420
AMERICAN JOURNAL OF OPHTHALMOLOGY
were negative. This supported the clinical and x-ray findings. Results of hemoglobin electrophoresis were normal, and the HLA pattern was not suggestive for Vogt-Koyanagi-Harada disease. Results of serologic tests for human immunodeficiency virus, herpes simplex virus, rubella, coxsackie, (para)influenza, measles, Epstein-Barr virus, Yersinia, Salmonella, Treponema pallidum, and Q fever were unremarkable. Tumor markers carcinoembryonic antigen, CA 19-9, and CA 125 were also unremarkable. The results of a Lyme-immunofluorescent test were 1:640 (normal, <1:80), and a Lyme-IgMimmunofluorescent test, 1:40 (normal, <1:10); the results of a Lyme-immunofluorescent test in the cerebrospinal fluid were marginal. When the results of the Lyme-immunofluorescent test were obtained, 100 mg of doxycycline twice daily was started orally for seven days. Meningeal symptoms resolved, and visual acuity was rapidly restored. Seven days after therapy, visual acuity was 20/25 in both eyes. Although a slight bilateral disk edema remained, the exudative retinal detachments had almost disappeared. Control titers at the end of therapy were as follows: Lyme-immunofluorescent test, 1:320; Lyme-IgM-immunofluorescent test, <1:20; and Lyme-immunofluorescent test in cerebrospinal fluid, <1:10. The number of cells in the cerebrospinal fluid was 15/dl. This study indicates a strong correlation between choroidal lesions, optic disk edema, bilateral central exudative retinal detachments, and lymphocytic pleocytosis in the cerebrospinal fluid, and positive Lyme serologic findings. The clinical course of the disease (worsening of symptoms with corticosteroids, rapid resolutions with doxycycline) and the decreasing serum Lyme-IgM-immunofluorescent test (as well as the Lyme-IgG-immunofluorescent test) after successful therapy strongly suggest an infection with Borrelia species. In selected diffuse choroidal lesions, we propose that a thorough laboratory examination should be performed, including tests for Lyme disease.
References 1. Steere, A. C , Duray, P. H., Kauffmann, D. J. H., and Wormser, G. P.: Unilateral blindness caused bv infection with the Lyme disease spirochete. Ann. Intern. Med. 103:382, 1985.
April, 1988
2. Schechter, S. L.: Lyme disease associated with optic neuropathy. Am. J. Med. 81:143, 1986. 3. Wu, G., Lincoff, A., Ellsworth, R. ML, and Haik, B. G.: Optic disk edema and Lyme disease. Ann. Ophthalmol. 18:252, 1986. 4. Schmidt, R., and Ackermann, R.: Durch Zecken iibertragene Meningo-Polyneuritis. Fortschr. Neurol. Psychiatr. 53:145, 1985. 5. Salih, S. Y., and Rich, L. F.: Relapsing fever. In Fraunfelder, F. T., Roy, F. H., and Meyer, S. M. (eds.): Current Ocular Therapy, ed. 2, Philadelphia, W. B. Saunders Co., 1985, pp. 24-25.
Venous Stasis Retinopathy and Gaisbock's Syndrome Laurence R. A v i n s , M . D . , and Thomas K. Krummenacher, M . D . Department of Ophthalmology, St. Louis University. Inquiries to Laurence R. Avins, M.D., 816 University Club Tower, 1034 S. Brentwood Blvd., St. Louis, MO 63117. In many previous studies central retinal vein occlusion has been related to generalized vascular disease. 1 Venous occlusive disease in the eye has also been reported as a complication of hyperviscosity (primary and secondary polycythemia, myeloma, and acute dehydration), coagulation disorders, systemic vasculitis (systemic lupus erythematosus and scleroderma), and abnormalities of the optic disk (drusen and sarcoid). We recently studied a case of nonischemic central retinal vein occlusion associated with Gaisbock's syndrome. A 56-year-old man in apparent good health reported a one-month history of decreased vision in the left eye. Best corrected visual acuity was R.E.: 20/20 and L.E.: 20/200 + . There was sludging of blood in the conjunctival capillaries. A subtle left afferent pupillary defect was noted. Intraocular pressure was 18 mm Hg in both eyes. Slit-lamp biomicroscopy demonstrated a few vitreous cells in the left eye, and both disks were hyperemic and congested. In the right eye there was moderate retinal venous dilation and tortuosity, with scattered small inner retinal hemorrhages in all four quadrants. The macula was not edematous on clinical examination. Retinal venous congestion and hemorrhage in the left eye were more marked, with peripapillary cotton-wool spots and advanced cystoid macular edema. There