Treatment of Klippel-Feil syndrome with symptomatic atlantoaxial instability in a 7-year-old boy: A case report

TL;DR

A 7-year-old boy with Klippel-Feil syndrome was successfully treated for spinal instability and myelopathy using a surgical procedure involving decompression and stabilization.

Contribution

The paper presents a successful treatment approach for juvenile Klippel-Feil syndrome with symptomatic atlantoaxial instability.

Findings

The patient showed improvement after C1 decompression and CT-guided C1/C2 stabilization.

Intraoperative neuromonitoring was safely used during the procedure.

Symptomatic myelopathy due to C1 posterior arch stenosis was effectively addressed.

Abstract

Klippel-Feil syndrome (KFS) is a congenital deformity of the cervical spine. Clinical symptoms of KFS are reduced range of motion, short neck and low hairline. In adult KFS patients the deformity can lead to adjacent segmental instability with spinal canal stenosis, radiculopathy and myelopathy. This article reports about the diagnostics and treatment management of juvenile KFS patient with myelopathy due to instability of the C1/C2 segment, subsequent stenosis through the posterior arch of C1 and symptomatic myelopathy. This 7‑year-old boy could be successfully treated with C1 decompression and computer tomography (CT) guided C1/C2 stabilization with pedicle screws under intraoperative neuromonitoring.

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