Journal of child neurology
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Review Case Reports
Agenesis of internal carotid artery in a child with ipsilateral Horner's syndrome.
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. ⋯ 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.
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The authors previously used spectral Doppler imaging to determine optic nerve blood flow velocities in normal children. In the current study, we measured central retinal artery and central retinal vein blood flow velocities by spectral Doppler imaging in 38 healthy children and 18 children with elevated intracranial pressure between ages 4 and 17. ⋯ Central retinal vein maximal blood flow velocity was also significantly reduced in children with elevated intracranial pressure; ANOVA P < .02 (normal children 4.2 cm/s [SD 0.9] versus children with elevated intracranial pressure 3.6 cm/s [SD 0.7]). Spectral Doppler imaging is a noninvasive test well tolerated in children that identifies blood flow velocity changes in elevated intracranial pressure.