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AJNR - American Journal of Neuroradiology

Published ahead of print on November 16, 2007
doi: 10.3174/ajnr.A0878

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American Journal of Neuroradiology 29:306-307, February 2008
© 2008 American Society of Neuroradiology

Case Report
PEDIATRICS

Klippel-Feil Syndrome and Sprengel Deformity Combined with an Intraspinal Course of the Left Subclavian Artery and a Bovine Aortic Arch Variant

F. Floemer^a, O. Magerkurth^a, C. Jauckus^a, J. Lütschg^b and J.F. Schneider^a

^a Departments of Pediatric Radiology, University Children’s Hospital Basel (UKBB), Basel, Switzerland
^b Neuropediatrics, University Children’s Hospital Basel (UKBB), Basel, Switzerland

Please address correspondence to J.F. Schneider, MD, Department of Pediatric Radiology, University Children’s Hospital UKBB, Römergasse 8, 4058 Basel, Switzerland; e-mail: jacques.schneider{at}ukbb.ch

SUMMARY: We present a case of Klippel-Feil syndrome and Sprengel deformity with a bovine aortic arch and an aberrant course of the left subclavian artery in a 14-year-old boy. CT and MR imaging of the neck and upper thorax demonstrated a cervical osseous segmentation anomaly, a left common carotid artery originating from the innominate artery, and a left subclavian artery coursing through the intraspinal space at the C6 through T1 level. Possible embryonic mechanisms and clinical significance of this variant are reviewed.