J Clin Neurol.  2015 Apr;11(2):192-196. 10.3988/jcn.2015.11.2.192.

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

• Deprez FC
• Coulier J
• Rommel D
• Boschi A

• KMID: 2242666
• DOI: http://doi.org/10.3988/jcn.2015.11.2.192

Abstract

BACKGROUND
Horner syndrome (HS), also known as Claude-Bernard-Horner syndrome or oculosympathetic palsy, comprises ipsilateral ptosis, miosis, and facial anhidrosis.
CASE REPORT
We 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).
CONCLUSIONS
CT 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.

Keyword

congenital horner syndrome; internal carotid artery agenesis; heterochromia iridis; computed tomography

MeSH Terms

Aged
Carotid Artery, Internal*
Diagnosis
Horner Syndrome*
Humans
Hypohidrosis
Miosis
Paralysis
Skull Base

Figure

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  Fig. 1 Magnetic resonance imaging series. Axial (A) and coronal (B) maximal intensity projections of three-dimensional time-of-flight (TOF) angiographic series demonstrating an extremely spindly left internal carotid artery (ICA). The left anterior cerebral artery (ACA) and middle cerebral artery (MCA) are supplied both by a permeable anterior communicating artery and a large posterior communicant artery (PCoA) from the basilar artery (BA). (C) Axial TOF angiographic study showing an important narrowing of the left ICA (white arrow). 1: ICA, 2: BA, 3: left PCoA, 4: ACA segment A1, 5: ACA segment A2, 6: MCA, 7: vertebral arteries.

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  Fig. 2 Heterochromia with left-iris hypopigmentation and left-sided miosis. This patient compensates for a left superior palpebral ptosis by active raising his left eyebrow.

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  Fig. 3 Unenhanced skull CT. 3D volume-rendering reconstruction of (A) the inferior face of the skull base and (B) axial view of the skull base, demonstrating a right normal carotid canal (white arrowhead) and a left highly hypoplastic carotid canal (white arrow). On axial maximal intensity projection (MIP) view of the cavernous and clinoid segment of the internal carotid artery (ICA) (C4-C5) and the sella turcica (C), it can be seen that the left hypoplastic ICA is not affected by atheromatous calcifications. MIP sagittal oblique reconstructions of the carotid canals (white arrows) demonstrate a right normal carotid canal (D) and a left highly hypoplastic carotid canal (E).

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