sure to light. Although M gordonae is commonly found in the environment, this is only the second report of corneal involvement, to our knowledge. Topi¬
cal corticosteroids inhibit the cellular response and their use may have con¬ tributed to this patient's infection. In vitro sensitivity testing revealed
susceptibility to amikacin, ciprofloxacin, and rifampin; however, these anti¬
biotics were not effective. The low in vivo response rate may have been due to the slow growth and replication rates of M gordonae or poor penetra¬ tion of the antibiotics through intact
epithelium. Although dual therapy with ciprofloxacin and amikacin has been documented to be synergistic against M fortuitum keratitis in rab¬ bits, our patient did not exhibit a clini¬ cal response.3 Surgery was required to preserve the integrity of the globe.
The diagnosis of atypical mycobacterial keratitis should be considered ear¬ ly in any case of nonresponsive corneal ulcer. Early diagnosis mandates stain¬ ing of smears for acid-fast organisms and culturing on Lowenstein-Jensen medium. However, as with other atyp¬ ical mycobacterial corneal infections, medical therapy alone may be unsuc¬ cessful in eradicating M gordonae keratitis. Nunzio Sossi, MD, PhD Robert M. Feldman, MD Sandy T. Feldman, MD Beatrice E. Frueh, MD Gordon McGuire, PharmD Charles Davis, MD La Jolla, Calif This study was supported in part by Physi¬ cian Scientist Award AG000353 (Dr S. Feldman). to Reprint requests the Department of Ophthal¬ mology 0946, Shiley Eye Center, University of Cal¬ ifornia, San Diego, La Jolla, CA 92093 (Dr S.
Feldman).
1. Newman PE, Goodman RA, Waring GO, et al. A cluster of cases of Mycobacterium chelonae associated with outpatient office procedures. Am J
failure of the parathyroid glands, adrenal cortex, gonad glands, pancreatic beta cells, gastric parietal cells, and thyroid gland. Mucocutaneous candidiasis, viral hepatitis, and dystrophy of dental enamel and nails may result from APECED. Alopecia, vitiligo, and keratopathy may also occur.1 We have followed up a 39-year-old man with APECED who demonstrates optic nerve and retinal changes that we believe are autoimmune. Report of a Case.\p=m-\At age 19 years, the
patient experienced decreased visual acuity
right eye. Recently, he noted progressive loss of vision in his left eye over a 6-month period. His best corrected visual acuity was 20/400 OD and 20/40 OS. Color vision was absent in the right eye and in his
normal in the left eye. An afferent pupillary defect was present in the right eye. Results of slit lamp examination and intraocular pressures were normal in both eyes. Ophthalmoscopic examination revealed a pale optic disc in the right eye. The retinal arterioles were attenuated and the periph¬ ery had bone spicule pigment changes with retinal pigment atrophy (Figure). In the left eye, the disc appeared minimally pale. The vessels and retinal periphery appeared normal. Automated visual field testing re¬ vealed arcuate visual field defects in both eyes consistent with optic nerve disease. Results of serum antibody tests revealed markedly elevated titers against retina and optic nerve. A course of systemic steroids did not result in a decrease in the antibody titers, and results of his ophthalmologic examination remained unchanged.
Comment. —Gass3 reviewed the syn¬ drome of keratoconjunctivitis, superfi¬ cial moniliasis, idiopathic hypoparathyroidism, and Addison's disease. Al¬ though not described in any detail, one patient in the article was noted to have retinitis pigmentosa. Our patient had bilateral optic atrophy. He also showed bone spicule retinal pigmentary changes and narrowing of the arteriole consistent with retinal photoreceptor
Ophthalmol. 1984;97:344.
2. Moore MB, Newton C, Kaufman HE. Chronic keratitis caused by mycobacterial gordonae. Am J Ophthalmol. 1986;102:516. 3. O'Brien TP, Sawusch MR, Dick JD, Gottsch JD. Comparative topical treatment of atypical mycobacterial keratitis in rabbits. Invest Ophthalmol Vis Sci. 1989;30(suppl):363.
spicule pigment changes pigment atrophy. Bone
with retinal
Retinal and Optic Nerve Manifestations of Autoimmune
Polyendocrinopathy-CandidiasisDystrophy
Ectodermal
Autoimmune polyendocrinopathy\x=req-\ candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive disease characterized by a variable
Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 06/02/2015
loss. His family history of inherited retinal or optic nerve abnormalities was negative. We hypothesize that at¬ rophy of the retina and optic nerve in the presence of autoantibodies di¬ rected against these tissues strongly suggests that the ocular findings are autoimmune and part of the syndrome. Larry W. Wood, MD Lee M. Jampol, MD Mark J. Daily, MD Chicago, Ill 1. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical variations of autoimmune polyendo-
crinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med.
1990;322:1829-1836. 2. Thirkill CE, Fitzgerald P, Sergott RC, Roth AM, Tyler NK, Keltner JL. Cancer associated retinopathy (CAR Syndrome) with antibodies reacting with retinal, optic nerve, and cancer cells. N Engl J Med. 1989;321:1589-1594.
3. Gass JDM. The syndrome of keratoconjunctivitis, superficial moniliasis, idiopathic hypoparathyroidism, and Addison's disease. Am J Ophthal-
mol. 1962;54:660-674.
Choroidal Folds and Dural Cavernous Sinus Fistula Dural
sinus fistulas may unresolution with of clinical signs and symptoms. We report such a case and document the first association of choroidal folds with this vascular cavernous
dergo spontaneous transient worsening malformation.
Report of a Case.\p=m-\A 66-year-old woman evaluated for sudden worsening of right eye redness that had been present for 6 weeks. Visual acuity was 20/30 OD and 20/25 OS. Pupils were normal. Examination of the right eye showed 5-mm proptosis, limited supraduction and infraduction, upper and lower eyelid edema, chemosis, and dilated episcleral vessels. Results of left eye was
examination were normal. Intraocular pressures were 36 mm Hg OD and 16 mm Hg OS. Spontaneous venous pulsations were absent on the right, but present on the left. The fundi were otherwise normal. Computed tomography demonstrated a prominent right superior ophthalmic vein and enlargement of the right extraocular muscles. A presumptive diagnosis of dural cavernous sinus fistula was made. Magnetic resonance imaging showed enlargement of the right cavernous sinus and findings con¬ sistent with slowed flow or thrombosis within the right superior ophthalmic vein
(Fig 1). During the next 2 months, the patient experienced marked improvement in her
external appearance yet a decline in her vision in the right eye. Visual acuity was 20/70 OD and 20/25 OS. Pupils were normal and extraocular movements were full. There was 1-mm right-sided proptosis, and the episcleral vessels on the right were only mildly dilated. Choroidal folds and a few scattered intraretinal hemorrhages were
Fig 1. — ,-weighted cranial magnetic resonance images. Left, Axial view demonstrates increased signal in right superior ophthalmic vein (arrow) consistent with slow flow or thrombosis. Right, Coronal scan shows enlargement of right cavernous sinus (arrow).
Fig 2. —Fluorescein angiogram demonstrates acteristic of choroidal folds. present in the right fundus. The left fundus
normal. Fluorescein angiography of the right eye confirmed the presence of choroi¬ dal folds (Fig 2). Within 4 months visual acuity returned to 20/25 OD with a normal fundus. was
of
Comment.— Spontaneous resolution a durai cavernous sinus fistula may
alternating subretinal light and dark lines char¬
follow a period of worsening of signs and symptoms.1 Thrombosis of the su¬ perior ophthalmic vein may precede regression of these lesions. During the period of clinical observation, cerebral arteriography may be deferred. Our patient fits this clinical profile and in addition, developed choroidal folds and
Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 06/02/2015
mild venous stasis retinopathy during her course of clinical improvement. Many conditions have been associ¬ ated with choroidal folds, including hypermetropia, hypotony, orbital tu¬ mors, ocular tumors, thyroid eye dis¬ ease, macular degeneration, uveitis, scleritis, optic disc swelling, and ve¬ nous occlusion.2 Choroidal folds have not been documented with durai cav¬ ernous fistulas, although other re¬ ported disturbances of the ocular circu¬ lation are retinal venous stasis and occlusion, abnormal choroidal blood flow, and choroidal detachments.3 Cho¬ roidal congestion has been suggested as an etiologic factor in the develop¬ ment of both choroidal detachment and choroidal folds.2 In summary, our patient developed choroidal folds during spontaneous res¬ olution of a durai cavernous sinus fistu¬ la. This is likely part of a spectrum of findings related to altered choroidal circulation. Lee G. Gonshor, MD Lanning B. Kline, MD Birmingham, Ala Reprint requests to Children's Hospital Tower, 1600 Seventh Ave S, Suite 555, Birmingham, AL 35233 (Dr Kline). 1. Sergott RC, Grossman RI, Savino PJ, Bosley TM, Schatz NJ. The syndrome of paradoxical wors¬ ening of dural-cavernous sinus arteriovenous mal¬ formations. Ophthalmology. 1987;94:205-212. 2. Cangemi FE, Trempe CL, Walsh JB. Choroi¬ dal folds. Am J Ophthalmol. 1978;86:380-387. 3. Keltner JL, Satterfield D, Dublin AB, Lee
BCP. Durai and carotid
cavernous
sinus fistulas:
diagnosis, management, and complications. Oph¬ thalmology. 1987;94:1585-1600.
cal corticosteroids inhibit the cellular response and their use may have con¬ tributed to this patient's infection. In vitro sensitivity testing revealed
susceptibility to amikacin, ciprofloxacin, and rifampin; however, these anti¬
biotics were not effective. The low in vivo response rate may have been due to the slow growth and replication rates of M gordonae or poor penetra¬ tion of the antibiotics through intact
epithelium. Although dual therapy with ciprofloxacin and amikacin has been documented to be synergistic against M fortuitum keratitis in rab¬ bits, our patient did not exhibit a clini¬ cal response.3 Surgery was required to preserve the integrity of the globe.
The diagnosis of atypical mycobacterial keratitis should be considered ear¬ ly in any case of nonresponsive corneal ulcer. Early diagnosis mandates stain¬ ing of smears for acid-fast organisms and culturing on Lowenstein-Jensen medium. However, as with other atyp¬ ical mycobacterial corneal infections, medical therapy alone may be unsuc¬ cessful in eradicating M gordonae keratitis. Nunzio Sossi, MD, PhD Robert M. Feldman, MD Sandy T. Feldman, MD Beatrice E. Frueh, MD Gordon McGuire, PharmD Charles Davis, MD La Jolla, Calif This study was supported in part by Physi¬ cian Scientist Award AG000353 (Dr S. Feldman). to Reprint requests the Department of Ophthal¬ mology 0946, Shiley Eye Center, University of Cal¬ ifornia, San Diego, La Jolla, CA 92093 (Dr S.
Feldman).
1. Newman PE, Goodman RA, Waring GO, et al. A cluster of cases of Mycobacterium chelonae associated with outpatient office procedures. Am J
failure of the parathyroid glands, adrenal cortex, gonad glands, pancreatic beta cells, gastric parietal cells, and thyroid gland. Mucocutaneous candidiasis, viral hepatitis, and dystrophy of dental enamel and nails may result from APECED. Alopecia, vitiligo, and keratopathy may also occur.1 We have followed up a 39-year-old man with APECED who demonstrates optic nerve and retinal changes that we believe are autoimmune. Report of a Case.\p=m-\At age 19 years, the
patient experienced decreased visual acuity
right eye. Recently, he noted progressive loss of vision in his left eye over a 6-month period. His best corrected visual acuity was 20/400 OD and 20/40 OS. Color vision was absent in the right eye and in his
normal in the left eye. An afferent pupillary defect was present in the right eye. Results of slit lamp examination and intraocular pressures were normal in both eyes. Ophthalmoscopic examination revealed a pale optic disc in the right eye. The retinal arterioles were attenuated and the periph¬ ery had bone spicule pigment changes with retinal pigment atrophy (Figure). In the left eye, the disc appeared minimally pale. The vessels and retinal periphery appeared normal. Automated visual field testing re¬ vealed arcuate visual field defects in both eyes consistent with optic nerve disease. Results of serum antibody tests revealed markedly elevated titers against retina and optic nerve. A course of systemic steroids did not result in a decrease in the antibody titers, and results of his ophthalmologic examination remained unchanged.
Comment. —Gass3 reviewed the syn¬ drome of keratoconjunctivitis, superfi¬ cial moniliasis, idiopathic hypoparathyroidism, and Addison's disease. Al¬ though not described in any detail, one patient in the article was noted to have retinitis pigmentosa. Our patient had bilateral optic atrophy. He also showed bone spicule retinal pigmentary changes and narrowing of the arteriole consistent with retinal photoreceptor
Ophthalmol. 1984;97:344.
2. Moore MB, Newton C, Kaufman HE. Chronic keratitis caused by mycobacterial gordonae. Am J Ophthalmol. 1986;102:516. 3. O'Brien TP, Sawusch MR, Dick JD, Gottsch JD. Comparative topical treatment of atypical mycobacterial keratitis in rabbits. Invest Ophthalmol Vis Sci. 1989;30(suppl):363.
spicule pigment changes pigment atrophy. Bone
with retinal
Retinal and Optic Nerve Manifestations of Autoimmune
Polyendocrinopathy-CandidiasisDystrophy
Ectodermal
Autoimmune polyendocrinopathy\x=req-\ candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive disease characterized by a variable
Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 06/02/2015
loss. His family history of inherited retinal or optic nerve abnormalities was negative. We hypothesize that at¬ rophy of the retina and optic nerve in the presence of autoantibodies di¬ rected against these tissues strongly suggests that the ocular findings are autoimmune and part of the syndrome. Larry W. Wood, MD Lee M. Jampol, MD Mark J. Daily, MD Chicago, Ill 1. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical variations of autoimmune polyendo-
crinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med.
1990;322:1829-1836. 2. Thirkill CE, Fitzgerald P, Sergott RC, Roth AM, Tyler NK, Keltner JL. Cancer associated retinopathy (CAR Syndrome) with antibodies reacting with retinal, optic nerve, and cancer cells. N Engl J Med. 1989;321:1589-1594.
3. Gass JDM. The syndrome of keratoconjunctivitis, superficial moniliasis, idiopathic hypoparathyroidism, and Addison's disease. Am J Ophthal-
mol. 1962;54:660-674.
Choroidal Folds and Dural Cavernous Sinus Fistula Dural
sinus fistulas may unresolution with of clinical signs and symptoms. We report such a case and document the first association of choroidal folds with this vascular cavernous
dergo spontaneous transient worsening malformation.
Report of a Case.\p=m-\A 66-year-old woman evaluated for sudden worsening of right eye redness that had been present for 6 weeks. Visual acuity was 20/30 OD and 20/25 OS. Pupils were normal. Examination of the right eye showed 5-mm proptosis, limited supraduction and infraduction, upper and lower eyelid edema, chemosis, and dilated episcleral vessels. Results of left eye was
examination were normal. Intraocular pressures were 36 mm Hg OD and 16 mm Hg OS. Spontaneous venous pulsations were absent on the right, but present on the left. The fundi were otherwise normal. Computed tomography demonstrated a prominent right superior ophthalmic vein and enlargement of the right extraocular muscles. A presumptive diagnosis of dural cavernous sinus fistula was made. Magnetic resonance imaging showed enlargement of the right cavernous sinus and findings con¬ sistent with slowed flow or thrombosis within the right superior ophthalmic vein
(Fig 1). During the next 2 months, the patient experienced marked improvement in her
external appearance yet a decline in her vision in the right eye. Visual acuity was 20/70 OD and 20/25 OS. Pupils were normal and extraocular movements were full. There was 1-mm right-sided proptosis, and the episcleral vessels on the right were only mildly dilated. Choroidal folds and a few scattered intraretinal hemorrhages were
Fig 1. — ,-weighted cranial magnetic resonance images. Left, Axial view demonstrates increased signal in right superior ophthalmic vein (arrow) consistent with slow flow or thrombosis. Right, Coronal scan shows enlargement of right cavernous sinus (arrow).
Fig 2. —Fluorescein angiogram demonstrates acteristic of choroidal folds. present in the right fundus. The left fundus
normal. Fluorescein angiography of the right eye confirmed the presence of choroi¬ dal folds (Fig 2). Within 4 months visual acuity returned to 20/25 OD with a normal fundus. was
of
Comment.— Spontaneous resolution a durai cavernous sinus fistula may
alternating subretinal light and dark lines char¬
follow a period of worsening of signs and symptoms.1 Thrombosis of the su¬ perior ophthalmic vein may precede regression of these lesions. During the period of clinical observation, cerebral arteriography may be deferred. Our patient fits this clinical profile and in addition, developed choroidal folds and
Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 06/02/2015
mild venous stasis retinopathy during her course of clinical improvement. Many conditions have been associ¬ ated with choroidal folds, including hypermetropia, hypotony, orbital tu¬ mors, ocular tumors, thyroid eye dis¬ ease, macular degeneration, uveitis, scleritis, optic disc swelling, and ve¬ nous occlusion.2 Choroidal folds have not been documented with durai cav¬ ernous fistulas, although other re¬ ported disturbances of the ocular circu¬ lation are retinal venous stasis and occlusion, abnormal choroidal blood flow, and choroidal detachments.3 Cho¬ roidal congestion has been suggested as an etiologic factor in the develop¬ ment of both choroidal detachment and choroidal folds.2 In summary, our patient developed choroidal folds during spontaneous res¬ olution of a durai cavernous sinus fistu¬ la. This is likely part of a spectrum of findings related to altered choroidal circulation. Lee G. Gonshor, MD Lanning B. Kline, MD Birmingham, Ala Reprint requests to Children's Hospital Tower, 1600 Seventh Ave S, Suite 555, Birmingham, AL 35233 (Dr Kline). 1. Sergott RC, Grossman RI, Savino PJ, Bosley TM, Schatz NJ. The syndrome of paradoxical wors¬ ening of dural-cavernous sinus arteriovenous mal¬ formations. Ophthalmology. 1987;94:205-212. 2. Cangemi FE, Trempe CL, Walsh JB. Choroi¬ dal folds. Am J Ophthalmol. 1978;86:380-387. 3. Keltner JL, Satterfield D, Dublin AB, Lee
BCP. Durai and carotid
cavernous
sinus fistulas:
diagnosis, management, and complications. Oph¬ thalmology. 1987;94:1585-1600.
