HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Spinal cord infarction remote from maximal compression in a patient with Morquio syndrome.

Abstract
Morquio syndrome, or mucopolysaccharidosis type IV, is a rare enzyme deficiency disorder and results in skeletal dysplasia. Odontoid dysplasia is common among affected patients, resulting in atlantoaxial instability and spinal cord compression. Surgical treatments include decompression and prophylactic fusion, during which intraoperative neuromonitoring is important to alert the surgical team to changes in cord function so that they can prevent or mitigate spinal cord injury. This report describes a 16-year-old girl with Morquio syndrome who developed paraplegia due to thoracic spinal cord infarction during foramen magnum and atlantal decompression. This tragic event demonstrates the following: 1) that patients with Morquio syndrome are at risk for ischemic spinal cord injury at levels remote from areas of maximal anatomical compression while under anesthesia in the prone position, possibly due to impaired cardiac output; 2) the significance of absent motor evoked potential responses in the lower limbs with preserved upper-limb responses in an ambulatory patient; 3) the importance of establishing intraoperative neuromonitoring baseline assessments prior to turning patients to the prone position following induction of anesthesia; and 4) the importance of monitoring cardiac output during prone positioning in patients with chest wall deformity.
AuthorsCalvin K W Tong, James C H Chen, D Douglas Cochrane
JournalJournal of neurosurgery. Pediatrics (J Neurosurg Pediatr) Vol. 9 Issue 6 Pg. 608-12 (Jun 2012) ISSN: 1933-0715 [Electronic] United States
PMID22656250 (Publication Type: Case Reports, Journal Article)
Topics
  • Adolescent
  • Decompression, Surgical
  • Female
  • Humans
  • Infarction (etiology, pathology, surgery)
  • Magnetic Resonance Imaging
  • Mucopolysaccharidosis IV (complications, pathology)
  • Paraplegia (etiology, pathology, surgery)
  • Spinal Cord Compression (etiology, pathology, surgery)
  • Thoracic Vertebrae
  • Thoracic Wall (pathology)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: