C L I N I C A L
A N D
E X P E R I M E N T A L
OPTOMETRY CLINICAL COMMUNICATION
Occlusive retinal vasculitis following routine cataract surgerya Clin Exp Optom 2014; 97: 568–569
Y Eve Hsing MBBS BAppSc (Optom) Hons Joseph Park MBBS FRANZCO Department of Ophthalmology, University of Queensland, Royal Brisbane and Women’s Hospital, Brisbane, Australia E-mail: [email protected]
Submitted: 2 February 2014 Revised: 14 April 2014 Accepted for publication: 24 April 2014 Cataract surgery is one of the most commonly performed surgical procedures performed worldwide with high success rates. Suboptimal vision after routine cataract surgery may occur due to cystoid macular oedema, uveitis, posterior capsular opacification, retinal detachment and endophthalmitis.1 We present a case of occlusive retinal vasculitis occurring soon after uncomplicated cataract surgery, an association which has not previously been reported.
CASE HISTORY A 65-year-old Caucasian woman presented one week after uneventful right cataract surgery with painless blurred vision in her right eye. She had been compliant with postoperative topical ciprofloxacin 0.3 per cent and prednisolone acetate 1.0 per cent/ phenylephrine hydrochloride 0.12 per cent. Visual acuity had decreased from 6/7.5 on day 1 post-surgery to 6/12. A right relative afferent pupil defect was noted, which had not been present pre-operatively. Intraocular pressure and anterior segment examination were normal except for 1/2+ anterior chamber cells. A large area of retinal periarteritis and periphlebitis was seen ophthalmoscopically, extending from the mid-peripheral fundus toward and reaching the macular region. There was evidence of
a Part of this manuscript was presented as a poster at the RANZCO 45th Annual Scientific Congress in Hobart, Nov 2-6, 2013
DOI:10.1111/cxo.12181
patchy pallor temporal to the right fovea (Figure 1A). Fundus fluorescein angiography (FFA) showed arteriolar and venular leakage, as well as peripheral capillary nonperfusion (Figure 1B). The patient did not have any notable previous ocular history to suggest sympathetic ophthalmia. The left eye was unaffected. Her medical history was significant only for hypothyroidism and systemic review did not elicit any underlying infective, rheumatological or autoimmune symptoms. After aqueous and vitreous samples were obtained for polymerase chain reaction, analysis of herpes simplex virus 1 and 2, varicella zoster virus, Epstein-Barr virus and cytomegalovirus (none detected), the patient was admitted to hospital to receive intravenous aciclovir for possible early acute retinal necrosis (ARN). A range of other investigations was performed to exclude compromised immunity, infective causes and autoimmune conditions. Serology for hepatitis B and C, HIV, varicella zoster virus, herpes simplex 1 and 2 viruses, cytomegalovirus, Epstein-Barr virus, toxoplasmosis and syphilis were negative. Tuberculosis was not detected on Quantiferon TB Gold assay. Inflammatory markers, namely erythrocyte sedimentation rate and C-creative protein, were five mm/hour and less than two mg/dl, respectively (within normal limits). Nonspecific anti-nuclear autoantibody titre of 1:160 was mildly raised but the absence of anti-nuclear antibodies and extractable nuclear antigens ruled out systemic lupus erythematosus. Angiotensin converting enzyme, serum calcium (sarcoidosis), human leukocyte antigen (HLA)-B51 (Behçet’s disease) and anti-neutrophil cytoplasmic antibody (granulomatosis with polyangiitis, formerly Wegner’s granulomatosis) were all within normal limits. Chest X-ray further dispelled diagnoses of tuberculosis and sarcoidosis. Urine analysis did not reveal evidence of renal vasculitis. Despite three days of systemic anti-viral treatment, the vasculitis progressed with
Clinical and Experimental Optometry 97.6 November 2014
568
increasing ischaemia and development of fluid at the macula (Figure 1C). Vision reduced to counting fingers. A diagnosis of isolated occlusive retinal vasculitis was made. In consultation with the rheumatologist, three days of high-dose intravenous corticosteroids followed by maintenance oral steroids were administered. Three cycles of intravenous cyclophosphamide were also given. Vision at nine months remained stable at 6/9 and there was significant improvement of the parafoveal ischaemia, occlusive vasculitis (Figure 2A) and macular oedema (Figure 2B) on maintenance methotrexate and a weaning oral prednisone dose. Repeat fluorescein angiography excluded retinal neovascularisation but showed persistent peripheral capillary non-perfusion superotemporally. Thus, photocoagulation laser therapy was performed to prevent neovascularisation and associated vitreous haemorrhage (Figure 2A). DISCUSSION This paper highlights an unusual case of occlusive vasculitis of both the retinal arterioles and venules developing within one week after routine cataract surgery. Retinal vasculitis has previously been seen after pars plana vitrectomy for removing retained lens fragments2 and as an early sign in post-operative endophthalmitis;3 however, these were unlikely in this case as the posterior capsule was intact and the eye remained quiet. It is possible the intraocular inflammation may have been masked by use of topical steroids at presentation. Therefore, a broad spectrum of differential diagnoses had to be excluded. Retinal vasculitis has been associated with ocular infections and inflammatory syndromes, non-vasculitic systemic inflammatory disease, underlying systemic vasculitis, acute leukaemia and malignancy-related retinopathy.4 Acute retinal necrosis was the provisional diagnosis and initial treatment © 2014 The Authors
Clinical and Experimental Optometry © 2014 Optometrists Association Australia
Isolated occlusive retinal vasculitis Hsing and Park
ocular inflammation or vasculitis. There may be a causal relationship between the inflammatory condition and cataract surgery, which would warrant further investigation. It should be noted that despite a lack of infective source found on extensive investigation, this remains potentially a case of infective retinal vasculitis, which has been treated, at least partially, by both empirical systemic antiviral therapy and topical antibiotics. In this patient, an excellent recovery of vision was achieved and maintained with long-term immunosuppressive treatment and prophylactic sector photocoagulation to ischaemic areas of retina. REFERENCES
Figure 2. After nine months immunosuppressive treatment A) Resolution of vasculitis; sector panretinal photocoagulation performed superotemporally B) Optical coherence tomograph confirming resolution of macular thickening
Figure 1. Before immunosuppressive treatment A) Periarteritis and periphlebitis with area of ischaemic pallor temporal to right fovea B) Fluorescein angiogram highlighting widespread capillary non-perfusion C) Optical coherence tomograph showing fluid at the right macula
was empirical. When laboratory testing failed to show a herpetic cause, management for a likely inflammatory aetiology was indicated to prevent severe and permanent retinal damage and risk of blindness.
1. Kanski JJ, Bowling B. Lens: Management of agerelated cataract. In: Clinical Ophthalmology: A Systemic Approach, 7th ed. Edinburgh: Elsevier, 2011. 2. Örnek K, Onaran Z, Yimazbas P. Retinal vasculitis following pars plana vitrectomy for retained lens material. Clin Exp Optom 2011; 94: 106–107. 3. Subbiah S, McAvoy CE, Best JL. Retinal vasculitis as an early sign of bacterial post-operative endophthalmitis. Eye (Lond) 2010; 24: 1411–1420. 4. Rosenbaum JT, Ku J, Ali A, Choi D, Suhler EB. Patients with retinal vasculitis rarely suffer from systemic vasculitis. Semin Arthritis Rheum 2012; 41: 859– 865. 5. Bisighini SL, Pagliuso LM. Retinal vasculitis: a diagnostic dilemma. Clin Eye Vis Care 1997; 9: 71–84. 6. Cronin KM, Manning RA, Richards CP, Rothova A, Tabbara KF. Diagnostic and therapeutic challenges. Retina 2012; 32: 1428–1434.
The diagnosis of isolated occlusive retinal vasculitis was made after clinically excluding infective and specific systemic and ocular inflammatory conditions, as supported by negative laboratory results.5 Systemic treatment was instigated as vision became threatened;6 however, immunosuppressive therapy, preferably steroid-sparing to minimise the numerous adverse effects secondary to prolonged high-dose steroid use, should not be started until an infective aetiology has been satisfactorily ruled out to avoid exacerbation of infection.6 Additionally, the patient must be closely monitored for relapse with titration of maintenance therapy in conjunction with the rheumatologist. This is the first reported case of isolated occlusive retinal vasculitis developing soon after uncomplicated cataract surgery in a previously normal eye with no evidence of
© 2014 The Authors Clinical and Experimental Optometry © 2014 Optometrists Association Australia
Clinical and Experimental Optometry 97.6 November 2014
569
Copyright of Clinical & Experimental Optometry is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
A N D
E X P E R I M E N T A L
OPTOMETRY CLINICAL COMMUNICATION
Occlusive retinal vasculitis following routine cataract surgerya Clin Exp Optom 2014; 97: 568–569
Y Eve Hsing MBBS BAppSc (Optom) Hons Joseph Park MBBS FRANZCO Department of Ophthalmology, University of Queensland, Royal Brisbane and Women’s Hospital, Brisbane, Australia E-mail: [email protected]
Submitted: 2 February 2014 Revised: 14 April 2014 Accepted for publication: 24 April 2014 Cataract surgery is one of the most commonly performed surgical procedures performed worldwide with high success rates. Suboptimal vision after routine cataract surgery may occur due to cystoid macular oedema, uveitis, posterior capsular opacification, retinal detachment and endophthalmitis.1 We present a case of occlusive retinal vasculitis occurring soon after uncomplicated cataract surgery, an association which has not previously been reported.
CASE HISTORY A 65-year-old Caucasian woman presented one week after uneventful right cataract surgery with painless blurred vision in her right eye. She had been compliant with postoperative topical ciprofloxacin 0.3 per cent and prednisolone acetate 1.0 per cent/ phenylephrine hydrochloride 0.12 per cent. Visual acuity had decreased from 6/7.5 on day 1 post-surgery to 6/12. A right relative afferent pupil defect was noted, which had not been present pre-operatively. Intraocular pressure and anterior segment examination were normal except for 1/2+ anterior chamber cells. A large area of retinal periarteritis and periphlebitis was seen ophthalmoscopically, extending from the mid-peripheral fundus toward and reaching the macular region. There was evidence of
a Part of this manuscript was presented as a poster at the RANZCO 45th Annual Scientific Congress in Hobart, Nov 2-6, 2013
DOI:10.1111/cxo.12181
patchy pallor temporal to the right fovea (Figure 1A). Fundus fluorescein angiography (FFA) showed arteriolar and venular leakage, as well as peripheral capillary nonperfusion (Figure 1B). The patient did not have any notable previous ocular history to suggest sympathetic ophthalmia. The left eye was unaffected. Her medical history was significant only for hypothyroidism and systemic review did not elicit any underlying infective, rheumatological or autoimmune symptoms. After aqueous and vitreous samples were obtained for polymerase chain reaction, analysis of herpes simplex virus 1 and 2, varicella zoster virus, Epstein-Barr virus and cytomegalovirus (none detected), the patient was admitted to hospital to receive intravenous aciclovir for possible early acute retinal necrosis (ARN). A range of other investigations was performed to exclude compromised immunity, infective causes and autoimmune conditions. Serology for hepatitis B and C, HIV, varicella zoster virus, herpes simplex 1 and 2 viruses, cytomegalovirus, Epstein-Barr virus, toxoplasmosis and syphilis were negative. Tuberculosis was not detected on Quantiferon TB Gold assay. Inflammatory markers, namely erythrocyte sedimentation rate and C-creative protein, were five mm/hour and less than two mg/dl, respectively (within normal limits). Nonspecific anti-nuclear autoantibody titre of 1:160 was mildly raised but the absence of anti-nuclear antibodies and extractable nuclear antigens ruled out systemic lupus erythematosus. Angiotensin converting enzyme, serum calcium (sarcoidosis), human leukocyte antigen (HLA)-B51 (Behçet’s disease) and anti-neutrophil cytoplasmic antibody (granulomatosis with polyangiitis, formerly Wegner’s granulomatosis) were all within normal limits. Chest X-ray further dispelled diagnoses of tuberculosis and sarcoidosis. Urine analysis did not reveal evidence of renal vasculitis. Despite three days of systemic anti-viral treatment, the vasculitis progressed with
Clinical and Experimental Optometry 97.6 November 2014
568
increasing ischaemia and development of fluid at the macula (Figure 1C). Vision reduced to counting fingers. A diagnosis of isolated occlusive retinal vasculitis was made. In consultation with the rheumatologist, three days of high-dose intravenous corticosteroids followed by maintenance oral steroids were administered. Three cycles of intravenous cyclophosphamide were also given. Vision at nine months remained stable at 6/9 and there was significant improvement of the parafoveal ischaemia, occlusive vasculitis (Figure 2A) and macular oedema (Figure 2B) on maintenance methotrexate and a weaning oral prednisone dose. Repeat fluorescein angiography excluded retinal neovascularisation but showed persistent peripheral capillary non-perfusion superotemporally. Thus, photocoagulation laser therapy was performed to prevent neovascularisation and associated vitreous haemorrhage (Figure 2A). DISCUSSION This paper highlights an unusual case of occlusive vasculitis of both the retinal arterioles and venules developing within one week after routine cataract surgery. Retinal vasculitis has previously been seen after pars plana vitrectomy for removing retained lens fragments2 and as an early sign in post-operative endophthalmitis;3 however, these were unlikely in this case as the posterior capsule was intact and the eye remained quiet. It is possible the intraocular inflammation may have been masked by use of topical steroids at presentation. Therefore, a broad spectrum of differential diagnoses had to be excluded. Retinal vasculitis has been associated with ocular infections and inflammatory syndromes, non-vasculitic systemic inflammatory disease, underlying systemic vasculitis, acute leukaemia and malignancy-related retinopathy.4 Acute retinal necrosis was the provisional diagnosis and initial treatment © 2014 The Authors
Clinical and Experimental Optometry © 2014 Optometrists Association Australia
Isolated occlusive retinal vasculitis Hsing and Park
ocular inflammation or vasculitis. There may be a causal relationship between the inflammatory condition and cataract surgery, which would warrant further investigation. It should be noted that despite a lack of infective source found on extensive investigation, this remains potentially a case of infective retinal vasculitis, which has been treated, at least partially, by both empirical systemic antiviral therapy and topical antibiotics. In this patient, an excellent recovery of vision was achieved and maintained with long-term immunosuppressive treatment and prophylactic sector photocoagulation to ischaemic areas of retina. REFERENCES
Figure 2. After nine months immunosuppressive treatment A) Resolution of vasculitis; sector panretinal photocoagulation performed superotemporally B) Optical coherence tomograph confirming resolution of macular thickening
Figure 1. Before immunosuppressive treatment A) Periarteritis and periphlebitis with area of ischaemic pallor temporal to right fovea B) Fluorescein angiogram highlighting widespread capillary non-perfusion C) Optical coherence tomograph showing fluid at the right macula
was empirical. When laboratory testing failed to show a herpetic cause, management for a likely inflammatory aetiology was indicated to prevent severe and permanent retinal damage and risk of blindness.
1. Kanski JJ, Bowling B. Lens: Management of agerelated cataract. In: Clinical Ophthalmology: A Systemic Approach, 7th ed. Edinburgh: Elsevier, 2011. 2. Örnek K, Onaran Z, Yimazbas P. Retinal vasculitis following pars plana vitrectomy for retained lens material. Clin Exp Optom 2011; 94: 106–107. 3. Subbiah S, McAvoy CE, Best JL. Retinal vasculitis as an early sign of bacterial post-operative endophthalmitis. Eye (Lond) 2010; 24: 1411–1420. 4. Rosenbaum JT, Ku J, Ali A, Choi D, Suhler EB. Patients with retinal vasculitis rarely suffer from systemic vasculitis. Semin Arthritis Rheum 2012; 41: 859– 865. 5. Bisighini SL, Pagliuso LM. Retinal vasculitis: a diagnostic dilemma. Clin Eye Vis Care 1997; 9: 71–84. 6. Cronin KM, Manning RA, Richards CP, Rothova A, Tabbara KF. Diagnostic and therapeutic challenges. Retina 2012; 32: 1428–1434.
The diagnosis of isolated occlusive retinal vasculitis was made after clinically excluding infective and specific systemic and ocular inflammatory conditions, as supported by negative laboratory results.5 Systemic treatment was instigated as vision became threatened;6 however, immunosuppressive therapy, preferably steroid-sparing to minimise the numerous adverse effects secondary to prolonged high-dose steroid use, should not be started until an infective aetiology has been satisfactorily ruled out to avoid exacerbation of infection.6 Additionally, the patient must be closely monitored for relapse with titration of maintenance therapy in conjunction with the rheumatologist. This is the first reported case of isolated occlusive retinal vasculitis developing soon after uncomplicated cataract surgery in a previously normal eye with no evidence of
© 2014 The Authors Clinical and Experimental Optometry © 2014 Optometrists Association Australia
Clinical and Experimental Optometry 97.6 November 2014
569
Copyright of Clinical & Experimental Optometry is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
