604
Tan and Chang
4. Burn J, Brennan P, Little J, et al. Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study. Lancet 1998;351:311-16. 5. McElhinney DB, Geiger E, Blinder J, et al. NKX2.5 mutations in patients with congenital heart disease. J Am Coll Cardiol 2003;42:1650-55. 6. Goel N, Kumar V, Seth A, Ghosh B. Proliferative retinopathy in a child with congenital cyanotic heart disease. J AAPOS 2010;14:455-6. 7. Tsai FF, Spindle J, Lazzaro EC, Olumba KC. Central retinal artery occlusion in a patient with tetralogy of Fallot. J AAPOS 2013;17:621-2. 8. Mansour AM, Bitar FF, Traboulsi EI, et al. Ocular pathology in congenital heart disease. Eye 2004;19:29-34. 9. VanderVeen DK, Pasquale LR, Fulton AB. Central retinal vein occlusion in a young child with cyanotic heart disease. Arch Ophthalmol 1997;115:1077. 10. Qiao Y, Tyson C, Hrynchak M, et al. Clinical application of 2.7M Cytogenetics array for CNV detection in subjects with idiopathic autism and/or intellectual disability. Clinical Genetics 2012;83:145-54.
An unusual case of coats disease associated with Takayasu arteritis Pok Chien Tan, MB ChB, and Benjamin Chong-Ming Chang, FRCOphth A 3-year-old Malay boy with strabismus in the left eye was found to have Coats disease in the same eye and underlying Takayasu arteritis. Coats disease is usually idiopathic but can be associated with systemic and ocular conditions. To our knowledge, this is the first reported case of Coats disease associated with Takayasu arteritis.
Case Report
A
3-year-old Malay boy presented at Khoo Teck Puat Hospital, Singapore, with left eye strabismus of 2 months’ duration. He had no significant medical or family history. On examination, his best-corrected visual acuity was 20/25 in the right eye and counting fingers in the left eye. Orthoptic assessment was consistent with a left sensory exotropia. Anterior segment examination was unremarkable. Dilated fundus examination of the left eye revealed the presence of extensive yellow exudates and edema at the posterior pole, with telangiectatic vessels and fusiform dilation of retinal arterioles (Figure 1). The fundus of the right eye appeared normal. A diagnosis of
Author affiliations: Ophthalmology and Visual Science Department, Khoo Teck Puat Hospital, Singapore, KK Women’s and Children’s Hospital, Singapore Submitted April 16, 2014. Revision accepted July 6, 2014. Published online November 12, 2014. Correspondence: Pok Chien Tan, MB ChB, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828 (email: [email protected], [email protected]). J AAPOS 2014;18:604-605. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.07.164
Volume 18 Number 6 / December 2014
FIG 1. Fundus photograph of the right eye (A) and left eye (B), which shows extensive yellow exudates and edema at the posterior pole with telangiectatic vessels and fusiform dilation of retinal arterioles.
Coats disease was made and diode laser photocoagulation to the telangiectatic vessels of the left eye was performed under general anesthesia. Intraoperatively the patient was found to have severe hypertension that necessitated intensive-care monitoring and control of blood pressure. Magnetic resonance angiography (MRA) renal vessels and magnetic resonance imaging (MRI) of the thorax were subsequently performed. The investigations revealed bilateral renal artery stenosis and tapered narrowing of the prerenal abdominal aorta distal to the origin of the mesenteric artery with periaortic soft tissue, which were consistent with Takayasu arteritis. Blood investigations showed raised inflammatory markers (erythrocyte sedimentation rate of 101 mm/hr and C-reactive protein of 21.6 mg/L) and elevated serum renin and aldosterone. Other tests, including full blood count, renal panel, thyroid function test, liver function test, TB T-spot test, chest X-ray and autoimmune workup were unremarkable. The patient responded well to oral prednisolone, methotrexate, and antihypertensive medications and was discharged after 18 days of in-patient care. On follow-up, progressive resolution of most of the exudates and retinal edema was noted with a return to normal retinal vessel caliber at 9 months postoperatively (Figure 2). Best-corrected visual acuity at that time was 20/200 in the left eye.
Discussion To our knowledge, this is the first case showing an association of Coats disease with Takayasu arteritis. Coats disease is unilateral in most cases and classically presents with yellow lipid-rich subretinal and intraretinal exudates and abnormalities in the retinal vessels, including telangiectasia, aneurysmal dilatations, and vascular tortuosity,1 as in our case. In later stages, the exudates can progress to a total retinal detachment.2 Untreated, the disease can lead to neovascular glaucoma and phthisis bulbi in advanced stages.1 The incidental finding of severe hypertension intraoperatively gave rise to a suspicion of the underlying Takayasu arteritis. Takayasu arteritis is characterized by attenuation of the aorta and its branches, as a result of autoimmune inflammation of the arteries,3 which was corroborated by
Journal of AAPOS
Volume 18 Number 6 / December 2014
Hildebrand and Painter
605
2. Rubin MP, Mukai S. Coats’ disease. Int Ophthalmol Clin 2008;48: 149-58. 3. Peter J, David S, Danda D, Peter JV, Horo S, Joseph G. Ocular manifestations of Takayasu arteritis: a cross sectional study. Retina 2011; 31:1170-78. 4. Uyama M, Asayma K. Retinal vascular changes in Takayasu’s arteritis (pulseless disease): occurrence and evolution of the lesion. Doc Ophthalmol Proc Series 1976;9:549-54. 5. Panja M, Mondal PC. Current status of aortoarteritis in India. J Assoc Physicians India 2004;52:48-52. 6. He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats’ disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthalmol 2010;248:1519-21. 7. Harada M, Yoshida H, Mitusyama K, et al. Aortitis syndrome (Takayasu’s arteritis) with cataract and elevated serum level of vascular endothelial growth factor. Scand J Rheumatol 1998;27:78-9.
FIG 2. Fundus photograph of the left eye showing resolution of most of the exudates and retinal edema postoperatively.
radiologic findings in our case. Reported ocular manifestations of Takayasu arteritis include Takayasu (hypoperfusive) retinopathy, which is characterized by distention of veins, microaneurysms, and formation of arteriovenous anastomoses.4 Takayasu arteritis can also manifest as hypertensive retinopathy or mixed retinopathy, that is, presence of both hypoperfusive and hypertensive retinopathy.5 A recent cross-sectional study reported that Takayasu retinopathy and hypertensive retinopathy were present in 14.6% and 16.4%, respectively, of Takayasu arteritis patients; 1.6% had mixed retinopathy.3 It is possible that in this case Takayasu arteritis was mimicking Coats disease. Although some of the ocular signs described for Takayasu arteritis such, as distension of veins and microaneurysms, were found in our case, Takayasu arteritis does not usually present with extensive retinal exudates and the majority of patients have bilateral disease.3 It seems more likely that this is a case of Coats disease with coexisting Takayasu arteritis, and that the underlying arteritis and inflammatory process may have exacerbated the clinical presentation of Coats disease. He and colleagues6 reported raised levels of vascular endothelial growth factor (VEGF) in eyes with Coats disease, and Harada and colleagues7 reported elevated level of VEGF in the serum of patients with Takayasu arteritis. It is difficult to accurately ascertain their combined role in the etiopathogenesis of the present case.
Literature Search PubMed was last searched on April 16, 2014, without date or language restrictions, using the following terms: Coats disease, Takayasu arteritis, Takayasu retinopathy, Coats disease AND Takayasu arteritis, Coats disease, AND Takayasu retinopathy. References 1. Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol 2001;131:561-71.
Journal of AAPOS
A simple technique for temporary eyelid closure in severe exposure keratopathy G€ oran Darius Hildebrand, FRCS, FRCOphth, and Sally L. Painter, FRCOphth We describe a novel and simple technique for temporary lid closure in severe bilateral corneal exposure keratopathy, where a rapid method for corneal protection is required. The aim is to create a stable dressing base for secure closure of the eyelids that also allows instillation of medication and examination of the eye.
C
orneal exposure may be prevented and treated with eyelid closure, which may be achieved through occlusive dressings, botulinum toxin induced ptosis, sutures with or without bolsters, and glue or surgical tarsorrhaphies.1-6 Simple taping techniques are often problematic because tape does not adhere well to oily skin; eyelids do not stay closed and there is a potential for rubbing of the tape against the ulcerated cornea. Dressing techniques that have been described for patients under general anesthesia are not appropriate for awake patients because they have the potential to remove eyelashes.7 Surgical intervention may neither be appropriate nor possible in an unwell patient.
Author affiliations: Department of Pediatric Ophthalmology, Royal Berkshire Hospital, Reading and King Edward VII Hospital, Windsor, United Kingdom Submitted April 9, 2014. Revision accepted July 7, 2014. Published online November 12, 2014. Correspondence: Mr. G€oran Darius Hildebrand, FRCS, FRCOphth, Department of Ophthalmology, Reading and King Edward VII Hospital, Windsor, UK (email: [email protected]). J AAPOS 2014;18:605-606. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.07.171
Tan and Chang
4. Burn J, Brennan P, Little J, et al. Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study. Lancet 1998;351:311-16. 5. McElhinney DB, Geiger E, Blinder J, et al. NKX2.5 mutations in patients with congenital heart disease. J Am Coll Cardiol 2003;42:1650-55. 6. Goel N, Kumar V, Seth A, Ghosh B. Proliferative retinopathy in a child with congenital cyanotic heart disease. J AAPOS 2010;14:455-6. 7. Tsai FF, Spindle J, Lazzaro EC, Olumba KC. Central retinal artery occlusion in a patient with tetralogy of Fallot. J AAPOS 2013;17:621-2. 8. Mansour AM, Bitar FF, Traboulsi EI, et al. Ocular pathology in congenital heart disease. Eye 2004;19:29-34. 9. VanderVeen DK, Pasquale LR, Fulton AB. Central retinal vein occlusion in a young child with cyanotic heart disease. Arch Ophthalmol 1997;115:1077. 10. Qiao Y, Tyson C, Hrynchak M, et al. Clinical application of 2.7M Cytogenetics array for CNV detection in subjects with idiopathic autism and/or intellectual disability. Clinical Genetics 2012;83:145-54.
An unusual case of coats disease associated with Takayasu arteritis Pok Chien Tan, MB ChB, and Benjamin Chong-Ming Chang, FRCOphth A 3-year-old Malay boy with strabismus in the left eye was found to have Coats disease in the same eye and underlying Takayasu arteritis. Coats disease is usually idiopathic but can be associated with systemic and ocular conditions. To our knowledge, this is the first reported case of Coats disease associated with Takayasu arteritis.
Case Report
A
3-year-old Malay boy presented at Khoo Teck Puat Hospital, Singapore, with left eye strabismus of 2 months’ duration. He had no significant medical or family history. On examination, his best-corrected visual acuity was 20/25 in the right eye and counting fingers in the left eye. Orthoptic assessment was consistent with a left sensory exotropia. Anterior segment examination was unremarkable. Dilated fundus examination of the left eye revealed the presence of extensive yellow exudates and edema at the posterior pole, with telangiectatic vessels and fusiform dilation of retinal arterioles (Figure 1). The fundus of the right eye appeared normal. A diagnosis of
Author affiliations: Ophthalmology and Visual Science Department, Khoo Teck Puat Hospital, Singapore, KK Women’s and Children’s Hospital, Singapore Submitted April 16, 2014. Revision accepted July 6, 2014. Published online November 12, 2014. Correspondence: Pok Chien Tan, MB ChB, Khoo Teck Puat Hospital, 90 Yishun Central, Singapore 768828 (email: [email protected], [email protected]). J AAPOS 2014;18:604-605. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.07.164
Volume 18 Number 6 / December 2014
FIG 1. Fundus photograph of the right eye (A) and left eye (B), which shows extensive yellow exudates and edema at the posterior pole with telangiectatic vessels and fusiform dilation of retinal arterioles.
Coats disease was made and diode laser photocoagulation to the telangiectatic vessels of the left eye was performed under general anesthesia. Intraoperatively the patient was found to have severe hypertension that necessitated intensive-care monitoring and control of blood pressure. Magnetic resonance angiography (MRA) renal vessels and magnetic resonance imaging (MRI) of the thorax were subsequently performed. The investigations revealed bilateral renal artery stenosis and tapered narrowing of the prerenal abdominal aorta distal to the origin of the mesenteric artery with periaortic soft tissue, which were consistent with Takayasu arteritis. Blood investigations showed raised inflammatory markers (erythrocyte sedimentation rate of 101 mm/hr and C-reactive protein of 21.6 mg/L) and elevated serum renin and aldosterone. Other tests, including full blood count, renal panel, thyroid function test, liver function test, TB T-spot test, chest X-ray and autoimmune workup were unremarkable. The patient responded well to oral prednisolone, methotrexate, and antihypertensive medications and was discharged after 18 days of in-patient care. On follow-up, progressive resolution of most of the exudates and retinal edema was noted with a return to normal retinal vessel caliber at 9 months postoperatively (Figure 2). Best-corrected visual acuity at that time was 20/200 in the left eye.
Discussion To our knowledge, this is the first case showing an association of Coats disease with Takayasu arteritis. Coats disease is unilateral in most cases and classically presents with yellow lipid-rich subretinal and intraretinal exudates and abnormalities in the retinal vessels, including telangiectasia, aneurysmal dilatations, and vascular tortuosity,1 as in our case. In later stages, the exudates can progress to a total retinal detachment.2 Untreated, the disease can lead to neovascular glaucoma and phthisis bulbi in advanced stages.1 The incidental finding of severe hypertension intraoperatively gave rise to a suspicion of the underlying Takayasu arteritis. Takayasu arteritis is characterized by attenuation of the aorta and its branches, as a result of autoimmune inflammation of the arteries,3 which was corroborated by
Journal of AAPOS
Volume 18 Number 6 / December 2014
Hildebrand and Painter
605
2. Rubin MP, Mukai S. Coats’ disease. Int Ophthalmol Clin 2008;48: 149-58. 3. Peter J, David S, Danda D, Peter JV, Horo S, Joseph G. Ocular manifestations of Takayasu arteritis: a cross sectional study. Retina 2011; 31:1170-78. 4. Uyama M, Asayma K. Retinal vascular changes in Takayasu’s arteritis (pulseless disease): occurrence and evolution of the lesion. Doc Ophthalmol Proc Series 1976;9:549-54. 5. Panja M, Mondal PC. Current status of aortoarteritis in India. J Assoc Physicians India 2004;52:48-52. 6. He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats’ disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthalmol 2010;248:1519-21. 7. Harada M, Yoshida H, Mitusyama K, et al. Aortitis syndrome (Takayasu’s arteritis) with cataract and elevated serum level of vascular endothelial growth factor. Scand J Rheumatol 1998;27:78-9.
FIG 2. Fundus photograph of the left eye showing resolution of most of the exudates and retinal edema postoperatively.
radiologic findings in our case. Reported ocular manifestations of Takayasu arteritis include Takayasu (hypoperfusive) retinopathy, which is characterized by distention of veins, microaneurysms, and formation of arteriovenous anastomoses.4 Takayasu arteritis can also manifest as hypertensive retinopathy or mixed retinopathy, that is, presence of both hypoperfusive and hypertensive retinopathy.5 A recent cross-sectional study reported that Takayasu retinopathy and hypertensive retinopathy were present in 14.6% and 16.4%, respectively, of Takayasu arteritis patients; 1.6% had mixed retinopathy.3 It is possible that in this case Takayasu arteritis was mimicking Coats disease. Although some of the ocular signs described for Takayasu arteritis such, as distension of veins and microaneurysms, were found in our case, Takayasu arteritis does not usually present with extensive retinal exudates and the majority of patients have bilateral disease.3 It seems more likely that this is a case of Coats disease with coexisting Takayasu arteritis, and that the underlying arteritis and inflammatory process may have exacerbated the clinical presentation of Coats disease. He and colleagues6 reported raised levels of vascular endothelial growth factor (VEGF) in eyes with Coats disease, and Harada and colleagues7 reported elevated level of VEGF in the serum of patients with Takayasu arteritis. It is difficult to accurately ascertain their combined role in the etiopathogenesis of the present case.
Literature Search PubMed was last searched on April 16, 2014, without date or language restrictions, using the following terms: Coats disease, Takayasu arteritis, Takayasu retinopathy, Coats disease AND Takayasu arteritis, Coats disease, AND Takayasu retinopathy. References 1. Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol 2001;131:561-71.
Journal of AAPOS
A simple technique for temporary eyelid closure in severe exposure keratopathy G€ oran Darius Hildebrand, FRCS, FRCOphth, and Sally L. Painter, FRCOphth We describe a novel and simple technique for temporary lid closure in severe bilateral corneal exposure keratopathy, where a rapid method for corneal protection is required. The aim is to create a stable dressing base for secure closure of the eyelids that also allows instillation of medication and examination of the eye.
C
orneal exposure may be prevented and treated with eyelid closure, which may be achieved through occlusive dressings, botulinum toxin induced ptosis, sutures with or without bolsters, and glue or surgical tarsorrhaphies.1-6 Simple taping techniques are often problematic because tape does not adhere well to oily skin; eyelids do not stay closed and there is a potential for rubbing of the tape against the ulcerated cornea. Dressing techniques that have been described for patients under general anesthesia are not appropriate for awake patients because they have the potential to remove eyelashes.7 Surgical intervention may neither be appropriate nor possible in an unwell patient.
Author affiliations: Department of Pediatric Ophthalmology, Royal Berkshire Hospital, Reading and King Edward VII Hospital, Windsor, United Kingdom Submitted April 9, 2014. Revision accepted July 7, 2014. Published online November 12, 2014. Correspondence: Mr. G€oran Darius Hildebrand, FRCS, FRCOphth, Department of Ophthalmology, Reading and King Edward VII Hospital, Windsor, UK (email: [email protected]). J AAPOS 2014;18:605-606. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.07.171
