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Adie syndrome: evidence for refractive error and accommodative asymmetry as the cause of amblyopia
with birdshot retinochoroidopathy may not be reflected in change of Snellen visual acuity and that visual symptoms do not necessarily parallel changes in fundus lesions. Patients with only subjective alteration in vision may benefit from treatment. Visual dysfunction can be identified by electrophysiologic tests,1 but such studies were not performed in our patients. The improvement in automated visual field in Case 1 is consistent with recovery of retinal function after treatment. We have seen only two additional men with this disorder, one of whom had reduced vision from diabetic retinopathy and the other who was lost to follow-up. Only one of nine women we are following up has a bestcorrected visual acuity better than 20/40 in either eye. The effect of sex on the course of disease and response to treatment should be investigated in future studies of this disorder. In contrast to our experience with these two patients, a case series by Bloch-Michel and Frau4 reported that 15 of 20 patients with birdshot retinochoroiditis required prednisone at doses of 10 mg daily or higher for at least 6 months before vision was stabilized. Visual acuity outcomes were not stated for the remaining 25%. The good response observed in our patients could not be attributed to short follow-up periods or young age; their ages were comparable to the median ages (within the fifth or sixth decades) reported in large series.2– 4 Our cases indicate a subset of patients with birdshot retinochoroidopathy may do well over long periods of time with minimal therapy consisting of low-dose oral corticosteroids. Such cases have not been presented in large published series of patients with this disease.
PURPOSE:
To report objective changes in accommodation in a child with Adie syndrome. METHODS: A child aged 2 years 10 months when initially examined was found to have good visual acuity in both eyes, a low degree of hypermetropia (isometropic), and Adie pupil presumed to be caused by chicken pox that had occurred 2 months earlier. Amblyopia developed but responded well to treatment, which involved correction of the refractive error and occlusion therapy. Objective changes in the refraction of the eye were measured on the Canon R1 autorefractor at 3.8 m and 33 cm. RESULT: The degree of accommodation in the affected eye when both eyes were open was markedly reduced. CONCLUSION: The presence of isometropic hypermetropia, which remains uncorrected when Adie syndrome is present, can lead to the development of amblyopia in a child. (Am J Ophthalmol 1999;128: 118 –119. © 1999 by Elsevier Science Inc. All rights reserved.)
A
REFERENCES
1. Le Hoang P, Ryan SJ. Birdshot retinochoroidopathy. In: Pepose JS, Holland GN, Wilhelmus KR, editors. Ocular infection and immunity. St Louis: Mosby-Year Book, 1996: 570 –578. 2. Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthalmol 1980;89:31– 45. 3. Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol 1988;72: 646 – 659. 4. Bloch-Michel E, Frau E. Birdshot retinochoroidopathy and HLA-A29⫹ and HLA-A29-idiopathic retinal vasculitis: comparative study of 56 cases. Can J Opthalmol 1991;26:361–366. 5. Vitale AT, Rodriguez A, Foster CS. Low-dose cyclosporine therapy in the treatment of birdshot retinochoroidopathy. Ophthalmology 1993;101:822– 831.
Adie Syndrome: Evidence for Refractive Error and Accommodative Asymmetry as the Cause of Amblyopia
Accepted for publication Jan 14, 1999. From the Department of Ophthalmology and Orthoptics, University of Sheffield, Sheffield, United Kingdom. Inquiries to Alison Y. Firth, Department of Ophthalmology and Orthoptics, University of Sheffield, Royal Hallamshire Hospital, Glossop Rd, Sheffield S10 2JF, UK; fax: 0114 276 6381 E-mail: a.firth@ sheffield.ac.uk
Alison Y. Firth, MSc, DBO(T) 118
AMERICAN JOURNAL
DIE SYNDROME IS CHARACTERIZED BY A DILATED PU-
pil that is minimally reactive or unresponsive to light and reduced or absent deep tendon reflexes. It is uncommon in children. In March 1994, a child aged 2 years 10 months was initially examined with a right-sided Adie pupil that was presumed to be caused by chicken pox that occurred 2 months earlier. Diagnosis was confirmed by brisk constriction of the affected pupil upon instillation of dilute pilocarpine. Visual acuity with each eye separately was 6/5 (tested with single optotypes). Six months later, the patient’s visual acuity was RE: 20/20 and LE: 20/16.67 (single optotypes), and refraction disclosed ⫹2.00 diopters of hypermetropia in either eye. Refractive correction was not prescribed. Fundi were normal, and the child was asked to return in 6 months. When the patient was examined in March 1995, visual acuity was RE: 20/80 and LE: 20/30 on Snellen chart with key card and RE: 20/30 and LE: 20/20 with single optotypes. No strabismus was present, but right central suppression was present on the 4⌬ prism test. Hypermetropic correction was ordered, and 4 hours of left occlusion therapy per day was prescribed. After 5 months of parttime occlusion therapy, visual acuity had improved to RE: 20/30 with refractive correction and remained LE: 20/30. Refractive correction (regularly updated) continued to be worn full time, and the child was discharged in September 1998 at age 7 years 3 months with a visual acuity of RE: 20/20 ⫹1 and LE: 6/5 ⫹1 with refractive correction and RE: 20/40 ⫹1 and LE: 20/20 without
OF
OPHTHALMOLOGY
JULY 1999
TABLE 1. Objective Refraction (Best Spherical Equivalent) in Diopters Under Monocular Conditions* Distance and Target
Right Eye
Left Eye
33 cm, 20/20 33 cm, 20/200 3.8 m, 20/20 Change in Refraction (D) 3.8 m, 20/20, to 33 cm, 20/20
⫺2.370 ⫺2.372 0.500
⫺2.445 ⫺2.405 0.663
2.870
3.108
REFERENCES
D ⫽ diopters. *Fellow eye occluded.
TABLE 2. Objective Refraction (Best Spherical Equivalent) in Diopters Under Binocular Conditions* Distance and Target
Right Eye
Left Eye
33 cm, 20/20 33 cm, 20/200 3.8 m, 20/20 Change in Refraction (D) 3.8 m, 20/20, to 33 cm, 20/20
⫺0.540 ⫺0.532 1.900
⫺2.995 ⫺2.498 1.373
2.440
4.368
D ⫽ diopters. *Both eyes open.
1. Agbeja AM, Dutton GN. Adie’s syndrome as a cause of amblyopia. J Pediatr Ophthalmol Strab 1987;24:176 –177. 2. Dutton GN, Paul R. Adie syndrome in a child: a case report. J Pediatr Ophthalmol Strab 1992;29:126. 3. Soylev MF, Saatci O, Kavukcu S, Ergin M. Adie’s syndrome in childhood. Acta Paediatrica Japonica 1997;39:395–396. 4. Ukai K, Ishikawa S. Accommodative fluctuations in Adie’s syndrome. Ophthalmol Physiol Opt 1989;9:76 –78.
Spontaneous Regression of Orbital Langerhans Cell Granulomatosis in a Three-Year-Old Girl Jennifer Hasenyager Smith, MD, Lilia Fulton, BA, and Joan M. O’Brien, MD To report a case of spontaneous regression of orbital Langerhans cell granulomatosis. METHOD: Case report. A 3-year-old girl was initially examined with a 5-week history of slowly progressive blepharoptosis and periorbital swelling of the left eye. RESULTS: Computed tomographic scan showed a mass in the left orbit eroding into the left frontal bone; fineneedle aspiration confirmed diagnosis of Langerhans cell granulomatosis. After initial biopsy, the patient was treated by close observation alone. Six months after initial examination, the monostotic lesion had completely resolved. CONCLUSIONS: In some cases of monostotic Langerhans cell granulomatosis, initial biopsy followed by observation alone may allow for the spontaneous regression of the lesion. This conservative approach to treatment is an important therapeutic option that may spare the patient the adverse effects of surgical resection, radiation, or chemotherapy. (Am J Ophthalmol 1999;128: 119 –121. © 1999 by Elsevier Science Inc. All rights reserved.) PURPOSE:
correction. At this time, objective measures of refractive changes were recorded from each eye without refractive correction. Measurements were recorded when both eyes were open and when the fellow eye was occluded for fixation at 33 cm and 3.8 m when fixing 20/20 Snellen equivalent, and at 33 cm when fixing 20/20 Snellen equivalent. Three measurements were taken in each situation and the best spherical equivalent calculated. The means are given in Tables 1 and 2. Monocularly (Table 1), the change in refraction (accommodation) that occurred was similar in either eye, demonstrating that despite Adie syndrome, the necessary amplitude of accommodation can be achieved; however, when both eyes were open, the accommodative change in the right eye was noticeably reduced (Table 2). When fixing at 3.8 m with both eyes open, some degree of anisometropia was present (0.527 diopters). Adie syndrome as a cause of amblyopia has previously been suggested in a case in which anisometropia was present.1–2 In our patient, the first refraction showed no sign of anisometropia, although 0.5 diopters was found at later refraction. Soylev and associates3 reported a case of mild amblyopia in a child diagnosed with Adie syndrome at age 8 years in whom anisocoria went unnoticed but was thought to have been present for 5 years. Ukai and Ishikawa4 have presented results of objective dynamic accommodation patterns in two young adult females with Adie syndrome. Upon monocular testing, each showed an VOL. 128, NO. 1
overshoot in the affected eye when changing fixation from distance to near, which the authors suggest may be caused by delay in the accommodation control system. In our patient, the accommodative level achieved is similar in either eye under monocular conditions. Our patient illustrates how anisometropic amblyopia may develop in Adie syndrome accompanied by apparent isohypermetropia.
Accepted for publication Feb 2, 1999. From the Department of Ophthalmology, University of California, San Francisco, San Francisco, California. Inquiries to Joan M. O’Brien, MD, Department of Ophthalmology, University of California, San Francisco, 10 Kirkham St, Box 0730, San Francisco, CA 94143-0730; fax: (415) 476-0336; e-mail: aleja@itsa. ucsf.edu
BRIEF REPORTS
119
PURPOSE:
To report objective changes in accommodation in a child with Adie syndrome. METHODS: A child aged 2 years 10 months when initially examined was found to have good visual acuity in both eyes, a low degree of hypermetropia (isometropic), and Adie pupil presumed to be caused by chicken pox that had occurred 2 months earlier. Amblyopia developed but responded well to treatment, which involved correction of the refractive error and occlusion therapy. Objective changes in the refraction of the eye were measured on the Canon R1 autorefractor at 3.8 m and 33 cm. RESULT: The degree of accommodation in the affected eye when both eyes were open was markedly reduced. CONCLUSION: The presence of isometropic hypermetropia, which remains uncorrected when Adie syndrome is present, can lead to the development of amblyopia in a child. (Am J Ophthalmol 1999;128: 118 –119. © 1999 by Elsevier Science Inc. All rights reserved.)
A
REFERENCES
1. Le Hoang P, Ryan SJ. Birdshot retinochoroidopathy. In: Pepose JS, Holland GN, Wilhelmus KR, editors. Ocular infection and immunity. St Louis: Mosby-Year Book, 1996: 570 –578. 2. Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthalmol 1980;89:31– 45. 3. Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol 1988;72: 646 – 659. 4. Bloch-Michel E, Frau E. Birdshot retinochoroidopathy and HLA-A29⫹ and HLA-A29-idiopathic retinal vasculitis: comparative study of 56 cases. Can J Opthalmol 1991;26:361–366. 5. Vitale AT, Rodriguez A, Foster CS. Low-dose cyclosporine therapy in the treatment of birdshot retinochoroidopathy. Ophthalmology 1993;101:822– 831.
Adie Syndrome: Evidence for Refractive Error and Accommodative Asymmetry as the Cause of Amblyopia
Accepted for publication Jan 14, 1999. From the Department of Ophthalmology and Orthoptics, University of Sheffield, Sheffield, United Kingdom. Inquiries to Alison Y. Firth, Department of Ophthalmology and Orthoptics, University of Sheffield, Royal Hallamshire Hospital, Glossop Rd, Sheffield S10 2JF, UK; fax: 0114 276 6381 E-mail: a.firth@ sheffield.ac.uk
Alison Y. Firth, MSc, DBO(T) 118
AMERICAN JOURNAL
DIE SYNDROME IS CHARACTERIZED BY A DILATED PU-
pil that is minimally reactive or unresponsive to light and reduced or absent deep tendon reflexes. It is uncommon in children. In March 1994, a child aged 2 years 10 months was initially examined with a right-sided Adie pupil that was presumed to be caused by chicken pox that occurred 2 months earlier. Diagnosis was confirmed by brisk constriction of the affected pupil upon instillation of dilute pilocarpine. Visual acuity with each eye separately was 6/5 (tested with single optotypes). Six months later, the patient’s visual acuity was RE: 20/20 and LE: 20/16.67 (single optotypes), and refraction disclosed ⫹2.00 diopters of hypermetropia in either eye. Refractive correction was not prescribed. Fundi were normal, and the child was asked to return in 6 months. When the patient was examined in March 1995, visual acuity was RE: 20/80 and LE: 20/30 on Snellen chart with key card and RE: 20/30 and LE: 20/20 with single optotypes. No strabismus was present, but right central suppression was present on the 4⌬ prism test. Hypermetropic correction was ordered, and 4 hours of left occlusion therapy per day was prescribed. After 5 months of parttime occlusion therapy, visual acuity had improved to RE: 20/30 with refractive correction and remained LE: 20/30. Refractive correction (regularly updated) continued to be worn full time, and the child was discharged in September 1998 at age 7 years 3 months with a visual acuity of RE: 20/20 ⫹1 and LE: 6/5 ⫹1 with refractive correction and RE: 20/40 ⫹1 and LE: 20/20 without
OF
OPHTHALMOLOGY
JULY 1999
TABLE 1. Objective Refraction (Best Spherical Equivalent) in Diopters Under Monocular Conditions* Distance and Target
Right Eye
Left Eye
33 cm, 20/20 33 cm, 20/200 3.8 m, 20/20 Change in Refraction (D) 3.8 m, 20/20, to 33 cm, 20/20
⫺2.370 ⫺2.372 0.500
⫺2.445 ⫺2.405 0.663
2.870
3.108
REFERENCES
D ⫽ diopters. *Fellow eye occluded.
TABLE 2. Objective Refraction (Best Spherical Equivalent) in Diopters Under Binocular Conditions* Distance and Target
Right Eye
Left Eye
33 cm, 20/20 33 cm, 20/200 3.8 m, 20/20 Change in Refraction (D) 3.8 m, 20/20, to 33 cm, 20/20
⫺0.540 ⫺0.532 1.900
⫺2.995 ⫺2.498 1.373
2.440
4.368
D ⫽ diopters. *Both eyes open.
1. Agbeja AM, Dutton GN. Adie’s syndrome as a cause of amblyopia. J Pediatr Ophthalmol Strab 1987;24:176 –177. 2. Dutton GN, Paul R. Adie syndrome in a child: a case report. J Pediatr Ophthalmol Strab 1992;29:126. 3. Soylev MF, Saatci O, Kavukcu S, Ergin M. Adie’s syndrome in childhood. Acta Paediatrica Japonica 1997;39:395–396. 4. Ukai K, Ishikawa S. Accommodative fluctuations in Adie’s syndrome. Ophthalmol Physiol Opt 1989;9:76 –78.
Spontaneous Regression of Orbital Langerhans Cell Granulomatosis in a Three-Year-Old Girl Jennifer Hasenyager Smith, MD, Lilia Fulton, BA, and Joan M. O’Brien, MD To report a case of spontaneous regression of orbital Langerhans cell granulomatosis. METHOD: Case report. A 3-year-old girl was initially examined with a 5-week history of slowly progressive blepharoptosis and periorbital swelling of the left eye. RESULTS: Computed tomographic scan showed a mass in the left orbit eroding into the left frontal bone; fineneedle aspiration confirmed diagnosis of Langerhans cell granulomatosis. After initial biopsy, the patient was treated by close observation alone. Six months after initial examination, the monostotic lesion had completely resolved. CONCLUSIONS: In some cases of monostotic Langerhans cell granulomatosis, initial biopsy followed by observation alone may allow for the spontaneous regression of the lesion. This conservative approach to treatment is an important therapeutic option that may spare the patient the adverse effects of surgical resection, radiation, or chemotherapy. (Am J Ophthalmol 1999;128: 119 –121. © 1999 by Elsevier Science Inc. All rights reserved.) PURPOSE:
correction. At this time, objective measures of refractive changes were recorded from each eye without refractive correction. Measurements were recorded when both eyes were open and when the fellow eye was occluded for fixation at 33 cm and 3.8 m when fixing 20/20 Snellen equivalent, and at 33 cm when fixing 20/20 Snellen equivalent. Three measurements were taken in each situation and the best spherical equivalent calculated. The means are given in Tables 1 and 2. Monocularly (Table 1), the change in refraction (accommodation) that occurred was similar in either eye, demonstrating that despite Adie syndrome, the necessary amplitude of accommodation can be achieved; however, when both eyes were open, the accommodative change in the right eye was noticeably reduced (Table 2). When fixing at 3.8 m with both eyes open, some degree of anisometropia was present (0.527 diopters). Adie syndrome as a cause of amblyopia has previously been suggested in a case in which anisometropia was present.1–2 In our patient, the first refraction showed no sign of anisometropia, although 0.5 diopters was found at later refraction. Soylev and associates3 reported a case of mild amblyopia in a child diagnosed with Adie syndrome at age 8 years in whom anisocoria went unnoticed but was thought to have been present for 5 years. Ukai and Ishikawa4 have presented results of objective dynamic accommodation patterns in two young adult females with Adie syndrome. Upon monocular testing, each showed an VOL. 128, NO. 1
overshoot in the affected eye when changing fixation from distance to near, which the authors suggest may be caused by delay in the accommodation control system. In our patient, the accommodative level achieved is similar in either eye under monocular conditions. Our patient illustrates how anisometropic amblyopia may develop in Adie syndrome accompanied by apparent isohypermetropia.
Accepted for publication Feb 2, 1999. From the Department of Ophthalmology, University of California, San Francisco, San Francisco, California. Inquiries to Joan M. O’Brien, MD, Department of Ophthalmology, University of California, San Francisco, 10 Kirkham St, Box 0730, San Francisco, CA 94143-0730; fax: (415) 476-0336; e-mail: aleja@itsa. ucsf.edu
BRIEF REPORTS
119