BACKGROUND: Keutel syndrome is a rare autosomal-recessive condition 
characterized by abnormal cartilage calcification. Neuroimaging findings 
associated with this condition have been randomly described in the literature.
OBJECTIVE: To systematically evaluate the neuroimaging findings in a series of 
children with Keutel syndrome to broaden our base of knowledge.
MATERIALS AND METHODS: Four children with confirmed Keutel syndrome were 
reviewed for the brain, head and neck imaging findings.
RESULTS: Three of the four children, all siblings, showed evidence of moyamoya 
syndrome. All four siblings had pinna cartilage calcification.
CONCLUSION: We propose that Keutel syndrome be considered and included among the 
secondary causes of moyamoya syndrome. In children with petrified auricle and 
neurological symptoms, Keutel syndrome should be considered and brain MRI with 
MRA is required.