Cilioretinal Artery Territory Infarction Associated With Papilledema in a Patient With Neurofibromatosis Type 2

A 14-year-old girl presented with a history of left-sided headache and acute bilateral blurred vision. She had a remote history of oral tetracycline use for the treatment of acne vulgaris, which had been discontinued for 1 month. The patient was diagnosed with drug-induced intracranial hypertension (IH) and treated with oral acetazolamide with subsequent resolution of symptoms. IH, a known rare complication of the tetracycline class of antibiotics, can also have a delayed presentation after discontinuation of the medication.

Clinical Observation Cilioretinal Artery Territory Infarction Associated With Papilledema in a Patient With Neurofibromatosis Type 2 Omar A. Mahroo, PhD, FRCOphth, Moin D. Mohamed, PhD, FRCOphth, Elizabeth M. Graham, FRCOphth, Samantha S. Mann, MD, FRCOphth, Gordon T. Plant, MD, FRCP, Shazia K. Afridi, MRCP, PhD, Christopher J. Hammond, MD, FRCOphth Abstract: Cilioretinal artery territory infarction can occur in isolation or in association with other vascular compromise of the retinal circulation. Our patient, an 18-year-old woman with neurofibromatosis type 2, developed a cilioretinal artery territory infarction in the setting of papilledema. Our case, together with one previous report, suggests that cilioretinal artery territory infarction in the context of papilledema, although rare, is a real entity. Journal of Neuro-Ophthalmology 2016;36:58-60 doi: 10.1097/WNO.0000000000000279 © 2015 by North American Neuro-Ophthalmology Society C ilioretinal artery territory infarction can occur in isolation or in association with other vascular compromise of the retina, such as retinal vein occlusion (1-5). We report a case associated with papilledema and suggest potential pathophysiologic mechanisms. CASE REPORT An 18-year-old woman with neurofibromatosis type 2 (NF2) reported blurring of vision in her left eye. Her right eye had been phthisical for many years. She had bilateral vestibular Department of Ophthalmology (OAM, MDM, EMG, SM, GTP, CJH), Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Department of Ophthalmology (OAM, CJH), King's College London, London, United Kingdom; and Department of Neurology (SA), Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the full text and PDF versions of this article on the journal's Web site (www. jneuro-ophthalmology.com). Address correspondence to Omar A. Mahroo, PhD, FRCOphth, Department of Ophthalmology, St Thomas' Hospital Campus, King's College London, Westminster Bridge Road, London SE1 7EH, United Kingdom; E-mail: [e-mail] 58 schwannomas, multiple meningiomas including one involving the right optic nerve sheath, and schwannomas of the right third nerve and spine. Mild left optic disc edema had been noted 3 months previously, with normal visual acuity (20/20), color vision, and visual fields. On our examination, vision was 20/30 in the left eye, with intact color vision and a cecocentral scotoma in the left visual field by confrontation technique. Ocular motility was consistent with a left sixth nerve palsy. Ophthalmoscopy coupled with optical coherence tomography (OCT) demonstrated a swollen optic disc with pale, cloudy, inner retinal swelling along the course of a cilioretinal artery (Fig. 1). Brain magnetic resonance imaging (MRI) revealed no compressive lesion of the left optic nerve but significant increase in size of a left parasagittal meningioma. Computed tomographic venogram did not show cerebral venous sinus compression or thrombosis. The increased optic disc swelling and sixth nerve palsy were attributed to increased intracranial pressure, likely because of growth of a parasagittal meningioma. Systemic investigations failed to detect a vasculitic or thromboembolic cause of her cilioretinal infarction. This included thrombophilia blood tests, carotid artery Doppler ultrasound, and negative testing for antinuclear and antineutrophil cytoplasmic antibodies and extractable nuclear antigen. The patient was prescribed acetazolamide and aspirin. Four weeks later, there was less left optic disc edema, resolution of retinal pallor, and inner retinal thinning evident on OCT (Fig. 2; See Supplemental Digital Content, Figure E1, http://links.lww.com/WNO/A165). Her visual acuity remained 20/30 in the left eye, and her ophthalmic examination was otherwise unchanged. DISCUSSION Cilioretinal artery territory infarction with papilledema has not been reported previously. In our patient, MRI excluded Mahroo et al: J Neuro-Ophthalmol 2016; 36: 58-60 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 1. A. The left fundus shows optic disc swelling and an area of retinal pallor in the distribution of a cilioretinal artery. B. Spectral domain optical coherence tomography (OCT) reveals marked swelling of the optic disc and thickened hyperintense inner retina (arrows) nasal to the fovea. C. Panels show the respective location of OCT scans. a compressive lesion of the left optic nerve. Therefore, the disc swelling with intact visual function coupled with new onset sixth nerve palsy supported the diagnosis of increased intracranial pressure. The increased tumor size directly raised intracranial pressure. Cilioretinal artery territory infarction is rare at such an age (vasculopathy is not usually associated with NF2); systemic investigations for a thrombotic or embolic cause were negative. We propose 2 potential mechanisms for the clinical findings in our patient. First, possibly the marked disc edema raised the proximal pressure in the cilioretinal circulation, either through direct compression or through increasing the venous pressure, such that blood flow in the posterior ciliary arteries was preferentially diverted to the lower pressure choroidal circulation. This is similar to the mechanism McLeod (5) postulated in patients with cilioretinal infarction and central retinal vein occlusion. Second, perhaps the marked optic disc swelling occluded the cilioretinal artery through extrinsic pressure. Why is cilioretinal artery territory infarction rarely seen in papilledema? It is possible that our case had some predisposing variant anatomy. McLeod (5) suggested that a more distal point of origin of the cilioretinal artery from the posterior ciliary arteries would make infarction more likely in the context of a retinal vein occlusion. In our patient, possibly the configuration of the origin of the cilioretinal artery from the FIG. 2. A. Four weeks later, there is less optic disc edema with resolution of retinal pallor. B. On OCT, there is less optic disc swelling, and the inner retina nasal to the fovea is now thinned (arrows). C. Panels show respective locations of OCT scans. Mahroo et al: J Neuro-Ophthalmol 2016; 36: 58-60 59 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation posterior ciliary circulation might have been unique, perhaps in terms of the angle of entry or size of the lumen, or a location more distally in the posterior ciliary circulation. This would make the inlet pressure particularly low, rendering compromise of forward flow more likely in the presence of back pressure from optic nerve head swelling. Interestingly, Lamirel et al (6) described a patient with idiopathic intracranial hypertension who developed a choroidal infarction in the left eye. The fundus appearance was strikingly similar to that of our patient. Subsequently, McCannel (7) argued that the findings reported by Lamirel et al were due to a cilioretinal rather than choroidal infarction. The OCT results in our patient showed inner retinal thinning on follow-up, confirming that this was not a choroidal infarction. Thus, cilioretinal artery territory infarction because of papilledema would appear to be a real, although rare, entity. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: O. A. Mahroo, M. D. Mohamed, E. M. Graham, S. Mann, G. T. Plant, S. Afridi, C. J. Hammond; b. Acquisition of data: O. A. Mahroo; c. Analysis and interpretation of data: O. A. Mahroo, M. D. Mohamed, E. M. Graham, S. Mann, G. T. Plant, S. Afridi, C. J. Hammond. Category 2: a. Drafting the manuscript: O. A. Mahroo; b. Revising it for intellectual content: O. A. Mahroo, M. D. Mohamed, E. M. Graham, S. Mann, G. T. Plant, 60 S. Afridi, C. J. Hammond. Category 3: a. Final approval of the completed manuscript: O. A. Mahroo, M. D. Mohamed, E. M. Graham, S. Mann, G. T. Plant, S. Afridi, C. J. Hammond. REFERENCES 1. McLeod D, Ring CP. Cilio-retinal infarction after retinal vein occlusion. 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Choroidal infarction or cilioretinal artery occlusion in the setting of elevated intracranial pressure due to fulminant idiopathic intracranial hypertension? J Neuroophthalmol. 2011;31:194-195. Mahroo et al: J Neuro-Ophthalmol 2016; 36: 58-60 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.