Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome

Iris heterochromia is typically seen in association with congenital Horner syndrome. A man in his 40s with congenital iris heterochromia, blue in the right and brown in the left, presented with left-sided Horner syndrome. This was associated with recent change in color of his brown left iris to blue similar to the right iris. This case demonstrates a unique case of adult-onset Horner syndrome with reversal of iris heterochromia.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome Tanyatuth Padungkiatsagul, MD, Lakshmi Leishangthem, MD, Heather E. Moss, MD, PhD FIG. 1. Anterior segment photograph from time of clinical evaluation shows reversal of iris heterochromia; the left iris changed into blue color similar to the right eye. Some of the congenital brown iris color remains evident at the pupillary border and in the peripheral iris. Abstract: Iris heterochromia is typically seen in association with congenital Horner syndrome. A man in his 40s with congenital iris heterochromia, blue in the right and brown in the left, presented with left-sided Horner syndrome. This was associated with recent change in color of his brown left iris to blue similar to the right iris. This case demonstrates a unique case of adult-onset Horner syndrome with reversal of iris heterochromia. Journal of Neuro-Ophthalmology 2021;41:e230–231 doi: 10.1097/WNO.0000000000001089 © 2020 by North American Neuro-Ophthalmology Society A man in his 40s presented with anisocoria and mild left ptosis for 2 years. He had a medical history of hypertension, multiple traumatic brain injuries (concussion during high school sports), and congenital iris heterochromia, blue in the right and brown in the left. He was aware of his congenital iris heterochromia since childhood. Recently, he noticed changing in his left iris color from brown to blue. He had no visual disturbance and was otherwise healthy. He Department of Ophthalmology (TP), Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; Department of Ophthalmology (TP, LL, HEM), Stanford University, Palo Alto, California; and Department of Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California. NIH P30 026877, Research to Prevent Blindness. The authors report no conflicts of interest. Address correspondence to Tanyatuth Padungkiatsagul, Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Phayathai, Bangkok, Thailand 10400; E-mail: [e-mail] e230 denied any history of previous intraocular surgery. His family history was negative for iris heterochromia. Neuroophthalmology examination revealed anisocoria. In bright light, his right and left pupil sizes were 3 and 1.5 mm, respectively. In dim light, his right pupil dilated to 5 mm and left pupil dilated to 2.5 mm. There was mild ptosis in his left eye and anhidrosis in the left V1, V2 distribution. Slit-lamp examination revealed blue-colored left iris with residual brown-colored area at pupillary margin and peripheral iris. The right iris was uniformly blue (Fig. 1). Review of a photograph from 4 years before as well as his driver’s license confirmed that the left iris color change and mild left ptosis were acquired (Fig. 2). In addition, there were no mid-iris transillumination defects, Krukenberg spindle, or exfoliative materials noticed on slit-lamp examination. His corneas were clear, nonedematous, and devoid of any deposition rings or keratic precipitates. Anterior chambers were devoid of anterior chamber cell/flare or evidence of Fuchs heterochromic iridocyclitis. Gonioscopy was not performed. Fundus examination revealed normally pigmented retina. His cranial nerve examination was unremarkable. He did not have a white forelock. A diagnosis of left-sided Horner syndrome was confirmed with 1% apraclonidine testing. Neuroimaging studies including MRI and MRA of the brain and neck with and without contrast extended to upper chest were unremarkable. A diagnosis of idiopathic Horner syndrome was made. Iris heterochromia, caused by deficiency of sympathetic activity interfering with melanin pigmentation of the melanocytes in the superficial stroma of the iris, is described as one of the clinical features of congenital Horner Padungkiatsagul et al: J Neuro-Ophthalmol 2021; 41: e230-e231 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay temic diseases that may also cause iris heterochromia, such as Waardenburg syndrome or oculocutaneous albinism, were not consistent with the clinical picture in our case. FIG. 2. Portrait photograph from 4 years before presentation demonstrates pre-existing congenital iris heterochromia, when the right iris was blue and left iris was brown. syndrome. However, acquired Horner syndrome with iris heterochromia has been rarely reported (1–3). We contribute a unique case of adult-onset Horner syndrome with reversal of iris heterochromia as he had different iris colors from congenital iris heterochromia and subsequently changed into a similarly blue color in both eyes. Significant negative pertinent findings from slit-lamp examination have excluded other causes in the differential diagnosis for adultonset acquired iris heterochromia including pigment dispersion syndrome, true or pseudoexfoliation syndrome, and Fuchs heterochromic iridocyclitis. Moreover, other rare sys- Padungkiatsagul et al: J Neuro-Ophthalmol 2021; 41: e230-e231 STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss; b. Acquisition of data: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss; c. Analysis and interpretation of data: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss. Category 2: a. Drafting the manuscript: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss; b. Revising it for intellectual content: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss. Category 3: a. Final approval of the completed manuscript: T. Padungkiatsagul, L. Leishangthem, and H. E. Moss. REFERENCES 1. Beynat J, Soichot P, Bidot S, Dugas B, Creuzot-Garcher C, Bron A. Iris heterochromia in acquired Horner’s syndrome. J Fr Ophtalmol. 2007;30:e19. 2. Ulusoy MO, Kivanc SA, Atakan M, Mayali H. Post-thyroidectomy iatrogenic Horner’s syndrome with heterochromia. J Curr Ophthalmol. 2016;28:46–47. 3. Diesenhouse MC, Palay DA, Newman NJ, To K, Albert DM. Acquired heterochromia with horner syndrome in two adults. Ophthalmology. 1992;99:1815–1817. e231 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.