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Agenesis of Internal Carotid Artery in a Child with Ipsilateral Horner's Syndrome

Department of Child Neurology, Hospital Sant Joan de Déu for Children, University of Barcelona, Barcelona, Spain, cfons{at}hsjdbcn.org

Monica Vasconcelos, MD

Department of Child Neurology, Hospital Pediatrico de Coimbra, Coimbra, Portugal

Mariona Vidal, MD

Department of Ophthalmology, Hospital Sant Joan de Deu for Children, University of Barcelona, Barcelona, Spain

Ramón Puy, MD

Department of Radiology, Hospital Sant Joan de Déu for Children, University of Barcelona, Barcelona, Spain

Antonio Capdevila, MD

Department of Radiology, Hospital Sant Joan de Deu for Children, University of Barcelona, Barcelona, Spain

Laia Sanchez, MD

Department of Pediatrics, Hospital Sant Joan de Deu for Children, University of Barcelona, Barcelona, Spain

Jaime Campistol, MD, PhD

Department of Child Neurology, Hospital Sant Joan de Deu for Children, University of Barcelona, Barcelona, Spain

Horner's syndrome is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and facial anhydrosis. This case study reports a 7-year-old boy with right miosis, mild blepharoptosis, and iris hypopigmentation detected in a routine pediatric follow-up without ipsilateral facial anhydrosis, flushing, or pain. There was no history of birth trauma and test with cocaine provoked no response of the right pupil, suggesting right Horner's syndrome. Mediastinal tumor was ruled out and brain magnetic resonance imaging incidentally showed absence of flow in the right internal carotid artery. Subsequent magnetic resonance angiography demonstrated agenesis of the right internal carotid artery without other vascular-associated malformations. The final diagnosis was right, congenital Horner's syndrome due to ipsilateral internal carotid agenesis. We describe in detail the radiological findings and pathophysiological mechanisms of this unusual association.

Key Words: Horner's syndrome • internal carotid artery agenesis • magnetic resonance angiography

Journal of Child Neurology, Vol. 24, No. 1, 101-104 (2009)
DOI: 10.1177/0883073808321049


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