Occipital-cervical instability in Morquio disease: a presentation of 2 cases and a review of the literature

• Published in: Revista española de cirugía ortopédica y traumatología 2014-11, Vol.58 (6), p.387-394
• Main Authors: Ruiz-Picazo, D, Díez-Ulloa, M A
• Format: Article
• Language: Spanish
• Subjects: Cervical Vertebrae - surgery; Child; Child, Preschool; Female; Humans; Joint Instability - diagnosis; Joint Instability - etiology; Joint Instability - surgery; Male; Mucopolysaccharidosis IV - complications; Mucopolysaccharidosis IV - diagnosis; Occipital Bone - surgery; Spinal Fusion
• ISSN: 1988-8856
• DOI: 10.1016/j.recot.2014.03.006

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. The cases of 2 patients with Morquio syndrome that required this type of surgery are presented, along with a review of the literature. 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. The neurological problems of both patients improved, and are currently having a normal life for their age. 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.