- Email: [email protected]
Tonic pupil, anterior ischemic optic neuropathy in a teenager with Takayasu arteritis
Correspondence not seen despite examination of multiple tumour sections. We believe the patient’s heavy smoking and immunosuppression caused by cancer and chronic chemotherapy predisposed him to development of invasive SCC.5 Despite the absence of established risk factors for intraocular invasion by SCC, the patient’s clinical presentation was that of vision loss resulting from intraocular tumour involvement. Our case highlights the importance of meticulous examination of the anterior segment, including the bulbar conjunctiva, in patients with visual loss from a serous retinal detachment. Epibulbar conjunctival SCC in the appropriate clinical setting can present in this manner.
Fig. 3 — Keratinizing squamous cell carcinoma arising from the perilimbal conjunctiva and invading underlying sclera (hematoxylin and eosin stain; original magnification 132; arrows indicate infiltrative nests).
of the tumour were well-differentiated with keratin formation, whereas other regions showed moderate differentiation, in contrast with the patient’s poorly differentiated oropharyngeal recurrence. Periodic acid–Schiff and mucicarmine stains were negative for mucin material. The final diagnosis was SCC, moderately to well-differentiated, involving the conjunctiva, lateral rectus muscle, sclera, and uveal tract with the growth pattern and degree of differentiation favoring the diagnosis of a second primary tumour and not a metastasis. Conjunctival SCC usually occurs in older male smokers with a history of sun and dust exposure.2 It tends to be superficially invasive; intraocular invasion rarely occurs in patients having undergone multiple surgical resections, cryotherapy, topical chemotherapy, and/or radiation.4 The mucoepidermoid and spindle cell variants are more locally aggressive and have higher recurrence rates.4 In this case, the patient had not received prior ocular treatments, and aggressive histologic SCC variants were
Tonic pupil, anterior ischemic optic neuropathy in a teenager with Takayasu arteritis This case report describes a 16-year-old female with a unilaterally tonic pupil and contralateral anterior ischemic optic neuropathy (AION) as the ocular features of Takayasu arteritis. A 16-year-old female reported sudden blurring of vision in the left eye. Systemic examination showed the presence of left hemiparesis along with nonpalpable upper limb pulses and weak left carotid pulsations. Magnetic resonance imaging of the brain (Fig. 1A) demonstrated an acute infarct in
Shaheen C. Kavoussi, * Debra J. Shetlar, † Andrew J. Barkmeier, ‡ Petros E. Carvounis §
* Yale University, New Haven, Conn.; †Michael E. DeBakey Veteran Affairs Medical Center, Houston, Tex.; ‡Mayo Clinic, Rochester, Minn.; §Cullen Eye Institute, Baylor College of Medicine, Houston, Tex.
Correspondence to: Petros E. Carvounis, MD: [email protected]
REFERENCES 1. Lee GA, Hirst LW. Retrospective study of ocular surface squamous neoplasia. Aust N Z J Ophthalmol. 1997;25:269-76. 2. Hamam R, Bhat P, Foster CS. Conjunctival/corneal intraepithelial neoplasia. Int Ophthalmol Clin. 2009;49:63-70. 3. Tabbara KF, Kersten R, Daouk N, Blodi FC. Metastatic squamous cell carcinoma of the conjunctiva. Ophthalmology. 1988;95:318-21. 4. Cohen BH, Green WR, Iliff NT, et al. Spindle cell carcinoma of the conjunctiva. Arch Ophthalmol. 1980;98:1809-13. 5. Shelil AE, Shields CL, Shields JA, Eagle RC. Aggressive conjunctival squamous cell carcinoma in a patient following liver transplantation. Arch Ophthalmol. 2003;121:280-2. Can J Ophthalmol 2013;48:e158–e159 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.08.012
the right periventricular frontoparietal lobe (the watershed territory of right middle and anterior cerebral artery). Magnetic resonance angiography (MRA) demonstrated complete occlusion of the origins of the right brachiocephalic artery along with its branches and left subclavian artery. There was near total occlusion of the left common carotid artery (Fig. 1B) in keeping with type IIa Takayasu arteritis.1 The best corrected visual acuity was measured as 6/6 in the right eye and 6/9 in the left eye. Examination of the pupil revealed anisocoria, with the right pupil measuring 5 mm and left pupil 2 mm in light. The right pupil was poorly reactive to light, and there was a relative afferent pupillary defect in the left eye that manifested with a
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e159
Correspondence
Fig. 1A — Magnetic resonance imaging brain diffusionweighted (A) and apparent diffusion coefficient map (B) images of the brain showing acute restricted diffusion (white arrow) in the right frontoparietal lobe. 1B, Magnetic resonance angiography of carotid vessels showing complete occlusion of right carotid and near-complete occlusion of the left common carotid artery (black arrow).
swinging flashlight test. The right pupillary reaction to a near fixation target was tonic, exhibiting light near dissociation. The right pupil constricted to 0.125% topical pilocarpine, thereby showing evidence of denervation supersensitivity. Vermiform movements of the right pupil with sectoral tonic iris constrictions were visible with a slitlamp examination, which were not present in the left pupil. The intraocular pressures were measured by Goldmann applanation tonometry as 8 and 11 mm Hg in the right and left eye, respectively. Anterior segment examination was normal in both eyes. A dilated examination of the fundus showed a normal disc and macula in the right eye, whereas the left eye revealed the presence of pallid disc edema with venous tortuosity (Fig. 2). Visual field testing by Humphrey visual field analyzer 30-2 was normal in the right eye. The left eye visual field showed a superior altitudinal field defect extending to the inferonasal quadrant (Fig. 3A) with a mean deviation of –23.84 dB. Based on the earlier findings, the patient was diagnosed with tonic pupil in the right eye and AION in the left eye with type IIa Takayasu arteritis. Thereafter she underwent bilateral carotid angioplasty with stenting. Ocular examination repeated after 3 months showed persistence of the tonic pupil and relative afferent pupillary defect with normal disc in the right eye and optic atrophy in the left eye with resolution of the optic disc edema (Fig. 4). Visual field testing showed marginal improvement in the left eye fields with the mean deviation changing from –23.84 to –14.79 dB (Fig. 3B). Takayasu arteritis is a nonspecific aortoarteritis characterized by granulomatous inflammation of the large arteries first described by Takayasu in 1908. The inflammation is pan-arteritic with proliferation of tunica intima, destruction of elastic tunica media, followed by fibrosis of the adventitia. The ischemic features due to vessel thrombosis and occlusion can range from transient ischemic attacks, stroke, mesenteric ischemia, renovascular hypertension, myocarditis to cardiac failure.2 It is more common in females, and the majority of patients present before the age of 40 years. Children usually have type I Takayasu arteritis with fever, arthralgias, and cardiac involvement simulating rheumatic fever or with
Fig. 2 — Fundus photograph of both eyes showing normal disc in the right eye and pallid disc edema in the left eye.
e160
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Correspondence
Fig. 3 — A, Humphrey visual field report 30-2 program with Swedish Interactive Threshold Algorithm ( SITA) standard of the left eye showing a superior altitudinal scotoma. B, Humphrey visual field report 30-2 program with SITA standard of the left eye showing slight recovery.
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e161
Correspondence
Fig. 3
e162
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Correspondence
Fig. 4 — Fundus photograph of the left eye showing optic atrophy.
features of hypertension because of renal vessel involvement (type IV).3 The ocular presentations in Takayasu arteritis may be variable and depend on the branches of the carotid artery involved, duration, and rate of vascular insufficiency and development of collateral blood supply. Pathogenesis of these manifestations is either hypoperfusion after occlusion of the branches of the carotid or hypertension secondary to renal artery involvement.4 The most common ocular feature of hypoperfusion in the carotid arteries is Takayasu retinopathy,4 followed by ocular ischemic syndrome leading to neovascular glaucoma, vitreous hemorrhage, and tractional retinal detachment. Hypertensive retinopathy and bilateral papilledema are less common and might occur because of renal artery stenosis.5 AION in Takayasu arteritis has been reported in few adult cases.6,7 Ocular features are rare in children, and the occurrence of AION has not yet been reported in children with Takayasu arteritis (search MEDLINE database). Hypoperfusion in the internal carotid vessels results in nonarteritic AION, whereas thrombotic narrowing of the posterior ciliary arteries can cause the arteritic type of AION.8 In our case, the acute-onset disc edema with pallor may be an arteritic form of AION that appears to be secondary to the near-total occlusion of the left internal carotid artery. Pallid disc edema is described as chalky white swelling of the optic disc in 68.7% of cases with arteritic AION,9 mostly caused by giant cell arteritis (GCA). However, in the younger age group, presence of pallid disc edema should arouse suspicion of other causes of vasculitis such as Takayasu arteritis because GCA is uncommon at this age. Tonic pupils caused by ischemia have been reported in GCA10 and resolving internal carotid artery occlusion.11 The mechanism by which these entities produce tonic pupils is not well-known. Ischemia of the ciliary ganglion
and postganglionic parasympathetic fibres,10,12 ischemia of the iris sphincter itself,12 or damage to the ciliary ganglion mediated by immunologic mechanisms13 are proposed as the mechanisms in patients with GCA. A literature search (MEDLINE database) did not show any reports of tonic pupil in Takayasu arteritis. The complete occlusion of right internal carotid artery as evidenced by the MRA may have resulted in the reduced orbital blood flow to the ciliary ganglion and postganglionic ciliary nerves resulting in ischemia and a tonic pupil. This case highlights the hitherto unreported features of tonic pupil and AION in a teenager with Takayasu arteritis. We suggest that the presence of features of ocular ischemia in children should raise the suspicion of an underlying age-specific systemic vasculitis like Takayasu disease. Jyoti Matalia, Nirupama Kasturi, Hemant D. Anaspure, Bhujang K. Shetty Narayana Nethralaya, Bangalore, India Correspondence to: Jyoti Matalia, DNB: [email protected]
REFERENCES 1. Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestation of Takayasu arteritis in India and Japan—new classification of angiographic findings. Angiology. 1997;48:369-79. 2. Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol. 2002;55:481-6. 3. Cakar N, Yalcinkaya F, Duzova A, et al. Takayasu arteritis in children. J Rheumatol. 2008;35:913-9. 4. Peter J, David S, Joseph G, Horo S, Danda D. Hypoperfusive and hypertensive ocular manifestations in Takayasu arteritis. Clin Ophthalmol. 2010;4:1173-6. 5. Chun YS, Park SJ, Park IK, Chung H, Lee J. The clinical and ocular manifestations of Takayasu arteritis. Retina. 2001;21:132-40. 6. Schmidt MH, Fox AJ, Nicolle DA. Bilateral anterior ischemic optic neuropathy as a presentation of Takayasu’s disease. J Neuroophthalmol. 1997;17:156-61. 7. Nithyanandam S, Mohan A, Sheth U. Anterior ischemic optic neuropathy in a case of Takayasu arteritis. Oman J Ophthalmol. 2010;3:94-5. 8. Hayreh SS. Ischemic optic neuropathy. Prog Retin Eye Res. 2009;28:34-62. 9. Hayreh SS. Anterior ischemic optic neuropathy. II. Fundus on ophthalmoscopy and fluorescein angiography. Br J Ophthalmol. 1974;58:964-80. 10. Foroozan R, Buono LM, Savino PJ, Sergott RC. Tonic pupils from giant cell arteritis. Br J Ophthalmol. 2003;87:510-2. 11. Wilhelm H. Tonic pupil caused by ischemia. Fortschr Ophthalmol. 1989;86:380-2. 12. Prasad S, Baccon J, Galetta SL. Mydriatic pupil in giant cell arteritis. J Neurol Sci. 2009;284:196-7. 13. Coppeto JR, Greco T. Mydriasis in giant cell arteritis. J Clin Neuroophthalmol. 1989;9:267-9. Can J Ophthalmol 2013;48:e159–e163 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.07.005
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e163
Fig. 3 — Keratinizing squamous cell carcinoma arising from the perilimbal conjunctiva and invading underlying sclera (hematoxylin and eosin stain; original magnification 132; arrows indicate infiltrative nests).
of the tumour were well-differentiated with keratin formation, whereas other regions showed moderate differentiation, in contrast with the patient’s poorly differentiated oropharyngeal recurrence. Periodic acid–Schiff and mucicarmine stains were negative for mucin material. The final diagnosis was SCC, moderately to well-differentiated, involving the conjunctiva, lateral rectus muscle, sclera, and uveal tract with the growth pattern and degree of differentiation favoring the diagnosis of a second primary tumour and not a metastasis. Conjunctival SCC usually occurs in older male smokers with a history of sun and dust exposure.2 It tends to be superficially invasive; intraocular invasion rarely occurs in patients having undergone multiple surgical resections, cryotherapy, topical chemotherapy, and/or radiation.4 The mucoepidermoid and spindle cell variants are more locally aggressive and have higher recurrence rates.4 In this case, the patient had not received prior ocular treatments, and aggressive histologic SCC variants were
Tonic pupil, anterior ischemic optic neuropathy in a teenager with Takayasu arteritis This case report describes a 16-year-old female with a unilaterally tonic pupil and contralateral anterior ischemic optic neuropathy (AION) as the ocular features of Takayasu arteritis. A 16-year-old female reported sudden blurring of vision in the left eye. Systemic examination showed the presence of left hemiparesis along with nonpalpable upper limb pulses and weak left carotid pulsations. Magnetic resonance imaging of the brain (Fig. 1A) demonstrated an acute infarct in
Shaheen C. Kavoussi, * Debra J. Shetlar, † Andrew J. Barkmeier, ‡ Petros E. Carvounis §
* Yale University, New Haven, Conn.; †Michael E. DeBakey Veteran Affairs Medical Center, Houston, Tex.; ‡Mayo Clinic, Rochester, Minn.; §Cullen Eye Institute, Baylor College of Medicine, Houston, Tex.
Correspondence to: Petros E. Carvounis, MD: [email protected]
REFERENCES 1. Lee GA, Hirst LW. Retrospective study of ocular surface squamous neoplasia. Aust N Z J Ophthalmol. 1997;25:269-76. 2. Hamam R, Bhat P, Foster CS. Conjunctival/corneal intraepithelial neoplasia. Int Ophthalmol Clin. 2009;49:63-70. 3. Tabbara KF, Kersten R, Daouk N, Blodi FC. Metastatic squamous cell carcinoma of the conjunctiva. Ophthalmology. 1988;95:318-21. 4. Cohen BH, Green WR, Iliff NT, et al. Spindle cell carcinoma of the conjunctiva. Arch Ophthalmol. 1980;98:1809-13. 5. Shelil AE, Shields CL, Shields JA, Eagle RC. Aggressive conjunctival squamous cell carcinoma in a patient following liver transplantation. Arch Ophthalmol. 2003;121:280-2. Can J Ophthalmol 2013;48:e158–e159 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.08.012
the right periventricular frontoparietal lobe (the watershed territory of right middle and anterior cerebral artery). Magnetic resonance angiography (MRA) demonstrated complete occlusion of the origins of the right brachiocephalic artery along with its branches and left subclavian artery. There was near total occlusion of the left common carotid artery (Fig. 1B) in keeping with type IIa Takayasu arteritis.1 The best corrected visual acuity was measured as 6/6 in the right eye and 6/9 in the left eye. Examination of the pupil revealed anisocoria, with the right pupil measuring 5 mm and left pupil 2 mm in light. The right pupil was poorly reactive to light, and there was a relative afferent pupillary defect in the left eye that manifested with a
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e159
Correspondence
Fig. 1A — Magnetic resonance imaging brain diffusionweighted (A) and apparent diffusion coefficient map (B) images of the brain showing acute restricted diffusion (white arrow) in the right frontoparietal lobe. 1B, Magnetic resonance angiography of carotid vessels showing complete occlusion of right carotid and near-complete occlusion of the left common carotid artery (black arrow).
swinging flashlight test. The right pupillary reaction to a near fixation target was tonic, exhibiting light near dissociation. The right pupil constricted to 0.125% topical pilocarpine, thereby showing evidence of denervation supersensitivity. Vermiform movements of the right pupil with sectoral tonic iris constrictions were visible with a slitlamp examination, which were not present in the left pupil. The intraocular pressures were measured by Goldmann applanation tonometry as 8 and 11 mm Hg in the right and left eye, respectively. Anterior segment examination was normal in both eyes. A dilated examination of the fundus showed a normal disc and macula in the right eye, whereas the left eye revealed the presence of pallid disc edema with venous tortuosity (Fig. 2). Visual field testing by Humphrey visual field analyzer 30-2 was normal in the right eye. The left eye visual field showed a superior altitudinal field defect extending to the inferonasal quadrant (Fig. 3A) with a mean deviation of –23.84 dB. Based on the earlier findings, the patient was diagnosed with tonic pupil in the right eye and AION in the left eye with type IIa Takayasu arteritis. Thereafter she underwent bilateral carotid angioplasty with stenting. Ocular examination repeated after 3 months showed persistence of the tonic pupil and relative afferent pupillary defect with normal disc in the right eye and optic atrophy in the left eye with resolution of the optic disc edema (Fig. 4). Visual field testing showed marginal improvement in the left eye fields with the mean deviation changing from –23.84 to –14.79 dB (Fig. 3B). Takayasu arteritis is a nonspecific aortoarteritis characterized by granulomatous inflammation of the large arteries first described by Takayasu in 1908. The inflammation is pan-arteritic with proliferation of tunica intima, destruction of elastic tunica media, followed by fibrosis of the adventitia. The ischemic features due to vessel thrombosis and occlusion can range from transient ischemic attacks, stroke, mesenteric ischemia, renovascular hypertension, myocarditis to cardiac failure.2 It is more common in females, and the majority of patients present before the age of 40 years. Children usually have type I Takayasu arteritis with fever, arthralgias, and cardiac involvement simulating rheumatic fever or with
Fig. 2 — Fundus photograph of both eyes showing normal disc in the right eye and pallid disc edema in the left eye.
e160
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Correspondence
Fig. 3 — A, Humphrey visual field report 30-2 program with Swedish Interactive Threshold Algorithm ( SITA) standard of the left eye showing a superior altitudinal scotoma. B, Humphrey visual field report 30-2 program with SITA standard of the left eye showing slight recovery.
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e161
Correspondence
Fig. 3
e162
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Correspondence
Fig. 4 — Fundus photograph of the left eye showing optic atrophy.
features of hypertension because of renal vessel involvement (type IV).3 The ocular presentations in Takayasu arteritis may be variable and depend on the branches of the carotid artery involved, duration, and rate of vascular insufficiency and development of collateral blood supply. Pathogenesis of these manifestations is either hypoperfusion after occlusion of the branches of the carotid or hypertension secondary to renal artery involvement.4 The most common ocular feature of hypoperfusion in the carotid arteries is Takayasu retinopathy,4 followed by ocular ischemic syndrome leading to neovascular glaucoma, vitreous hemorrhage, and tractional retinal detachment. Hypertensive retinopathy and bilateral papilledema are less common and might occur because of renal artery stenosis.5 AION in Takayasu arteritis has been reported in few adult cases.6,7 Ocular features are rare in children, and the occurrence of AION has not yet been reported in children with Takayasu arteritis (search MEDLINE database). Hypoperfusion in the internal carotid vessels results in nonarteritic AION, whereas thrombotic narrowing of the posterior ciliary arteries can cause the arteritic type of AION.8 In our case, the acute-onset disc edema with pallor may be an arteritic form of AION that appears to be secondary to the near-total occlusion of the left internal carotid artery. Pallid disc edema is described as chalky white swelling of the optic disc in 68.7% of cases with arteritic AION,9 mostly caused by giant cell arteritis (GCA). However, in the younger age group, presence of pallid disc edema should arouse suspicion of other causes of vasculitis such as Takayasu arteritis because GCA is uncommon at this age. Tonic pupils caused by ischemia have been reported in GCA10 and resolving internal carotid artery occlusion.11 The mechanism by which these entities produce tonic pupils is not well-known. Ischemia of the ciliary ganglion
and postganglionic parasympathetic fibres,10,12 ischemia of the iris sphincter itself,12 or damage to the ciliary ganglion mediated by immunologic mechanisms13 are proposed as the mechanisms in patients with GCA. A literature search (MEDLINE database) did not show any reports of tonic pupil in Takayasu arteritis. The complete occlusion of right internal carotid artery as evidenced by the MRA may have resulted in the reduced orbital blood flow to the ciliary ganglion and postganglionic ciliary nerves resulting in ischemia and a tonic pupil. This case highlights the hitherto unreported features of tonic pupil and AION in a teenager with Takayasu arteritis. We suggest that the presence of features of ocular ischemia in children should raise the suspicion of an underlying age-specific systemic vasculitis like Takayasu disease. Jyoti Matalia, Nirupama Kasturi, Hemant D. Anaspure, Bhujang K. Shetty Narayana Nethralaya, Bangalore, India Correspondence to: Jyoti Matalia, DNB: [email protected]
REFERENCES 1. Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestation of Takayasu arteritis in India and Japan—new classification of angiographic findings. Angiology. 1997;48:369-79. 2. Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol. 2002;55:481-6. 3. Cakar N, Yalcinkaya F, Duzova A, et al. Takayasu arteritis in children. J Rheumatol. 2008;35:913-9. 4. Peter J, David S, Joseph G, Horo S, Danda D. Hypoperfusive and hypertensive ocular manifestations in Takayasu arteritis. Clin Ophthalmol. 2010;4:1173-6. 5. Chun YS, Park SJ, Park IK, Chung H, Lee J. The clinical and ocular manifestations of Takayasu arteritis. Retina. 2001;21:132-40. 6. Schmidt MH, Fox AJ, Nicolle DA. Bilateral anterior ischemic optic neuropathy as a presentation of Takayasu’s disease. J Neuroophthalmol. 1997;17:156-61. 7. Nithyanandam S, Mohan A, Sheth U. Anterior ischemic optic neuropathy in a case of Takayasu arteritis. Oman J Ophthalmol. 2010;3:94-5. 8. Hayreh SS. Ischemic optic neuropathy. Prog Retin Eye Res. 2009;28:34-62. 9. Hayreh SS. Anterior ischemic optic neuropathy. II. Fundus on ophthalmoscopy and fluorescein angiography. Br J Ophthalmol. 1974;58:964-80. 10. Foroozan R, Buono LM, Savino PJ, Sergott RC. Tonic pupils from giant cell arteritis. Br J Ophthalmol. 2003;87:510-2. 11. Wilhelm H. Tonic pupil caused by ischemia. Fortschr Ophthalmol. 1989;86:380-2. 12. Prasad S, Baccon J, Galetta SL. Mydriatic pupil in giant cell arteritis. J Neurol Sci. 2009;284:196-7. 13. Coppeto JR, Greco T. Mydriasis in giant cell arteritis. J Clin Neuroophthalmol. 1989;9:267-9. Can J Ophthalmol 2013;48:e159–e163 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.07.005
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e163