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Congenital Horner's syndrome observed in an adult

  • Daniel A. Balikov
    Affiliations
    Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, Mich
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  • Wayne T. Cornblath
    Affiliations
    Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, Mich

    Department of Neurology, University of Michigan, Ann Arbor, Mich
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  • Rajesh C. Rao
    Correspondence
    Correspondence to Rajesh C. Rao, MD, Department of Ophthalmology, University of Michigan Medical Center, 1500 E Medical Center Drive, Ann Arbor, MI 48109
    Affiliations
    Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, Mich

    Rogel Cancer Center, University of Michigan, Ann Arbor, Mich

    Department of Pathology, University of Michigan, Ann Arbor, Mich

    Department of Human Genetics, University of Michigan, Ann Arbor, Mich

    A. Alfred Taubman Medical Research Institute, University of Michigan, Ann Arbor, Mich

    Section of Ophthalmology, Surgery Service, Veterans Administration Ann Arbor Health System, Ann Arbor, Mich
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Published:August 28, 2022DOI:https://doi.org/10.1016/j.jcjo.2022.07.013
      A 55-year-old woman reported, since infancy, right-sided ptosis (Fig. 1A, 1B), anhidrosis, exertional pallor (Fig. 1B), and lightly pigmented iris with miosis (Fig. 1C vs 1D). There was no history of birth trauma, malignancy, or vascular abnormality. Examination was otherwise normal other than a reversal of anisocoria with apraclonidine (before apraclonidine administration: Fig. 2A; after apraclonidine administration: Fig. 2B). These features indicate congenital Horner's syndrome, in which sympathetic innervation controlling Müller's muscle of the eyelid, iris dilator muscles, iris pigmentation, sweat glands, and vasomotor activity is disrupted. Ipsilateral anhidrosis implies a pre-superior cervical ganglionic lesion. Unlike acquired Horner's syndrome, congenital Horner's syndrome is generally a benign, idiopathic entity that does not require routine imaging beyond examination during infancy to exclude masses along the sympathetic chain.
      Figure 1
      Figure 1Right-sided ptosis (A, B), anhidrosis, exertional pallor (B), and lightly pigmented iris with miosis (C vs D).
      Figure 2
      Figure 2Before apraclonidine administration (A) and after apraclonidine administration (B).
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