title: Neuroimaging findings in children with Keutel syndrome.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Section of Pediatric Neuroradiology, Division of Pediatric Radiology Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Charlotte R. Bloomberg Children's Center, Sheikh Zayed Tower, Room 4174 1800 Orleans St., Baltimore, MD, 21287-0842, USA, tbosema1@jhmi.edu.
authors.forename: Thangamadhan
authors.initials: T
authors.lastname: Bosemani
authors.forename: Ryan J
authors.initials: RJ
authors.lastname: Felling
authors.forename: Emily
authors.initials: E
authors.lastname: Wyse
authors.forename: Monica S
authors.initials: MS
authors.lastname: Pearl
authors.forename: Aylin
authors.initials: A
authors.lastname: Tekes
authors.forename: Edward
authors.initials: E
authors.lastname: Ahn
authors.forename: Andrea
authors.initials: A
authors.lastname: Poretti
authors.forename: Thierry A G M
authors.initials: TA
authors.lastname: Huisman
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000293:Adolescent; D002114:Calcinosis / Q000175:diagnosis*; D002357:Cartilage Diseases / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006228:Hand Deformities, Congenital / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis*; D059906:Neuroimaging / Q000379:methods*; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
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