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Presumed Sarcoid Choroidopathy Mimicking Birdshot Retinochoroidopathy
Vol. 109, No.3
Letters to the Journal
357
References 1. Culbertson, W. W., and Ostler, H. B.: The floppy eyelid syndrome. Am. J. Ophthalmol. 92:568, 1981. 2. Moore, M. B., Harrington, J., and McCulley, J. P.: Floppy eyelid syndrome. Management including surgery. Ophthalmology 93:184,1986. 3. Gerner, E. W., and Hughes, S. M.: Floppy eyelid with hyperglycinemia. Am. J. Ophthalmol. 98:614, 1984.
Fig. 1 (Eiferman and associates). Spontaneous eversion of right upper eyelid demonstrating severe induration and papillary response. Spontaneous ectropion of the upper eyelid was described by Culbertson and Ostler' in obese men. There have been other cases reported in women" and in adults with hyperglycinemia." We treated a child with this condition. A 12-year-old boy was seen for severe chronic conjunctivitis of the right upper eyelid. He had a massive papillary reaction of the palpebral conjunctiva, a flaccid lateral canthal tendon and indurated tarsus that everted with slight upward traction (Figs. 1 and 2). A small punch biopsy showed chronic inflammation of the eyelid. Treatment consisted of nonincisioned tarsorrhaphy for two months to allow the swelling to resolve. This was followed by horizontal shortening of the upper eyelid combined with lateral canthal tendon reconstruction. The patient had been followed up for six months postoperatively and has remained asymptomatic. Spontaneous ectropion of the upper eyelid should be considered as part of the differential diagnosis of chronic conjunctivitis in children.
Fig. 2 (Eiferman and associates). Partial eversion of right upper eyelid and saddle nose.
Presumed Sarcoid Mimicking Birdshot Retinochoroidopathy
Choroidopathy
Roy D. Brod, M.D. Department of Ophthalmology, Geisinger Medical Center. Inquiries to Roy D. Brad, M.D., Department of Ophthalmology, Geisinger Medical Center, Danville, PA 17822. Birdshot retinochoroidopathy is a specific posterior uveitis syndrome in which the diagnosis is based on the characteristic fundus appearance of flat depigmented choroidal lesions frequently concentrated nasal to the optic nerve."! Although it is thought to be a single nosologic entity, the cause remains unknown. I examined a patient with ocular findings consistent with birdshot retinochoroidopathy and biopsy-proven sarcoidosis. Sarcoidosis should be considered in the medical examination of patients with birdshot retinochoroidopathy. A 44-year-old woman had a four-week history of blurred vision in her left eye. She had bilateral anterior uveitis, vitritis, and many yellow-fundus lesions, all of which were more progressive in the left eye. She also had mild disk edema in the left eye. A systemic evaluation disclosed hilar adenopathy on a chest X-ray and a subsequent transbronchial lymph node biopsy demonstrated noncaseating granulomatous inflammation consistent with sarcoidosis. She was treated with various combinations of oral, periocular, and topical corticosteroids for intermittent exacerbations of ocular inflammation over the following three years. Three years later, the patient was referred to my institution for retinal consultation because of progression of the fundus lesions. Visual acuity was R.E.: 20/20 and L.E.: 20/
358
March, 1990
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 1 (Brod). Fundus photograph of right eye showing optic nerve pallor and hypopigmented choroidallesions concentrated nasal to the optic nerve.
40-2. Mild anterior chamber cells were apparent in the right eye and moderate anterior chamber cells in the left eye. No iris nodules were apparent. The right eye demonstrated mild posterior vitreous cell and the left eye had moderate posterior vitreous cell. Ophthalmoscopy of the right eye showed mild disk pallor, arteriolar narrowing, and multiple, flat, depigmented choroidal lesions concentrated nasal to the optic nerve and extending into the midperipheral fundus (Fig. 1). In the left eye, the patient had moderate disk pallor, arteriolar narrowing, cystoid macular edema, and multiple, depigmented, flat choroidal lesions scattered throughout the posterior and midperipheral fundus (Fig. 2). Although most of the spots were not visible on fluorescein angiography, some demonstrated mild late staining. Color vision testing using the Farnsworth D-15 panel showed both eyes to be normal.
Peripheral visual fields were constricted; the left eye more so than the right. Electroretinography showed minimally decreased amplitude for both scotopic and photopic responses in the right eye and moderately reduced amplitudes for both scotopic and photopic responses in the left eye. HLA-A29 antigen was not apparent. Although there are numerous ocular manifestations of sarcoidosis, multiple depigmented choroidal lesions have rarely been reported.' Likewise, an extensive systemic evaluation for sarcoidosis has been infrequently performed in reported series of birdshot retinochoroidopathy cases." Priem and Oosterhuis" sought a diagnosis of sarcoidosis in 38 of their 102 reported cases of birdshot retinochoroidopathy and identified one case of biopsy-proven sarcoidosis. A genetic predisposition to this disease is suggested by the high association with HLAA29 antigen; however, the specific cause of birdshot retinochoroidopathy is unknown and may represent an ocular manifestation of several different pathogenic mechanisms."
References 1. Ryan, S. J" and Maumenee, A. E.: Birdshot retinochoroidopathy. Am. J. Ophthalmol. 89:31, 1980. 2. Gass, J. D. M.: Vitiliginous chorioretinitis. Arch. Ophthalmol. 99: 1778, 1981. 3. Priem, H. A., and Oosterhuis. J. A.: Birdshot chorioretinopathy. Clinical characteristics and evolution. Br. J. Ophthalmol. 72:646, 1988. 4. Spalton, D. J" and Sanders, M. D.: Fundus changes in histologically confirmed sarcoidosis. Br. J. Ophthalmol. 65:348, 1981.
Shadow- and Reflex-Free Use of Combined Fiberoptic Manipulators and Illuminators in Vitreous Surgery Mark Michels, M.D., and Yossi Sidikaro, M.D. Jules Stein Eye Institute, UCLA School of Medicine.
Inquiries to Yossi Sidikaro, M.D., 435 N. Roxbury Dr., Suite 410, Beverly Hills, CA 90210.
Fig. 2 (Brod). Fundus photograph of left eye demonstrating optic nerve pallor and numerous hypopigmen ted choroidal lesions.
A primary concern of modern vitreoretinal surgery has been adequate visualization of the operative field. "Bright reflexes" occurring at "every optical interface" as described by Parel, Macherner. and Aumayr' prompted them to
Letters to the Journal
357
References 1. Culbertson, W. W., and Ostler, H. B.: The floppy eyelid syndrome. Am. J. Ophthalmol. 92:568, 1981. 2. Moore, M. B., Harrington, J., and McCulley, J. P.: Floppy eyelid syndrome. Management including surgery. Ophthalmology 93:184,1986. 3. Gerner, E. W., and Hughes, S. M.: Floppy eyelid with hyperglycinemia. Am. J. Ophthalmol. 98:614, 1984.
Fig. 1 (Eiferman and associates). Spontaneous eversion of right upper eyelid demonstrating severe induration and papillary response. Spontaneous ectropion of the upper eyelid was described by Culbertson and Ostler' in obese men. There have been other cases reported in women" and in adults with hyperglycinemia." We treated a child with this condition. A 12-year-old boy was seen for severe chronic conjunctivitis of the right upper eyelid. He had a massive papillary reaction of the palpebral conjunctiva, a flaccid lateral canthal tendon and indurated tarsus that everted with slight upward traction (Figs. 1 and 2). A small punch biopsy showed chronic inflammation of the eyelid. Treatment consisted of nonincisioned tarsorrhaphy for two months to allow the swelling to resolve. This was followed by horizontal shortening of the upper eyelid combined with lateral canthal tendon reconstruction. The patient had been followed up for six months postoperatively and has remained asymptomatic. Spontaneous ectropion of the upper eyelid should be considered as part of the differential diagnosis of chronic conjunctivitis in children.
Fig. 2 (Eiferman and associates). Partial eversion of right upper eyelid and saddle nose.
Presumed Sarcoid Mimicking Birdshot Retinochoroidopathy
Choroidopathy
Roy D. Brod, M.D. Department of Ophthalmology, Geisinger Medical Center. Inquiries to Roy D. Brad, M.D., Department of Ophthalmology, Geisinger Medical Center, Danville, PA 17822. Birdshot retinochoroidopathy is a specific posterior uveitis syndrome in which the diagnosis is based on the characteristic fundus appearance of flat depigmented choroidal lesions frequently concentrated nasal to the optic nerve."! Although it is thought to be a single nosologic entity, the cause remains unknown. I examined a patient with ocular findings consistent with birdshot retinochoroidopathy and biopsy-proven sarcoidosis. Sarcoidosis should be considered in the medical examination of patients with birdshot retinochoroidopathy. A 44-year-old woman had a four-week history of blurred vision in her left eye. She had bilateral anterior uveitis, vitritis, and many yellow-fundus lesions, all of which were more progressive in the left eye. She also had mild disk edema in the left eye. A systemic evaluation disclosed hilar adenopathy on a chest X-ray and a subsequent transbronchial lymph node biopsy demonstrated noncaseating granulomatous inflammation consistent with sarcoidosis. She was treated with various combinations of oral, periocular, and topical corticosteroids for intermittent exacerbations of ocular inflammation over the following three years. Three years later, the patient was referred to my institution for retinal consultation because of progression of the fundus lesions. Visual acuity was R.E.: 20/20 and L.E.: 20/
358
March, 1990
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 1 (Brod). Fundus photograph of right eye showing optic nerve pallor and hypopigmented choroidallesions concentrated nasal to the optic nerve.
40-2. Mild anterior chamber cells were apparent in the right eye and moderate anterior chamber cells in the left eye. No iris nodules were apparent. The right eye demonstrated mild posterior vitreous cell and the left eye had moderate posterior vitreous cell. Ophthalmoscopy of the right eye showed mild disk pallor, arteriolar narrowing, and multiple, flat, depigmented choroidal lesions concentrated nasal to the optic nerve and extending into the midperipheral fundus (Fig. 1). In the left eye, the patient had moderate disk pallor, arteriolar narrowing, cystoid macular edema, and multiple, depigmented, flat choroidal lesions scattered throughout the posterior and midperipheral fundus (Fig. 2). Although most of the spots were not visible on fluorescein angiography, some demonstrated mild late staining. Color vision testing using the Farnsworth D-15 panel showed both eyes to be normal.
Peripheral visual fields were constricted; the left eye more so than the right. Electroretinography showed minimally decreased amplitude for both scotopic and photopic responses in the right eye and moderately reduced amplitudes for both scotopic and photopic responses in the left eye. HLA-A29 antigen was not apparent. Although there are numerous ocular manifestations of sarcoidosis, multiple depigmented choroidal lesions have rarely been reported.' Likewise, an extensive systemic evaluation for sarcoidosis has been infrequently performed in reported series of birdshot retinochoroidopathy cases." Priem and Oosterhuis" sought a diagnosis of sarcoidosis in 38 of their 102 reported cases of birdshot retinochoroidopathy and identified one case of biopsy-proven sarcoidosis. A genetic predisposition to this disease is suggested by the high association with HLAA29 antigen; however, the specific cause of birdshot retinochoroidopathy is unknown and may represent an ocular manifestation of several different pathogenic mechanisms."
References 1. Ryan, S. J" and Maumenee, A. E.: Birdshot retinochoroidopathy. Am. J. Ophthalmol. 89:31, 1980. 2. Gass, J. D. M.: Vitiliginous chorioretinitis. Arch. Ophthalmol. 99: 1778, 1981. 3. Priem, H. A., and Oosterhuis. J. A.: Birdshot chorioretinopathy. Clinical characteristics and evolution. Br. J. Ophthalmol. 72:646, 1988. 4. Spalton, D. J" and Sanders, M. D.: Fundus changes in histologically confirmed sarcoidosis. Br. J. Ophthalmol. 65:348, 1981.
Shadow- and Reflex-Free Use of Combined Fiberoptic Manipulators and Illuminators in Vitreous Surgery Mark Michels, M.D., and Yossi Sidikaro, M.D. Jules Stein Eye Institute, UCLA School of Medicine.
Inquiries to Yossi Sidikaro, M.D., 435 N. Roxbury Dr., Suite 410, Beverly Hills, CA 90210.
Fig. 2 (Brod). Fundus photograph of left eye demonstrating optic nerve pallor and numerous hypopigmen ted choroidal lesions.
A primary concern of modern vitreoretinal surgery has been adequate visualization of the operative field. "Bright reflexes" occurring at "every optical interface" as described by Parel, Macherner. and Aumayr' prompted them to