The Journal of Bone and Joint Surgery (American) 84:277-288 (2002)
© 2002 The Journal of Bone and Joint Surgery, Inc.
Congenital Osseous Anomalies of the Upper and Lower Cervical Spine in Children
James T. Guille, MD and
Henry H. Sherk, MD
Investigation performed at the Alfred I. duPont Hospital
for Children, Wilmington, Delaware, and MCP Hahnemann University
School of Medicine, Philadelphia, Pennsylvania
James T. Guille, MD
Department of Orthopaedics, Alfred I. duPont Hospital for Children,
P.O. Box 269, Wilmington, DE 19899
Henry H. Sherk, MD
Department of Orthopaedic Surgery, Medical College of Pennsylvania
Hospital, 3300 Henry Avenue, Philadelphia, PA 19129
The authors did not receive grants or outside funding in support
of their research or preparation of this manuscript. They did not
receive payments or other benefits or a commitment or agreement
to provide such benefits from a commercial entity. No commercial
entity paid or directed, or agreed to pay or direct, any benefits
to any research fund, foundation, educational institution, or other
charitable or nonprofit organization with which the authors are affiliated
or associated.
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Introduction
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Mutations of homeobox genes may be responsible for congenital
osseous anomalies of the cervical spine.
Congenital osseous anomalies of the cervical spine may herald
congenital malformations of other organ systems, such as those of
the kidney and heart.
Most congenital anomalies of the cervical spine are innocuous
and may go undetected throughout life.
Translational instability of adjacent vertebral bodies in a congenitally
stenotic cervical spinal canal has serious implications.
No existing prognostic classification system can predict which
patients with a congenital osseous anomaly of the cervical spine
are at risk for future neurologic injury.
Congenital osseous anomalies of the upper and lower cervical
spine are rare, yet their detection and treatment are important,
as these deformities often set the stage for cervical spine instability
and potential neurologic injury. Many of these conditions are associated
with various syndromes and are, in part, developmental conditions.
The presence of a congenital osseous anomaly . . . [Full Text of this Article]

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