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

Abstract

INTRODUCTION:

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.

PATIENTS AND 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.

CONCLUSION:

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: D Ruiz-Picazo, M A Díez-Ulloa
• Journal: Revista espanola de cirugia ortopedica y traumatologia (Rev Esp Cir Ortop Traumatol) 2014 Nov-Dec Vol. 58 Issue 6 Pg. 387-94 ISSN: 1988-8856 [Electronic] Spain
• Vernacular Title: Inestabilidad occipitocervical en la enfermedad de Morquio. Presentación de 2 casos y revisión de la literatura.
• PMID: 24954140 (Publication Type: Case Reports, Journal Article, Review)
• Copyright: Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

Topics

• Cervical Vertebrae (surgery)
• Child
• Child, Preschool
• Female
• Humans
• Joint Instability (diagnosis, etiology, surgery)
• Male
• Mucopolysaccharidosis IV (complications, diagnosis)
• Occipital Bone (surgery)
• Spinal Fusion