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The Annals of Thoracic Surgery, Vol 52, 1325-1328, Copyright © 1991 by The Society of Thoracic Surgeons


ARTICLES

Perioperative spinal canal narrowing in patients with Down's syndrome

SY DeLeon, MN Ilbawi, RT Egel, PE Miller, MY Obeid, L Podorovsky, JA Quinones and RF Sulayman
Heart Institute for Children, Christ Hospital and Medical Center, Oak Lawn, Illinois.

Two patients with Down's syndrome undergoing intracardiac operations had segmental and generalized myoclonic movements postoperatively and eventual brain death. Electroencephalography in 1 patient showed no seizure despite the presence of the myoclonic movements. Computed tomographic scan showed possible cerebellar hemorrhage. Ultrasound showed cerebral edema when the pupils became fixed and dilated. Because known postoperative neurologic complications could not fully explain the clinical course, and the myoclonic movements suggested spinal origin, we considered the possibility of atlantoaxial instability causing spinal cord damage related to perioperative head and neck positioning. Postmortem study on the second patient revealed 50% reduction of the spinal canal with hyperextension and 90-degree external rotation of the head and neck. In contrast, similar maneuvers in 3 infants without Down's syndrome resulted in only mild spinal canal narrowing. Although the myoclonic movements could be explained by spinal cord compression at the atlantoaxial level, the explanation for the eventual brain death is unclear. However, kinking of the vertebral arteries related to the positioning could have caused cerebellar ischemia, hemorrhage, and increased intracranial pressure. We believe that attention to the problem might bring further answers in the future.


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Arch Pediatr Adolesc MedHome page
S. M. Pueschel
Should Children With Down Syndrome Be Screened for Atlantoaxial Instability?
Arch Pediatr Adolesc Med, February 1, 1998; 152(2): 123 - 125.
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Copyright © 1991 by The Society of Thoracic Surgeons.