Maroteaux-lamy syndrome (mucopolysaccharidosis VI) with abnormal coronoid and condylar processes of the mandible: Report of a case with surgical intervention

Abstract

Maroteaux-Lamy syndrome (mucopolysaccharidoses VI) is a rare genetic disease results in functional deficiency of arylsulfatase B and accumulation of dermatan sulfate within lysosomes in various tissues. Here we present an 8-year-old male who was referred for treatment of limited mouth opening. The patient had been diagnosed with mucopolysaccharidoses VI at age of 6 months. He had received bone marrow transplantation at age of 3.5 years, developed graft-versus-host disease and subsequent vitiligo-like changes. The patient presented with short stature, and some skeletal anomalies. In radiographic images bilateral hyperplasia of the coronoid process, underdeveloped condyles, and dentigerous cyst-like follicles in the jaws were noted. Bilateral coronoidectomy was performed to improve the maximum opening of the mouth. Post-op follow-up examinations in 5 years showed moderate improvement. Abnormal coronoid and condylar processes are unusual in MPS VI. Proper management is needed to improve patients’ oral and general health for the remainder of their lives.