Left Ophthalmic Segment Internal Carotid Artery Aneurysm Treated with Flow Diversion in a Child with Apert Syndrome:...

Prevalence of intracranial aneurysms in children with Apert syndrome has not been described, and development of an aneurysm as a complication secondary to craniofacial surgery has never been reported.

We report the rare case of a 10-year-old boy with Apert syndrome who underwent craniofacial reconstruction surgery consisting of subcranial Le Fort III osteotomies, bilateral lateral canthopexies, and nasal nares dilations for midfacial hypoplasia and resultant obstructive sleep apnea, and on routine follow-up magnetic resonance imaging (MRI) 1 year later, he was found to have a large left ophthalmic internal carotid artery (ICA) aneurysm that was not seen on MRI obtained 2 years prior. Immediately after the craniofacial surgery, the patient experienced a severe headache behind his left eye and extraocular movement abnormalities that subsided over the next days to months. Given the new and rapid growth of the aneurysm on follow-up MRI, the patient underwent a diagnostic cerebral angiogram followed by successful flow diversion treatment of the aneurysm with the pipeline embolization device (Medtronic, Dublin, Ireland).

Post-procedurally, over the next year, the patient developed word-finding difficulty and stuttering speech. He was found to have in-stent ICA stenosis and middle cerebral artery (MCA) stenosis at the first follow-up and underwent an initial angioplasty. After several weeks, ICA, MCA, and anterior cerebral artery stenoses were identified, and the patient underwent angioplasties for the ICA and MCA stenoses. On follow-up examination after the second procedure, the patient had tremendous improvement in his speech difficulties and was doing well clinically.