title: [Occipital-cervical instability in Morquio disease: a presentation of 2 cases and a review of the literature].
abstract: BACKGROUND
  Morquio syndrome is caused by an inherited autosomal recessive enzyme deficiency. It presents with numerous musculoskeletal anomalies, among which atlantoaxial instability is highlighted, due it being life-threatening. Occipital-cervical arthrodesis surgery (and decompression) is indicated in these cases.
  
  
  OBJECTIVE
  The cases of 2 patients with Morquio syndrome that required this type of surgery are presented, along with a review of the literature.
  
  
  METHODS
  Two patients: one boy and one girl, with Morquio syndrome and high cervical instability, with neurological signs and symptoms, who were subjected to surgery using decompression and instrumented occipital-cervical arthrodesis and followed up for 6 months and one year, respectively.
  
  
  RESULTS
  The neurological problems of both patients improved, and are currently having a normal life for their age.
  
  
  CONCLUSIONS
  Occipital-cervical arthrodesis with decompression provides a safe biomechanical environment that prevents neurological involvement. It should be indicated before the appearance of symptoms or mechanical instability.
authors: Servicio de Cirugía Ortopédica y Traumatología, Complejo Hospitalario Universitario de Albacete, Albacete, España.
authors: D
authors: D
authors: Ruiz-Picazo
authors: Unidad de Raquis, Servicio de Cirugía Ortopédica y Traumatología, Complejo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, A Coruña, España. Electronic address: maximoalberto.diez@usc.es.
authors: M A
authors: MA
authors: Díez-Ulloa
mesh_terms: D002574:Cervical Vertebrae / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007593:Joint Instability / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000150:complications* / Q000175:diagnosis; D009777:Occipital Bone / Q000601:surgery*; D013123:Spinal Fusion
keywords: Artrodesis occipitocervical; Estenosis medular; Inestabilidad occipitocervical; Morquio syndrome; Occipital-cervical arthrodesis; Occipital-cervical instability; Spinal stenosis; Síndrome de Morquio