Eye (2018) 32, 457–459 © 2018 Macmillan Publishers Limited, part of Springer Nature. All rights reserved 0950-222X/18 www.nature.com/eye 1,2 2 2 Intermittent mydriasis PD Chamberlain , A Sadaka , S Berry CASE SERIES 1,2,3,4,5,6 associated with carotid and AG Lee vascular occlusion Abstract the literature as benign episodic pupillary dilation or BEUM. We report two patients with Purpose To describe two cases of 1 stereotyped, intermittent, neurologically acquired occlusive disease of the ipsilateral ICA Department of Ophthalmology, Blanton isolated, unilateral mydriasis in patients who developed multiple, stereotyped, neurologically isolated, transient episodes of Eye Institute, Houston with a history of acquired internal carotid Methodist Hospital, artery (ICA) occlusive disease on the mydriasis consistent with BEUM. We discuss the Houston, TX, USA ipsilateral side. possible mechanisms, differential diagnosis and 2 Patients Two patients with intermittent recommended evaluation for atypical cases for Department of episodic mydriasis. Ophthalmology, Baylor mydriasis. College of Medicine, Methods Case Series. Houston, TX, USA Results Case one: A 78-year-old man Case one 3 experienced 10 episodes of intermittent, Departments of Ophthalmology, Neurology, unilateral, and painless mydriasis in the left A 78-year-old man presented with 10 episodes of and Neurosurgery, Weill stereotyped, intermittent, unilateral, painless eye and had 100% occlusion of the left ICA Cornell Medical College, artery due to atherosclerotic disease. Case two: pupillary dilation of the left eye (OS) lasting New York, NY, USA A 26-year-old woman with history of migraine minutes to hours at a time without diplopia or fi 4Department of developed new painless, intermittent episodes ptosis. The rst episode happened approximately Ophthalmology, University 1 year prior to presentation. The episodes seemed of unilateral mydriasis after sustaining chest of Texas Medical Branch, trauma and was diagnosed with subsequent to be worse in the light. Past medical history was Galveston, TX, USA dissection and 65% occlusion of the ipsilateral notable for atherosclerotic large vessel, cardiac, 5 ICA. Neither patient developed permanent valvular, and coronary artery disease treated with Department of Ophthalmology, University anisocoria. coronary bypass grafting, left subclavian bypass, of Iowa Hospitals and Conclusion Benign episodic unilateral right carotid endarterectomy, cardiac pacemaker Clinics, Iowa City, IA, USA mydriasis (BEUM) typically presents in young placement, and aortic valve replacement. The most 6 women with a history of migraine. To our recent of these surgeries, aortic valve replacement, Department of knowledge, these are the first cases of episodic, took place 1 year prior to the onset of symptoms, Ophthalmology, UT MD Anderson Cancer Center, unilateral, neurologically isolated mydriasis but the rest of these surgeries took place 8 or more Houston, TX, USA associated with occlusive disease of the ICA in years prior to onset of symptoms. Because of the the English language ophthalmic literature. We extensive vascular disease history, a repeat carotid Correspondence: hypothesize that transient dysfunction of the Doppler was performed and showed complete AG Lee, Department of autonomic nervous system related to the ICA occlusion of the left ICA. This 100% occlusion of Ophthalmology, Blanton disease may account for the intermittent Eye Institute, Houston the left ICA had also been documented by carotid Methodist Hospital, 6560 mydriatic episodes in these patients and we ultrasound 6 months before. Fannin Street, Scurlock recommend consideration for imaging of the Neuro-ophthalmic exam showed visual acuity Tower 450, Houston, TX ICA in patients with atypical features for of 20/30 in the right eye (OD) and 20/20 in OS. 77030, USA + BEUM (for example, old age or males, non- Despite the complaint of BEUM, the pupils, were Tel: 713 441-8823; Fax: 713-793-1636. isolated mydriasis, or recent trauma). isocoric under light and dark lighting conditions – E-mail: aglee@ Eye (2018) 32, 457 459; doi:10.1038/eye.2017.127; and were normally reactive to light OU. There houstonmethodist.org published online 11 August 2017 was no dilation lag or relative afferent pupillary defect (RAPD) seen. Slit-lamp exam showed no Received: 31 December pupil irregularities, vermiform movements, or 2016 Introduction Accepted in revised form: other iris abnormalities. There was no ptosis or 8 June 2017 Intermittent, neurologically isolated, mydriasis ophthalmoplegia. The remainder of the eye Published online: is typically benign and has been referred to in examination was noncontributory. Magnetic 11 August 2017 Intermittent mydriasis PD Chamberlain et al 458 resonance (MR) scan and MR angiography of the head sympathetic overaction or parasympathetic underaction and neck showed 100% occlusion of the left cervical ICA is the cause for the transient mydriasis in our case one. but no other abnormalities. There was no clinical evidence In another report, a 37-year-old woman reported two for a Horner syndrome but no topical pharmacologic episodes of unilateral mydriasis separated by two years, testing was performed. and was found to have an ipsilateral ICA dissection following workup.3 She had a history of severe cervical Case two trauma in a motor vehicle accident several years prior to her first episode, and was suspected to have A 26-year-old female with a history of migraine presented oculosympathetic spasm as the mechanism (a type of with multiple, recurrent, intermittent, unilateral episodes autonomic hyperreflexia associated with cervical spinal of painless pupillary dilation OD. These occurred cord injury).4 It is also possible that surgical manipulation approximately 20 months after sustaining neck trauma by of the great vessels during one of case one’s many prior being kicked by a horse. At the time of her initial injury, surgeries could also have caused damage to the she suffered a right ICA dissection with secondary 65% oculosympathetic pathway with subsequent sympathetic stenosis and was treated with aspirin. In the months overaction. following the trauma she suffered a transient left In our case two, it is possible that oculosympathetic hemiparesis episode and a single 1-min episode of ‘spasm’ (overaction) following damage to the sympathetic vision loss OD (amaurosis fugax). Cervical CT nerve fibers surrounding the ICA either due to the initial fi angiography (CTA) con rmed a right carotid dissection ICA trauma or the subsequent ICA dissection may have which was stented. She was treated with aspirin, been responsible for the intermittent mydriasis. There are clopidogrel, and apaxiban following a negative cardiac also three reports of unilateral mydriasis in the acute workup for presumed transient ischemic attack and setting as the presenting sign of carotid occlusion amaurosis fugax OD. Exam showed visual acuity of – secondary to dissection,5 7 and mydriasis was thought 20/20 in both eyes and isocoric pupils in the light and in to be secondary to CN III ischemia. None of these the dark without RAPD. There was no ptosis or evidence previously reported cases displayed intermittent of Horner syndrome, and she exhibited full motility. mydriasis. Interestingly, our case two did not report Automated perimetric, slit-lamp, and fundus exams the transient mydriasis episodes at the time of initial were normal. No topical pharmacologic testing was injury and the symptoms occurred only later in her performed. course. Although it is unknown if the episodes in our two cases Discussion were causal or coincidental to the ICA disease we propose that transient autonomic dysfunction related to the ICA BEUM is a relatively common syndrome presumed to be could account for the intermittent episodes of pupillary due to transient dysfunction in the pupillary autonomic nervous system.1 BEUM typically affects younger women dilation. It is not clear if the isolated, episodic mydriasis is and is often associated with migraine.1 No workup or due to overaction of the oculosympathetic pathway or neuroimaging is recommended in typical cases of BEUM. underaction of the parasympathetic pathway, but In our two cases, however, atypical features were present given the ipsilateral ICA disease we suspect the that prompted further evaluation. In case one, the patient former despite the lack of clinical evidence for was an elderly male with no history of migraine and in oculosympathetic dysfunction (Horner syndrome) case two the patient had prior neck and chest trauma and clinically. The possibility of acephalgic migraine a secondary ICA dissection. A prior case report of equivalent in the elderly (case one) or migraine- intermittent mydriasis in a teenage patient with related BEUM (case two) cannot be completely congenital hypoplasia of the ICA has been described.2 excluded. Cranial MR imaging of that patient revealed an enlarged In summary, transient, neurologically isolated, posterior communicating artery (PComA) and it was mydriasis is typically benign (BEUM), but patients who hypothesized that possible intermittent CN III have non-neurologically isolated disease, do not have dysfunction was the mechanism. In our case one, we migraine, are elderly, vasculopathic, or male or who have initially hypothesized that perhaps occlusion of the left had recent trauma might be considered for further cranial ICA was associated with secondary enlargement of the and neck imaging of the ipsilateral ICA. To our PComA which could have