Multidisciplinary treatment approach of Morquio syndrome (Mucopolysaccharidosis Type IVA).

Abstract

Morquio syndrome (mucopolysaccharidosis type IVA) is an autosomal recessive disorder caused by the accumulation of mucopolysaccharides in lysosomes because of the deficiency of N-acetylgalactosamine-6-sulphate sulphatase. Patients with Morquio syndrome often need orthodontic treatment. This study demonstrates the correction of malocclusion by orthodontic treatment and the improvement of the masticatory function by prosthodontic treatment in a male patient affected by Morquio syndrome. Labial inclinations of mandibular and maxillary anterior teeth were corrected, and spaces necessary for prosthodontic restorations were preserved. After this procedure, the restorations were made, and Hawley appliances were used in both jaws for retention. Patient satisfaction and an acceptable occlusal relationship were achieved by following an optimal treatment plan when considering the general status of the patient with Morquio syndrome.

• Authors: Gökhan Onçağ, Aslihan M Ertan Erdinç, Ebru Cal
• Journal: The Angle orthodontist (Angle Orthod) Vol. 76 Issue 2 Pg. 335-40 (Mar 2006) ISSN: 0003-3219 [Print] United States
• PMID: 16539564 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Face (abnormalities, diagnostic imaging)
• Humans
• Male
• Malocclusion (diagnostic imaging, etiology, therapy)
• Mucopolysaccharidosis IV (complications)
• Orthodontics, Corrective (methods)
• Radiography