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Advanced coats' disease: A new finding
Advanced
Coats’
disease:
A new finding David H. Abramson, MD, FACS Camille A. Servodidio, RN, MPH, CRNO Katherine S. Budinger, BA, COA Les Saint-Louis, MD
Computed tomographic imaging can be a crucial aid for determining the diagnosis in a child who presents with leukocoria. A case of a 6’/2-month-old child with advanced Coats’ disease is presented in which a new finding on the computed tomographic scan helped confirm the diagnosis and distinguish it from retinoblastoma.
David H. Abramson, MD, FACS, Camille A. Servodidio, RN, MPH, CRNO, and are affiliated with Cornell University Medical College, New York, N. Y. Les SaintLouis, MD, is affiliated with Corinthian Diagnostic Radiology, New York, N. Y. Supported by the Louis and Rachel Rudin Foundation, Inc., New York. Reprint requests: David H. Abramson, MD, FACS, 70 E. 66th St., New York, NY 10027. Copyright 0 7 997 by the American Society of Ophthalmic Registered Nurses. 7060- 735X/97 $5.00 + 0 72/l/85753
114
Volume
(Insight 1997;22: 114-6)
oats’ diseasecan be defined as the presenceof abnormal, telangiectatic, leaking retinal ‘blood vesselswith associatedyellowish or greenish subretinal exudates. Coats’ diseaseis usually unilateral, affects males more often than females, and presentsbetween the agesof 8 and 10 years old.’ Retinoblastoma, the most common primary intraocular cancer of childhood, is depicted as one or more white calcified mass(es)in the fundus. Retinoblastoma may be unilateral or bilateral, affects boys as frequently as girls, and usually presents before 5 years of age.2 A pediatrician noted an absenceof a red reflex in the right eye of a 61/z-month-old girl and referred her to an ophthalmologist. Her medical history was unremarkable, and her birth weight was within normal limits. The right upper lid was less than 1 mm elevatedwhen compared with the left upper lid. Pupillary examination revealeda slightly larger pupil OD (3.0 x 2.5 mm) when compared with OS (2.5 mm). Visual acuity measured OD: NLP and OS: Fixed and Followed Light. Slit lamp examination under anesthesiarevealed clear corneasOU; OD measured 12.0 mm in vertical diameter by 13.0 mm in horizontal diameter; OS measured 11.Omm in vertical diameter by 11.Omm in horizontal
XXII, No. 4, December 1997
INSIGHT
diameter. A shallow anterior chamber, rubeosis iridis, and an anteriorly placed lens were noted OD. Heterochromia was not present. Intraocular pressuremeasured OD: 5 mm Hg and OS: 18.5 mm Hg with the Shiotz tonometer. The cornea, iris, lens, and anterior chamber of the left eye were normal. Fundus examination of the right eye revealeda multilobed detachment with telangiectatic abnormal vesselsnoted on the surfaceof the detachment. The peripheral edgesof the retina were folded over, and a rolled skirt of retina was present centrally that extended behind the lens across the iris and ciliary body and tapered out to the ora serrata.The subretinal materials were yellowish (Fig. 1). No fundus details were visible. B-scanconfirmed a total retinal detachment extending anterior to the ora serrata.The fundus of the left eye revealed a C/D of 0.0 and without retinoblastoma tumors. A computed tomography (CT) scan and a magnetic resonanceimaging (MRI) scan were performed of the eyesand orbits that showed an almost total opacification of the right globe with homogeneous intensity on all sequences.On CT scan, axial lengths measuredOD: 20 mm and OS: 21 mm. The right lens was displaced anteriorly; this is unusual in retinoblastoma. There
The Journal of the American
Society of Ophthalmic
Registered Nurses, Inc.
Abramson
Fig. 1. Anterior segment telangiectatic vessels.
photograph
Fig. 2. Computed tomographic ment in Coats’ disease.
depicting
scan with
was no calcification within either globe. The optic nerves, retrobulbar structures, sella, parasella, and brain were within normal limits. The CT scan also revealed a total retinal detachment with homogeneous subretinal fluid that was without calcification; no markedly thickened areas of the retina were present. The detached retina bowed from the anterior insertion at INSIGHT
The Journal of the American
Society of Ophthalmic
the retinal
schematic
drawing
detachment
of retinal
et al.
with
detach-
the ora serrata and ran parallel to the pars plana, ciliary body, and folded over itself just posterior to the lens; this helped to confirm the diagnosis of advanced Coats’ disease (Fig. 2). The homogeneous density within the right globe appeared hyperintense on T-2 weighted image and mildly hyperintense on proton and T-l weighted image; this is consistent with what others
Registered Nurses, Inc.
Volume XXII, No. 4, December 1997
11.5
Abramson
et al.
have reported as a homogeneous, hyperintense (ranging from moderate to high) signal on both T-l and T-2 weighted images depicting the subretinal material in Coats’ disease.3 COMMENTS
Distinguishing retinoblastoma from advanced Coats’ diseaseremains the greatest diagnostic challenge to the ophthalmic oncologist. Clinical featuresare helpful but not diagnostic. Although needle biopsy of Coats’ diseasemay be safe,needle perforation of eyeswith retinoblastoma is rarely done becauseof the fear of spreading the tumor and compromising life. Retinoblastoma is bilateral in 33% of the cases;in contrast, Coats’ diseaseis unilateral in virtually all cases.Retinoblastoma occurs equally in boys and girls whereas Coats’ diseaseis more common in boys. Bilateral retinoblastoma is usually diagnosed by 12 months of ageand unilateral retinoblastoma by 24 months of age;however, Coats’ diseaseoccurs at a mean ageof 7 years.Retinoblastoma rarely has peripheral retinal telangiectatic vesselswhereas Coats’ diseasecommonly has such vessels. Retinoblastoma with an associatedretinal detachment has a creamy white color (similar to mayonnaise); the subretinal fluid of Coats’ diseaseappearsyellow or almost green at times. Imaging studies are helpful but not pathognomonic. Ultrasound commonly
116
Volume XXII, No. 4, December 1997
revealscalcification in retinoblastoma that is found only rarely in Coats’ disease.MRI is sensitive for the presenceof exudate so that in Coats’ diseaseT-2 weighted images arebright. Retinoblastoma on T-2 weighted images is darker except when the subretinal fluid of retinoblastoma contains proteinaceous exudate, then it is also bright on T-2 weighted images. Advanced Coats’ diseasecausesa multilobed retinal detachment. Becauseof the viscous nature of the subretinal fluid, which consists of exudate, cholesterol, and macrophages,the material pushesthe retina anteriorally from the ora serrata.The retina is reflected onto the pars plana and back surfaceof the ciliary body and lens. This additional finding, demonstrated pathologically many times before, is now identified in vivo by modern CT scanning of the globe and may be helpful in distinguishing the life-threatening retinoblastoma from the sight and globe threatening nonmalignant Coats’ disease. References 1. Basic and Clinical Science Course, Section 6. Pediatric Ophthalmology and Strabismus. American Academy of Ophthalmology 1996-1997:119-20. 2. Basic and Clinical Science Course, Section 6. Pediatric Ophthalmology and Strabismus. American Academy of Ophthalmology 1996-1997:164-7. 3. DePotter P, Shields CL, Shields JA, Flanders AE. The role of magnetic resonance imaging in children with intraocular tumors and simulating lesions. Ophthalmology 1996;103:1774-83.
kiSlciWr
The Journal of the American
Society of Ophthalmic
Registered Nurses, Inc
Coats’
disease:
A new finding David H. Abramson, MD, FACS Camille A. Servodidio, RN, MPH, CRNO Katherine S. Budinger, BA, COA Les Saint-Louis, MD
Computed tomographic imaging can be a crucial aid for determining the diagnosis in a child who presents with leukocoria. A case of a 6’/2-month-old child with advanced Coats’ disease is presented in which a new finding on the computed tomographic scan helped confirm the diagnosis and distinguish it from retinoblastoma.
David H. Abramson, MD, FACS, Camille A. Servodidio, RN, MPH, CRNO, and are affiliated with Cornell University Medical College, New York, N. Y. Les SaintLouis, MD, is affiliated with Corinthian Diagnostic Radiology, New York, N. Y. Supported by the Louis and Rachel Rudin Foundation, Inc., New York. Reprint requests: David H. Abramson, MD, FACS, 70 E. 66th St., New York, NY 10027. Copyright 0 7 997 by the American Society of Ophthalmic Registered Nurses. 7060- 735X/97 $5.00 + 0 72/l/85753
114
Volume
(Insight 1997;22: 114-6)
oats’ diseasecan be defined as the presenceof abnormal, telangiectatic, leaking retinal ‘blood vesselswith associatedyellowish or greenish subretinal exudates. Coats’ diseaseis usually unilateral, affects males more often than females, and presentsbetween the agesof 8 and 10 years old.’ Retinoblastoma, the most common primary intraocular cancer of childhood, is depicted as one or more white calcified mass(es)in the fundus. Retinoblastoma may be unilateral or bilateral, affects boys as frequently as girls, and usually presents before 5 years of age.2 A pediatrician noted an absenceof a red reflex in the right eye of a 61/z-month-old girl and referred her to an ophthalmologist. Her medical history was unremarkable, and her birth weight was within normal limits. The right upper lid was less than 1 mm elevatedwhen compared with the left upper lid. Pupillary examination revealeda slightly larger pupil OD (3.0 x 2.5 mm) when compared with OS (2.5 mm). Visual acuity measured OD: NLP and OS: Fixed and Followed Light. Slit lamp examination under anesthesiarevealed clear corneasOU; OD measured 12.0 mm in vertical diameter by 13.0 mm in horizontal diameter; OS measured 11.Omm in vertical diameter by 11.Omm in horizontal
XXII, No. 4, December 1997
INSIGHT
diameter. A shallow anterior chamber, rubeosis iridis, and an anteriorly placed lens were noted OD. Heterochromia was not present. Intraocular pressuremeasured OD: 5 mm Hg and OS: 18.5 mm Hg with the Shiotz tonometer. The cornea, iris, lens, and anterior chamber of the left eye were normal. Fundus examination of the right eye revealeda multilobed detachment with telangiectatic abnormal vesselsnoted on the surfaceof the detachment. The peripheral edgesof the retina were folded over, and a rolled skirt of retina was present centrally that extended behind the lens across the iris and ciliary body and tapered out to the ora serrata.The subretinal materials were yellowish (Fig. 1). No fundus details were visible. B-scanconfirmed a total retinal detachment extending anterior to the ora serrata.The fundus of the left eye revealed a C/D of 0.0 and without retinoblastoma tumors. A computed tomography (CT) scan and a magnetic resonanceimaging (MRI) scan were performed of the eyesand orbits that showed an almost total opacification of the right globe with homogeneous intensity on all sequences.On CT scan, axial lengths measuredOD: 20 mm and OS: 21 mm. The right lens was displaced anteriorly; this is unusual in retinoblastoma. There
The Journal of the American
Society of Ophthalmic
Registered Nurses, Inc.
Abramson
Fig. 1. Anterior segment telangiectatic vessels.
photograph
Fig. 2. Computed tomographic ment in Coats’ disease.
depicting
scan with
was no calcification within either globe. The optic nerves, retrobulbar structures, sella, parasella, and brain were within normal limits. The CT scan also revealed a total retinal detachment with homogeneous subretinal fluid that was without calcification; no markedly thickened areas of the retina were present. The detached retina bowed from the anterior insertion at INSIGHT
The Journal of the American
Society of Ophthalmic
the retinal
schematic
drawing
detachment
of retinal
et al.
with
detach-
the ora serrata and ran parallel to the pars plana, ciliary body, and folded over itself just posterior to the lens; this helped to confirm the diagnosis of advanced Coats’ disease (Fig. 2). The homogeneous density within the right globe appeared hyperintense on T-2 weighted image and mildly hyperintense on proton and T-l weighted image; this is consistent with what others
Registered Nurses, Inc.
Volume XXII, No. 4, December 1997
11.5
Abramson
et al.
have reported as a homogeneous, hyperintense (ranging from moderate to high) signal on both T-l and T-2 weighted images depicting the subretinal material in Coats’ disease.3 COMMENTS
Distinguishing retinoblastoma from advanced Coats’ diseaseremains the greatest diagnostic challenge to the ophthalmic oncologist. Clinical featuresare helpful but not diagnostic. Although needle biopsy of Coats’ diseasemay be safe,needle perforation of eyeswith retinoblastoma is rarely done becauseof the fear of spreading the tumor and compromising life. Retinoblastoma is bilateral in 33% of the cases;in contrast, Coats’ diseaseis unilateral in virtually all cases.Retinoblastoma occurs equally in boys and girls whereas Coats’ diseaseis more common in boys. Bilateral retinoblastoma is usually diagnosed by 12 months of ageand unilateral retinoblastoma by 24 months of age;however, Coats’ diseaseoccurs at a mean ageof 7 years.Retinoblastoma rarely has peripheral retinal telangiectatic vesselswhereas Coats’ diseasecommonly has such vessels. Retinoblastoma with an associatedretinal detachment has a creamy white color (similar to mayonnaise); the subretinal fluid of Coats’ diseaseappearsyellow or almost green at times. Imaging studies are helpful but not pathognomonic. Ultrasound commonly
116
Volume XXII, No. 4, December 1997
revealscalcification in retinoblastoma that is found only rarely in Coats’ disease.MRI is sensitive for the presenceof exudate so that in Coats’ diseaseT-2 weighted images arebright. Retinoblastoma on T-2 weighted images is darker except when the subretinal fluid of retinoblastoma contains proteinaceous exudate, then it is also bright on T-2 weighted images. Advanced Coats’ diseasecausesa multilobed retinal detachment. Becauseof the viscous nature of the subretinal fluid, which consists of exudate, cholesterol, and macrophages,the material pushesthe retina anteriorally from the ora serrata.The retina is reflected onto the pars plana and back surfaceof the ciliary body and lens. This additional finding, demonstrated pathologically many times before, is now identified in vivo by modern CT scanning of the globe and may be helpful in distinguishing the life-threatening retinoblastoma from the sight and globe threatening nonmalignant Coats’ disease. References 1. Basic and Clinical Science Course, Section 6. Pediatric Ophthalmology and Strabismus. American Academy of Ophthalmology 1996-1997:119-20. 2. Basic and Clinical Science Course, Section 6. Pediatric Ophthalmology and Strabismus. American Academy of Ophthalmology 1996-1997:164-7. 3. DePotter P, Shields CL, Shields JA, Flanders AE. The role of magnetic resonance imaging in children with intraocular tumors and simulating lesions. Ophthalmology 1996;103:1774-83.
kiSlciWr
The Journal of the American
Society of Ophthalmic
Registered Nurses, Inc