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A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI

By Guirish A. Solanki, Tord D. Alden, Barbara K. Burton, Roberto Giugliani, Dafne D.G. Horovitz, Simon A. Jones, Christina Lampe, Kenneth W. Martin, Maura E. Ryan, Matthias K. Schaefer, Aisha Siddiqui, Klane K. White and Paul Harmatz

Abstract

Cervical cord compression is a sequela of mucopolysaccharidosis VI, a rare lysosomal storage disorder, and has devastating consequences. An international panel of orthopedic surgeons, neurosurgeons, anesthesiologists, neuroradiologists, metabolic pediatricians, and geneticists pooled their clinical expertise to codify recommenda-tions for diagnosing, monitoring, and managing cervical cord compression; for surgical intervention criteria; and for best airway management practices during imaging or anesthesia. The recommendations offer ideal best prac-tices but also attempt to recognize the worldwide spectrum of resource availability. Functional assessments and clinical neurological examinations remain the cornerstone for identification of early signs ofmyelopathy, butmagnetic resonance imaging is the gold standard for identificationof cervical cord com-pression. Difficult airways of MPS VI patients complicate the anesthetic and, thus, the surgical management of cervical cord compression. All patients with MPS VI require expert airway management during any surgical pro-cedure. Neurophysiological monitoring of the MPS VI patient during complex spine or head and neck surgery is considered standard practice but should also be considered for other procedures performed with the patient under general anesthesia, depending on the lengthand type of theprocedure. Surgical interventionsmay include cervical decompression, stabilization, or both. Specific techniques vary widely among surgeons. The onset, presentation, and rate of progression of cervical cord compression vary among patients with MPS VI. The availability of medical resources, the expertise and experience of members of the treatment team, and the standard treatment practices vary among centers of expertise. Referral to specialized, experienced MPS treatment centers should be considered for high-risk patients and those requiring complex procedures. Therefore, the key to optimal patient care is to implement best practices through meaningful communication among treatment team members at each center and among MPS VI specialists worldwide

Topics: Airway Management, Cervical Cord Compression, Practice Guideline, Spinal Cord Compression, Surgical Decompression, Compressão da Medula Espinal, Descompressão Cirúrgica
Publisher: Academic Press
Year: 2012
OAI identifier: oai:agregador.ibict.br.RI_FIOCRUZ:oai:localhost:icict/9173
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