Houston
Medical
Case
Summary
Arnold-Chiari
Malformation Complicated by Syringomyelia and Basilar Invagination
Arnold-Chiari Malformation
is a congenital condition in which the hindbrain protrudes downward
into the upper part of the spinal canal and pushes on vital structures
in the brain stem. The brain stem controls breathing, blood pressure,
heart rate and functions of the spinal cord. The cord controls movement,
walking, feeling, sensation. Syringomyelia is
a cyst in the upper spinal cord. Electrophysiological stimulation studies
of arms and legs showed impaired function of the spinal cord on right
side.
Basilar invagination,
is an upward displacement of a peg bone from the second cervical vertebra.
This area is critical to proper head movements.
Without treatment,
the changes in patient's spinal cord, brain and spinal column could
lead to increased difficulty eating/talking/swallowing, progressive
scoliosis, progressive weakness of the arms and legs and ultimately,
death.
Treatment/outcome:
Treatment involved decompressing the Chiari Malformation by enlarging
the area in which it sits, and fusing the high cervical spine to the
base of the skull. The fusion arrests the basilar invagination, while
the decompression relieves the pressure that causes headaches and the
cyst in the cervical spinal cord.
The combination
of conditions in the same patient is unusual. The procedures used had
to be modified to accomplish the treatment of both conditions. The outcome,
except for a superficial wound infection, was successful.
Physicians
Dr.
Ian Butler is a board-certified pediatric neurologist associated
with The University of Texas Medical School at Houston.
Dr.
James Baumgartner is a pediatric neurosurgeon specializing in pediatric
epilepsy and craniofacial surgery. He is associated with The University
of Texas Medical School at Houston and He is board-certified in neurosurgery.
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