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

F Floemer,J.F Schneider,C Jauckus,O Magerkurth,J Lutschg

doi:10.3174/ajnr.a0878

F Floemer, J.F Schneider + Show 3 more

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https://doi.org/10.3174/ajnr.a0878

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Abstract

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.

Highlights

Case Report A 14-year-old boy with known Klippel-Feil syndrome (KFS) clinically presented with repetitive episodes of severe occipital headache, vertigo, and nausea combined with neck and shoulder discomfort, which lasted for weeks
Multisegmental left-sided hemivertebral fusions were present at the levels C5 through T3 with a bifid spinous process, which led to a right-sided cervicothoracic angulation and scoliosis
The complete clinical triad is seen in Ͻ50% of the cases, and many patients with KFS demonstrate additional developmental anomalies

Summary

Introduction

Case Report A 14-year-old boy with known KFS clinically presented with repetitive episodes of severe occipital headache, vertigo, and nausea combined with neck and shoulder discomfort, which lasted for weeks. Clinical examination showed a markedly reduced vertebral range of motion. An SD and fusion of the left ribs 1–3 were known. Sonography demonstrated a prominent right vertebral artery without stenosis. Contrast-enhanced CT of the head, neck, and upper thorax was performed to exclude cranial abnormalities and cervical fracture

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