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Neonatal asteroid hyalosis
Neonatal Asteroid Hyalosis Aldo Fantin, MD,a C. Gail S u m m e r s , M D , a.b and J. D o u g l a s C a m e r o n , M D a ~c
Asteroid hyalosis, noted in 0.83% of routine eye examinations, 1 is uncommon in younger patients and is more frequently seen in patients more than 60 years old. It has been considered to be related to an aging process, and when it occurs in younger patients, ocular disease is typically associated. 1 We report a 4week-old patient with Down syndrome and bilateral congenital cataracts who had unilateral asteroid hyalosis. CASE REPORT A 2-week-old girl was referred to this institution for bilateral dimming of the red reflex. She was born at term and had a prenatal diagnosis of Down syndrome (trisomy 21) made by amniocentesis. Examination showed no optokinetic response or eye popping reflex. Pupils were equal and reactive, without a relative afferent pupillary defect. Corneal diameters measured 10.25 mm in both eyes, and intraocular pressures measured 21 mm Hg. Examination with slit lamp biomicroscopy showed bilateral Brushfield spots and dense nuclear cataracts; peripheral vacuoles were also detected in the left lens. After pupillary dilation, indirect ophthalmoscopy through reduced media showed sharp disc margins, a decreased foveal reflex and no signs of choroidal or retinal disease. B-scan ultrasonography showed no retinal detachment, intraocular masses, or abnormal vitreous echoes. Uneventful pars plana cataract extraction and mechanized anterior vitrectomy were performed in the left eye at age 3 weeks and in the right eye at age 4 weeks. During anterior vitrectomy of the right eye small intravitreal particles were noted with observation through the operating microscope. Histopathologic examination of the vitreous aspirate (Figure 1) confirmed the clinical diagnosis of asteroid hyalosis. The postoperative course was uneventful. Aphaldc
From the Departments of Ophthalmology,a Pediatrics,b and Laboratory Medicine and Pathology/University of Minnesota, Minneapolis, Minnesota. Supported in part by an unrestrictedgrant from Research to Prevent Blindness Inc., New York, New York. Submitted August 27, 199Z RevMon acceptedJanuary 19, 1998. J AAPOS 1998;2:253-4. Reprint requests: C Gall Summers, MD, Department of Ophthalmology, University of Minnesota, Box 493 UMHC, 420 Delaware St SE, Minneapolis, M N 55455-0501. Copyright © 1998 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/98 $5.00 + 0 75/1/90042 ffournal of AAPOS
cycloplegic retinoscopy was +18.25 +1.00 x 30 right eye and +17.50 +1.00 x 70 left eye. Ophthalmoscopic examination showed sharp, pink discs with a cup-to-disc ratio of 0.1, a flat and normal-appearing peripheral retina, and signs of beginning foveal development. Visual attentiveness was reduced at age 2 months. Standard electroretinography performed with a contact lens electrode showed a normal scotopic response. In view of the finding of asteroid hyalosis, uric acid and glycosylated hemoglobin were measured, and both were found to be normal. DISCUSSION It is unusual to find asteroid hyalosis in children. In a series of 76 patients with asteroid hyalosis, the youngest patient was 53 years old. 2 In another report of 101 cases with asteroid hyalosis, 1 the youngest patient was 35 years old and had bilateral asteroid hyalosis and bilateral retinal vein occlusions. The mean age of nondiabetic patients with asteroid hyalosis has been reported at 69.4 years in 1 series compared with a mean age of 47.6 years for diabetics with asteroid hyalosis. 3 Wasano et aP reported a series of 59 patients with asteroid hyalosis that included a 30year-old patient with diabetes mellitus. A case control study has shown an increased rate of diabetes mellitus, hypertension, and atherosclerotic vascular disease in patients with asteroid hyalosis. 1 Furthermore, Safir et al2 found elevated serum uric acid levels in 28% of 46 patients with asteroid hyalosis. 2 To our knowledge, our case represents the youngest patient reported with asteroid hyalosis. In addition, this case report is unique because of the associated congenital cataracts and Down syndrome. Asteroid hyalosis is characterized by the presence of minute, discrete particles suspended within the vitreous cavity. Examination with use of electron microscopy shows particles consisting of mulfilaminar membranes characteristic of complex lipids such as phospholipids. 4-6 X-ray spectroscopy has demonstrated the presence of calcium and phosphorus. 4-6 Detailed biomicroscopic examination of the vitreous of patients with retinitis pigmentosa (ages ranging between 5 and 65 years) has revealed a bilaterally shrunken and collapsed vitreous with central white and irregular opacities, 7 similar to the clinical appearance of asteroid hyalosis. Takahashi et al 8 performed biomicroscopy with an E1 Bayadi-Kajiura lens and found cotton ball or spindle-shaped opacities in the vitreous in 90.5% of eyes with retinitis pigmentosa (ages 9 to 67 years) and in 69% of eyes with high myopia (ages 24 to 76 years). Most of these patients had a posterior vitreous separation, which is unusual in asteroid hyalosis. 3, 6 Histopathologic studies August 1998
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2 ~4 Fantin, Summers, and Cameron
Journal ofAAPOS Volume 2 Number 4 August 1998 T h e r e were no clinical or electrophysiologic signs of a retinochoroidal degeneration in our infant patient with asteroid hyalosis. In addition, the patient had normal levels of uric acid and glycosylated hemoglobin. T h e asteroid particles, easily seen with an operating microscope, were confirmed by histopathologic examination. At this time, there is no explanation for the unusual association of unilateral asteroid hyalosis, bilateral congenital cataracts, and delayed macular development in this child with Down syndrome. REFERENCES
FI6 1 Multiple spheroidal, uniformly staining bodies are present in vitrectomy specimen consistent with calcium soaps of asteroid hyalosis. In addition, melanin granules from iris pigment epithelium and cortical remnants from crystalline lens are present in background. (Hematoxylin-eosin stain, original magnification ><400.) to confirm asteroid hyalosis were not performed in these patients. However, one patient with retinal degeneration because of Alstr6m syndrome was reported to have asteroid hyalosis at age 8 years, and unilateral asteroid hyalosis and bilateral absence of rods and cones were reported on necropsy at age 32 years. 9 These findings were similar to the asteroid hyalosis that has been recognized in retinitis pigmentosa and high myopia in that the opacities were localized in the posterior vitreous, near the retina, and are probably the result of chorioretinal degeneration.
1. Bergren RL, Brown GC, Duker JS. Prevalence and association of asteroid hyalosis with systemic diseases. Am J Ophthalmol 1991;111:28%93. 2. Safir A, Dram SN, Martin RG, Tare GW,, Mincey GJ. Is asteroid hyalosis ocular gout? Ann Ophthalmol 1990;22:70-7. 3. Wasano T, Hirokawa H, Tagawa H, Trempe CL, Buzney SM. Asteroid hyalosis: posterior vitreous detachment and diabetic retinopathy.Ann Ophthalmol 1987;19:255-8. 4. AdenisJP, Leboutet MJ, Loubet R. La hyalopathieast~roide: etude ultra-structurale de trois cas.J Fr Ophtalmol 1984;7:529-34. 5. Streeten BW. Vitreous asteroidbodies:ultrastruetural characteristics and composition.Arch Ophthalmol 1982;100:969-75. 6. TopilowHW,, Kenyon KR, Takahashi M, Freeman HM, To]entino FI, Hanninen LA. Asteroid hyalosis: biomicroscopy,ultrastrucmre, and composition.Arch Ophthalmol 1982;100:964-8. 7. Pruett RC. Retinitis pigmentosa: a biomicroscopica]study of vitreous abnormalities.Arch Ophthalmol 1975;93:603-8. 8. Takahashi M, Jalkh A, Hoskins J, Trempe CL, Schepens CL. Biomicroscopicevaluationand photography of Liquefiedvitreous in some vitreoretinaldisorders. Arch Ophthalmol 1981;99:1555-9. 9. SebagJ, Albert DM, Craft JL. The Alstr6m syndrome: ophthalmic histopathology and retinal ultrastructure. Br J Ophtha]mol 1984;68:494-501.
Asteroid hyalosis, noted in 0.83% of routine eye examinations, 1 is uncommon in younger patients and is more frequently seen in patients more than 60 years old. It has been considered to be related to an aging process, and when it occurs in younger patients, ocular disease is typically associated. 1 We report a 4week-old patient with Down syndrome and bilateral congenital cataracts who had unilateral asteroid hyalosis. CASE REPORT A 2-week-old girl was referred to this institution for bilateral dimming of the red reflex. She was born at term and had a prenatal diagnosis of Down syndrome (trisomy 21) made by amniocentesis. Examination showed no optokinetic response or eye popping reflex. Pupils were equal and reactive, without a relative afferent pupillary defect. Corneal diameters measured 10.25 mm in both eyes, and intraocular pressures measured 21 mm Hg. Examination with slit lamp biomicroscopy showed bilateral Brushfield spots and dense nuclear cataracts; peripheral vacuoles were also detected in the left lens. After pupillary dilation, indirect ophthalmoscopy through reduced media showed sharp disc margins, a decreased foveal reflex and no signs of choroidal or retinal disease. B-scan ultrasonography showed no retinal detachment, intraocular masses, or abnormal vitreous echoes. Uneventful pars plana cataract extraction and mechanized anterior vitrectomy were performed in the left eye at age 3 weeks and in the right eye at age 4 weeks. During anterior vitrectomy of the right eye small intravitreal particles were noted with observation through the operating microscope. Histopathologic examination of the vitreous aspirate (Figure 1) confirmed the clinical diagnosis of asteroid hyalosis. The postoperative course was uneventful. Aphaldc
From the Departments of Ophthalmology,a Pediatrics,b and Laboratory Medicine and Pathology/University of Minnesota, Minneapolis, Minnesota. Supported in part by an unrestrictedgrant from Research to Prevent Blindness Inc., New York, New York. Submitted August 27, 199Z RevMon acceptedJanuary 19, 1998. J AAPOS 1998;2:253-4. Reprint requests: C Gall Summers, MD, Department of Ophthalmology, University of Minnesota, Box 493 UMHC, 420 Delaware St SE, Minneapolis, M N 55455-0501. Copyright © 1998 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/98 $5.00 + 0 75/1/90042 ffournal of AAPOS
cycloplegic retinoscopy was +18.25 +1.00 x 30 right eye and +17.50 +1.00 x 70 left eye. Ophthalmoscopic examination showed sharp, pink discs with a cup-to-disc ratio of 0.1, a flat and normal-appearing peripheral retina, and signs of beginning foveal development. Visual attentiveness was reduced at age 2 months. Standard electroretinography performed with a contact lens electrode showed a normal scotopic response. In view of the finding of asteroid hyalosis, uric acid and glycosylated hemoglobin were measured, and both were found to be normal. DISCUSSION It is unusual to find asteroid hyalosis in children. In a series of 76 patients with asteroid hyalosis, the youngest patient was 53 years old. 2 In another report of 101 cases with asteroid hyalosis, 1 the youngest patient was 35 years old and had bilateral asteroid hyalosis and bilateral retinal vein occlusions. The mean age of nondiabetic patients with asteroid hyalosis has been reported at 69.4 years in 1 series compared with a mean age of 47.6 years for diabetics with asteroid hyalosis. 3 Wasano et aP reported a series of 59 patients with asteroid hyalosis that included a 30year-old patient with diabetes mellitus. A case control study has shown an increased rate of diabetes mellitus, hypertension, and atherosclerotic vascular disease in patients with asteroid hyalosis. 1 Furthermore, Safir et al2 found elevated serum uric acid levels in 28% of 46 patients with asteroid hyalosis. 2 To our knowledge, our case represents the youngest patient reported with asteroid hyalosis. In addition, this case report is unique because of the associated congenital cataracts and Down syndrome. Asteroid hyalosis is characterized by the presence of minute, discrete particles suspended within the vitreous cavity. Examination with use of electron microscopy shows particles consisting of mulfilaminar membranes characteristic of complex lipids such as phospholipids. 4-6 X-ray spectroscopy has demonstrated the presence of calcium and phosphorus. 4-6 Detailed biomicroscopic examination of the vitreous of patients with retinitis pigmentosa (ages ranging between 5 and 65 years) has revealed a bilaterally shrunken and collapsed vitreous with central white and irregular opacities, 7 similar to the clinical appearance of asteroid hyalosis. Takahashi et al 8 performed biomicroscopy with an E1 Bayadi-Kajiura lens and found cotton ball or spindle-shaped opacities in the vitreous in 90.5% of eyes with retinitis pigmentosa (ages 9 to 67 years) and in 69% of eyes with high myopia (ages 24 to 76 years). Most of these patients had a posterior vitreous separation, which is unusual in asteroid hyalosis. 3, 6 Histopathologic studies August 1998
255
2 ~4 Fantin, Summers, and Cameron
Journal ofAAPOS Volume 2 Number 4 August 1998 T h e r e were no clinical or electrophysiologic signs of a retinochoroidal degeneration in our infant patient with asteroid hyalosis. In addition, the patient had normal levels of uric acid and glycosylated hemoglobin. T h e asteroid particles, easily seen with an operating microscope, were confirmed by histopathologic examination. At this time, there is no explanation for the unusual association of unilateral asteroid hyalosis, bilateral congenital cataracts, and delayed macular development in this child with Down syndrome. REFERENCES
FI6 1 Multiple spheroidal, uniformly staining bodies are present in vitrectomy specimen consistent with calcium soaps of asteroid hyalosis. In addition, melanin granules from iris pigment epithelium and cortical remnants from crystalline lens are present in background. (Hematoxylin-eosin stain, original magnification ><400.) to confirm asteroid hyalosis were not performed in these patients. However, one patient with retinal degeneration because of Alstr6m syndrome was reported to have asteroid hyalosis at age 8 years, and unilateral asteroid hyalosis and bilateral absence of rods and cones were reported on necropsy at age 32 years. 9 These findings were similar to the asteroid hyalosis that has been recognized in retinitis pigmentosa and high myopia in that the opacities were localized in the posterior vitreous, near the retina, and are probably the result of chorioretinal degeneration.
1. Bergren RL, Brown GC, Duker JS. Prevalence and association of asteroid hyalosis with systemic diseases. Am J Ophthalmol 1991;111:28%93. 2. Safir A, Dram SN, Martin RG, Tare GW,, Mincey GJ. Is asteroid hyalosis ocular gout? Ann Ophthalmol 1990;22:70-7. 3. Wasano T, Hirokawa H, Tagawa H, Trempe CL, Buzney SM. Asteroid hyalosis: posterior vitreous detachment and diabetic retinopathy.Ann Ophthalmol 1987;19:255-8. 4. AdenisJP, Leboutet MJ, Loubet R. La hyalopathieast~roide: etude ultra-structurale de trois cas.J Fr Ophtalmol 1984;7:529-34. 5. Streeten BW. Vitreous asteroidbodies:ultrastruetural characteristics and composition.Arch Ophthalmol 1982;100:969-75. 6. TopilowHW,, Kenyon KR, Takahashi M, Freeman HM, To]entino FI, Hanninen LA. Asteroid hyalosis: biomicroscopy,ultrastrucmre, and composition.Arch Ophthalmol 1982;100:964-8. 7. Pruett RC. Retinitis pigmentosa: a biomicroscopica]study of vitreous abnormalities.Arch Ophthalmol 1975;93:603-8. 8. Takahashi M, Jalkh A, Hoskins J, Trempe CL, Schepens CL. Biomicroscopicevaluationand photography of Liquefiedvitreous in some vitreoretinaldisorders. Arch Ophthalmol 1981;99:1555-9. 9. SebagJ, Albert DM, Craft JL. The Alstr6m syndrome: ophthalmic histopathology and retinal ultrastructure. Br J Ophtha]mol 1984;68:494-501.