Congenital Horner Syndrome with Heterochromia Iridis Associated with Ipsilateral Internal Carotid Artery Hypoplasia

Abstract

BackgroundHorner syndrome (HS), also known as Claude-Bernard-Horner syndrome or oculosympathetic palsy, comprises ipsilateral ptosis, miosis, and facial anhidrosis.Case ReportWe report herein the case of a 67-year-old man who presented with congenital HS associated with ipsilateral hypoplasia of the internal carotid artery (ICA), as revealed by heterochromia iridis and confirmed by computed tomography (CT).ConclusionsCT evaluation of the skull base is essential to establish this diagnosis and distinguish aplasia from agenesis/hypoplasia (by the absence or hypoplasia of the carotid canal) or from acquired ICA obstruction as demonstrated by angiographic CT.

Congenital Horner’s Syndrome and Internal Carotid Artery Hypoplasia

“…Deprez et al have proposed that all cases of ICA agenesis, aplasia, or hypoplasia may actually be accompanied by Horner's syndrome if carefully examined. 2 The finding of heterochromia iridis in our patient is similar to other reported cases. [2][3][4] The common neural crest origin of the sympathetic nervous system ganglia and the iris melanocytes likely underlie this association.…”

“…2 The finding of heterochromia iridis in our patient is similar to other reported cases. [2][3][4] The common neural crest origin of the sympathetic nervous system ganglia and the iris melanocytes likely underlie this association. 3 Congenital ICA agenesis, aplasia, and hypoplasia are usually incidental findings but have on occasion also been found to be associated with stroke and intracranial aneurysms.…”

“…1 A few case reports have found the condition to co-exist with other rare congenital entities involving the internal carotid artery (ICA): agenesis, aplasia, or hypoplasia. [2][3][4] We present a case of a healthy 21-year-old male who was found to have a left eye ptosis during a routine consult with his family physician. Subsequently, an ophthalmologist made the diagnosis of Horner's syndrome.…”

Unilateral Straight Hair—A Symptom of Acquired Horner's Syndrome in a Neonate

“…Occurrence of Horner's syndrome due to neuroblastoma has been described in the literature. 16 Our patient showed no sign of Horner's syndrome before surgery nor was her iris involved which represents a typical symptom of congenital Horner's syndrome. 16 …”

Bilateral symmetrical nasal retinal hypopigmentation associated with iris heterochromia: A case report

“…Iris color is one of the most obvious physical characteristics of a person that is determined by the concentration and distribution of melanin in the iris. Iris heterochromia that is the difference in the coloration of the iris may be complete or partial (sectoral), as well as congenital or acquired (1). Patients with complete heterochromia have two different-colored eyes, such as a blue eye and a brown eye.…”