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Letters to the Editor Table 1.
483
Reasons for referrals back to consultants
Verona E Botha MBChB, John Ah-Chan FRANZCO, Siok Khim Taylor B Optom and Peiyun Wang MBChB Ophthalmology Department, Palmerston North Hospital, Palmerston North, New Zealand
Consultant referrals (n = 101) Uncontrolled IOP No management change Management change Medication only YAG PI’s Cataracts Trabeculectomy Molteno tube Controlled IOP Cataracts AMD YAG PI’s VF anomalies Lid lesion Cryotherapy Vascular lesion Keratitis Posterior capsular opacification Rubeiosis Trabeculectomy bleb appearance Diabetic retinopathy Consideration of discharge due to advanced age and frailty Pt prefers different drop
Number
%
8 68 56 6 3 2 1 76
10.5 89.5 82.4 8.8 4.4 2.9 1.5 100
5 4 3 3 1 1 1 1 1 1 1 1 1
20 16 12 12 4 4 4 4 4 4 4 4 4
1 25
4 100
AMD, age-related macular degeneration; IOP, intraocular pressure; VF, visual field; YAG PI, YAG Peripheral iridotomies.
progression were more likely to be those with longer delays in follow up, as seen in figure 4. However, as chronic glaucoma is typically a slowly progressive disease, not all those with progression may have been detected in the relatively short study period. Eighty-eight per cent of patients referred back to consultants for review underwent a change in their management, suggesting that the referrals were highly appropriate. The optometrists also detected other ocular diseases requiring referral to consultant clinics. The development of the SGC has been of great benefit in Palmerston North by addressing the glaucoma follow-up waiting list problem, freeing up clinic appointments for consultant ophthalmologists to see new and/or more complex patients. The reduction in delay between visits has allowed earlier detection of non-compliance, uncontrolled IOP and progression of disease, allowing earlier institution of management changes to preserve vision. At this stage, we feel that it is appropriate for consultant ophthalmologists to see all new referrals for glaucoma and only follow-up patients are seen in the SGC. We are continually improving the mechanisms set in place to ensure the continued safe monitoring of the clinic based on the Royal Australasian and New Zealand College of Ophthalmologists (RANZCO) glaucoma special interest group guidelines.7
Received 17 November 2014; accepted 20 November 2014.
REFERENCES 1. Hodapp E, Parrish RK II, Anderson DR. Clinical Decisions in Glaucoma. St Louis, MO: The CV Mosby, 1993; 84–126. 2. Gray SF, Spry PGD, Brookes ST et al. The Bristol shared care glaucoma study: outcome at follow up at 2 years. Br J Ophthalmol 2000; 84: 456–63. 3. Ho S, Vernon SA. Decision making in chronic glaucoma – optometrists vs ophthalmologists in a shared care service. Ophthalmic Physiol Opt 2011; 31: 168–73. 4. Chawla A, Patel I, Yuen C, Fenerty C. Patterns of adherence to NICE glaucoma guidance in two different service delivery models. Eye 2012; 26: 1412–7. 5. Hourihan F, Mitchell P. Factors associated with use of glaucoma medications in a population of older people: the Blue Mountains Eye Study. Aust NZ J Ophthalmol 1999; 27: 176–9. 6. Newman-Casey PA, Weizer JS, Heisler M, Lee PP, Stein JD. Systematic review of educational interventions to improve glaucoma medication adherence. Semin Ophthalmol 2013; 28: 191–201. 7. Royal Australian and New Zealand College of Ophthalmologists [RANZCO]. Guidelines for Collaborative Care of Glaucoma Patients. 2013. Available from: http://www .ranzco.edu/images/documents/policies/Guidelines_for _collaborative_care_of_glaucoma_patients.pdf
Intralesional steroid for orbital manifestations of Rosai–Dorfman disease Rosai–Dorfman disease (RDD) is a rare histiocytic disorder of unknown aetiology. We describe a patient with an orbital mass, which showed histopathological features of sinus histiocytosis and responded well to intralesional steroids. A 51-year-old Caucasian male with a three-week history of discomfort and redness in his left eye was seen and initially diagnosed with episcleritis. He represented a week later with increasing redness and vertical diplopia. His only pre-existing medical condition was wellcontrolled type 1 diabetes. On examination, his vision was 6/6 in both eyes with full colour vision and no relative afferent pupillary defect. He had mild injection of his bulbar conjunctiva and 3 mm of left proptosis with mild Conflict of interest: No stated conflict of interest. Funding sources: This work was kindly funded by the Frost TFC Charitable Trust and the Royal College of Ophthalmologists Fellowship.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
484
Letters to the Editor
Figure 1. A coronal slice from a contrast enhanced computed tomography (CT) scan taken two days after presentation. It shows a cuff of tissue in the infero-lateral aspect of the left orbit with bony remodelling. The abnormal tissue extends to the left inferior orbital fissure, pterygopalatine fossa and left retro-maxillary area.
restriction of left down-gaze and abduction. The remainder of his ophthalmic examination was unremarkable. He was managed with an 8Δ base-down Fresnel Prism. A contrast-enhanced computerized tomography (CT) was carried out, demonstrating a moderately enhancing lesion in the left inferotemporal orbit, infiltrating both the inferior and lateral rectus muscles and extending to the orbital apex (Fig. 1). The radiological appearances were thought to represent either a neoplastic or vasculitic process. His systemic investigations were unremarkable, and all blood tests were within normal parameters. An incisional biopsy was performed, and histopathology revealed an inflammatory cell infiltrate with macrophages, neutrophils and lymphocytes. Areas of emperipolesis were noted and macrophages stained strongly positive for S100 protein and CD68. These findings are typical of RDD (Fig. 2).
a
b
c
d
Figure 2. (a) (×50) is a low power view showing the inflammatory cell infiltrates surrounding several, transversely and obliquely sectioned, nerve fascicles. (b) (×400) is a high power view of the infiltrate showing large macrophages with neutrophils, lymphocytes and apoptotic bodies within their cytoplasm (the phenomenon of emperipolesis, black arrow). The macrophages are highlighted by strong positive immunohistochemical staining for S100 protein (c) and CD68 (d). This is an unusual immunophenotype that is typical of the abnormal large macrophages seen in Rosai–Dorfman disease. © 2014 Royal Australian and New Zealand College of Ophthalmologists
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Letters to the Editor As the finding was isolated, no local or systemic treatment was initiated. Three weeks later, his colour vision dropped from 17 plates to 13 plates with subjective reddesaturation and a mild relative afferent pupillary defect, suggesting optic-nerve compression. An urgent CT scan was carried out, which did not indicate any significant change from the previous imaging. The risks and benefits of local and systemic treatment were discussed, and the patient opted for an orbital floor injection of 40 mg triamcinolone acetonide. This was carried out in a clean room without complication. An improvement in colour vision and red-desaturation was noted at two weeks and diplopia at four weeks. He has now been followed up for 54 weeks with no deterioration or signs of recurrence. We present the clinical and histopathological findings of a patient with an orbital mass and reduced vision. Based on initial imaging, the differential diagnosis included lymphoma, vasculitis and a granulomatous process. However, biopsy indicated that the lesion contained a mixed infiltrate of lymphocytes and mature plasma cells with phagocytic histiocytes, known as emperipolesis (typical of RDD). RDD is a rare, idiopathic, non-malignant histiocytic proliferative disorder and is usually self-limited; however, its course can be prolonged in some cases. It is relatively uncommon in the orbit with a prevalence of 0.03%–2.3%.1 There is a slight male preponderance, and the average age at onset is 20 years. RDD presents most commonly with bilateral, painless cervical-lymphadenopathy and raised inflammatory markers.2 Extra-nodal RDD occurs in 40% of cases and often presents in the head and neck.3 The most common ophthalmic sites are the orbit and eyelid occurring in 8.5% of cases. There have been previous reports of orbital involvement being the initial or principal manifestation of the disease and in approximately 20% of these there is no evidence of lymphadenopathy or leukocytosis (as was the case in our patient). The management of RDD is ideally conservative, as it is a self-limiting disease. However, due to uncontrolled symptoms or spread of the disease, 50% of patients receive treatment. This can be in the form of antibiotics, antituberculous treatment, immunomodulators, radiation or surgery, depending on the site(s) of disease. Our patient had type-1 diabetes and was not keen to start a course of oral steroids; furthermore, his focus of disease was in his posterior orbit, making further debulking surgery difficult and high risk. A decision was taken to treat him with intralesional 40 mg triamcinolone acetonide, via an orbital floor approach. There has been one published case of intralesional steroid being used in orbital RDD; however, the case had already been treated with oral steroids and significant debulking surgery. There have also been reports of intralesional and topical steroid being use in cutaneous RDD lesions to good effect.4,5 In conclusion, we describe an interesting case of RDD presenting as an orbital mass with optic nerve compression and responding well to one injection of intralesional
485 steroid. Furthermore, the patient remained stable for over a year with regular follow-up and has not required any additional treatment.
Harry Petrushkin FRCOphth, Jon Salisbury FRCPath and Eoin O’Sullivan FRCOphth Ophthalmology Department, Kings College Hospital, London, UK Received 17 November 2014; accepted 20 November 2014.
REFERENCES 1. Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol Oncol 2008; 1: 1–7. BioMed Central Ltd. 2. McClain KL, Natkunam Y, Swerdlow SH. Atypical cellular disorders. Hematology Am Soc Hematol Educ Program 2004; 2004: 283–96. American Society of Hematology. 3. Mehraein Y, Wagner M, Remberger K et al. Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 2006; 59: 1320–6. BMJ Publishing Group Ltd and Association of Clinical Pathologists. 4. Cooper SL, Chavis PS, Fortney JA, Watkins JM, Caplan MJ, Jenrette JM. A case of orbital Rosai-Dorfman disease responding to radiotherapy. J Pediatr Hematol Oncol 2008; 30: 744–8. 5. Satter EK, Graham BS, Steger JW. Response of cutaneous Rosai–Dorfman disease to topical and intralesional steroids. Br J Dermatol 2003; 149: 672–4.
Outcomes of chronic hypotony following trabeculectomy Trabeculectomy is the most commonly performed incisional glaucoma procedure worldwide.1 The increasing use of antimetabolites as an adjunct to trabeculectomy has led to chronic hypotony rates ranging up to 18%.2 Chronic hypotony (intraocular pressure [IOP] < 6 mmHg over three months postoperatively) may lead to vision loss from sequelae such as choroidal effusion, maculopathy, optic neuropathy and cataract development.3 It is often included in the definition of failure following glaucoma surgery, despite some eyes with chronic hypotony maintaining good visual acuity without the above-mentioned complications. Indeed, a recent report from Saeedi found 40% of eyes with chronic hypotony following glaucoma surgery did not have associated physical signs.4
Ethics approval: The Royal Australian and New Zealand College of Ophthalmologists Human Research Ethics Committee (Reference number 44.13) Conflict of interest: None Funding sources: None
© 2014 Royal Australian and New Zealand College of Ophthalmologists
Letters to the Editor Table 1.
483
Reasons for referrals back to consultants
Verona E Botha MBChB, John Ah-Chan FRANZCO, Siok Khim Taylor B Optom and Peiyun Wang MBChB Ophthalmology Department, Palmerston North Hospital, Palmerston North, New Zealand
Consultant referrals (n = 101) Uncontrolled IOP No management change Management change Medication only YAG PI’s Cataracts Trabeculectomy Molteno tube Controlled IOP Cataracts AMD YAG PI’s VF anomalies Lid lesion Cryotherapy Vascular lesion Keratitis Posterior capsular opacification Rubeiosis Trabeculectomy bleb appearance Diabetic retinopathy Consideration of discharge due to advanced age and frailty Pt prefers different drop
Number
%
8 68 56 6 3 2 1 76
10.5 89.5 82.4 8.8 4.4 2.9 1.5 100
5 4 3 3 1 1 1 1 1 1 1 1 1
20 16 12 12 4 4 4 4 4 4 4 4 4
1 25
4 100
AMD, age-related macular degeneration; IOP, intraocular pressure; VF, visual field; YAG PI, YAG Peripheral iridotomies.
progression were more likely to be those with longer delays in follow up, as seen in figure 4. However, as chronic glaucoma is typically a slowly progressive disease, not all those with progression may have been detected in the relatively short study period. Eighty-eight per cent of patients referred back to consultants for review underwent a change in their management, suggesting that the referrals were highly appropriate. The optometrists also detected other ocular diseases requiring referral to consultant clinics. The development of the SGC has been of great benefit in Palmerston North by addressing the glaucoma follow-up waiting list problem, freeing up clinic appointments for consultant ophthalmologists to see new and/or more complex patients. The reduction in delay between visits has allowed earlier detection of non-compliance, uncontrolled IOP and progression of disease, allowing earlier institution of management changes to preserve vision. At this stage, we feel that it is appropriate for consultant ophthalmologists to see all new referrals for glaucoma and only follow-up patients are seen in the SGC. We are continually improving the mechanisms set in place to ensure the continued safe monitoring of the clinic based on the Royal Australasian and New Zealand College of Ophthalmologists (RANZCO) glaucoma special interest group guidelines.7
Received 17 November 2014; accepted 20 November 2014.
REFERENCES 1. Hodapp E, Parrish RK II, Anderson DR. Clinical Decisions in Glaucoma. St Louis, MO: The CV Mosby, 1993; 84–126. 2. Gray SF, Spry PGD, Brookes ST et al. The Bristol shared care glaucoma study: outcome at follow up at 2 years. Br J Ophthalmol 2000; 84: 456–63. 3. Ho S, Vernon SA. Decision making in chronic glaucoma – optometrists vs ophthalmologists in a shared care service. Ophthalmic Physiol Opt 2011; 31: 168–73. 4. Chawla A, Patel I, Yuen C, Fenerty C. Patterns of adherence to NICE glaucoma guidance in two different service delivery models. Eye 2012; 26: 1412–7. 5. Hourihan F, Mitchell P. Factors associated with use of glaucoma medications in a population of older people: the Blue Mountains Eye Study. Aust NZ J Ophthalmol 1999; 27: 176–9. 6. Newman-Casey PA, Weizer JS, Heisler M, Lee PP, Stein JD. Systematic review of educational interventions to improve glaucoma medication adherence. Semin Ophthalmol 2013; 28: 191–201. 7. Royal Australian and New Zealand College of Ophthalmologists [RANZCO]. Guidelines for Collaborative Care of Glaucoma Patients. 2013. Available from: http://www .ranzco.edu/images/documents/policies/Guidelines_for _collaborative_care_of_glaucoma_patients.pdf
Intralesional steroid for orbital manifestations of Rosai–Dorfman disease Rosai–Dorfman disease (RDD) is a rare histiocytic disorder of unknown aetiology. We describe a patient with an orbital mass, which showed histopathological features of sinus histiocytosis and responded well to intralesional steroids. A 51-year-old Caucasian male with a three-week history of discomfort and redness in his left eye was seen and initially diagnosed with episcleritis. He represented a week later with increasing redness and vertical diplopia. His only pre-existing medical condition was wellcontrolled type 1 diabetes. On examination, his vision was 6/6 in both eyes with full colour vision and no relative afferent pupillary defect. He had mild injection of his bulbar conjunctiva and 3 mm of left proptosis with mild Conflict of interest: No stated conflict of interest. Funding sources: This work was kindly funded by the Frost TFC Charitable Trust and the Royal College of Ophthalmologists Fellowship.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
484
Letters to the Editor
Figure 1. A coronal slice from a contrast enhanced computed tomography (CT) scan taken two days after presentation. It shows a cuff of tissue in the infero-lateral aspect of the left orbit with bony remodelling. The abnormal tissue extends to the left inferior orbital fissure, pterygopalatine fossa and left retro-maxillary area.
restriction of left down-gaze and abduction. The remainder of his ophthalmic examination was unremarkable. He was managed with an 8Δ base-down Fresnel Prism. A contrast-enhanced computerized tomography (CT) was carried out, demonstrating a moderately enhancing lesion in the left inferotemporal orbit, infiltrating both the inferior and lateral rectus muscles and extending to the orbital apex (Fig. 1). The radiological appearances were thought to represent either a neoplastic or vasculitic process. His systemic investigations were unremarkable, and all blood tests were within normal parameters. An incisional biopsy was performed, and histopathology revealed an inflammatory cell infiltrate with macrophages, neutrophils and lymphocytes. Areas of emperipolesis were noted and macrophages stained strongly positive for S100 protein and CD68. These findings are typical of RDD (Fig. 2).
a
b
c
d
Figure 2. (a) (×50) is a low power view showing the inflammatory cell infiltrates surrounding several, transversely and obliquely sectioned, nerve fascicles. (b) (×400) is a high power view of the infiltrate showing large macrophages with neutrophils, lymphocytes and apoptotic bodies within their cytoplasm (the phenomenon of emperipolesis, black arrow). The macrophages are highlighted by strong positive immunohistochemical staining for S100 protein (c) and CD68 (d). This is an unusual immunophenotype that is typical of the abnormal large macrophages seen in Rosai–Dorfman disease. © 2014 Royal Australian and New Zealand College of Ophthalmologists
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Letters to the Editor As the finding was isolated, no local or systemic treatment was initiated. Three weeks later, his colour vision dropped from 17 plates to 13 plates with subjective reddesaturation and a mild relative afferent pupillary defect, suggesting optic-nerve compression. An urgent CT scan was carried out, which did not indicate any significant change from the previous imaging. The risks and benefits of local and systemic treatment were discussed, and the patient opted for an orbital floor injection of 40 mg triamcinolone acetonide. This was carried out in a clean room without complication. An improvement in colour vision and red-desaturation was noted at two weeks and diplopia at four weeks. He has now been followed up for 54 weeks with no deterioration or signs of recurrence. We present the clinical and histopathological findings of a patient with an orbital mass and reduced vision. Based on initial imaging, the differential diagnosis included lymphoma, vasculitis and a granulomatous process. However, biopsy indicated that the lesion contained a mixed infiltrate of lymphocytes and mature plasma cells with phagocytic histiocytes, known as emperipolesis (typical of RDD). RDD is a rare, idiopathic, non-malignant histiocytic proliferative disorder and is usually self-limited; however, its course can be prolonged in some cases. It is relatively uncommon in the orbit with a prevalence of 0.03%–2.3%.1 There is a slight male preponderance, and the average age at onset is 20 years. RDD presents most commonly with bilateral, painless cervical-lymphadenopathy and raised inflammatory markers.2 Extra-nodal RDD occurs in 40% of cases and often presents in the head and neck.3 The most common ophthalmic sites are the orbit and eyelid occurring in 8.5% of cases. There have been previous reports of orbital involvement being the initial or principal manifestation of the disease and in approximately 20% of these there is no evidence of lymphadenopathy or leukocytosis (as was the case in our patient). The management of RDD is ideally conservative, as it is a self-limiting disease. However, due to uncontrolled symptoms or spread of the disease, 50% of patients receive treatment. This can be in the form of antibiotics, antituberculous treatment, immunomodulators, radiation or surgery, depending on the site(s) of disease. Our patient had type-1 diabetes and was not keen to start a course of oral steroids; furthermore, his focus of disease was in his posterior orbit, making further debulking surgery difficult and high risk. A decision was taken to treat him with intralesional 40 mg triamcinolone acetonide, via an orbital floor approach. There has been one published case of intralesional steroid being used in orbital RDD; however, the case had already been treated with oral steroids and significant debulking surgery. There have also been reports of intralesional and topical steroid being use in cutaneous RDD lesions to good effect.4,5 In conclusion, we describe an interesting case of RDD presenting as an orbital mass with optic nerve compression and responding well to one injection of intralesional
485 steroid. Furthermore, the patient remained stable for over a year with regular follow-up and has not required any additional treatment.
Harry Petrushkin FRCOphth, Jon Salisbury FRCPath and Eoin O’Sullivan FRCOphth Ophthalmology Department, Kings College Hospital, London, UK Received 17 November 2014; accepted 20 November 2014.
REFERENCES 1. Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol Oncol 2008; 1: 1–7. BioMed Central Ltd. 2. McClain KL, Natkunam Y, Swerdlow SH. Atypical cellular disorders. Hematology Am Soc Hematol Educ Program 2004; 2004: 283–96. American Society of Hematology. 3. Mehraein Y, Wagner M, Remberger K et al. Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 2006; 59: 1320–6. BMJ Publishing Group Ltd and Association of Clinical Pathologists. 4. Cooper SL, Chavis PS, Fortney JA, Watkins JM, Caplan MJ, Jenrette JM. A case of orbital Rosai-Dorfman disease responding to radiotherapy. J Pediatr Hematol Oncol 2008; 30: 744–8. 5. Satter EK, Graham BS, Steger JW. Response of cutaneous Rosai–Dorfman disease to topical and intralesional steroids. Br J Dermatol 2003; 149: 672–4.
Outcomes of chronic hypotony following trabeculectomy Trabeculectomy is the most commonly performed incisional glaucoma procedure worldwide.1 The increasing use of antimetabolites as an adjunct to trabeculectomy has led to chronic hypotony rates ranging up to 18%.2 Chronic hypotony (intraocular pressure [IOP] < 6 mmHg over three months postoperatively) may lead to vision loss from sequelae such as choroidal effusion, maculopathy, optic neuropathy and cataract development.3 It is often included in the definition of failure following glaucoma surgery, despite some eyes with chronic hypotony maintaining good visual acuity without the above-mentioned complications. Indeed, a recent report from Saeedi found 40% of eyes with chronic hypotony following glaucoma surgery did not have associated physical signs.4
Ethics approval: The Royal Australian and New Zealand College of Ophthalmologists Human Research Ethics Committee (Reference number 44.13) Conflict of interest: None Funding sources: None
© 2014 Royal Australian and New Zealand College of Ophthalmologists
