Successful management of severe coronoid process hyperplasia in a patient with mucopolysaccharidosis VI: a case report

Author:

Khadembaschi Darius1,Arvier John2,Beech Nicholas2,Dolan David3,McGill Jim4

Affiliation:

1. Faculty of Medicine, University of Queensland, St Lucia, Brisbane, Queensland, Australia

2. Department of Oral and Maxillofacial Surgery, Queensland Children’s Hospital, South Brisbane, Brisbane, Queensland, Australia

3. Department of Anaesthetics, Queensland Children’s Hospital, South Brisbane, Queensland, Australia

4. Department of Metabolic Medicine, Queensland Children’s Hospital, South Brisbane, Queensland, Australia

Abstract

Abstract Mucopolysaccharidoses are a group of rare lysosomal storage diseases caused by a deficiency of enzymes, which breakdown glycosaminoglycans, with consequent dysfunction of affected tissues. Mandibular coronoid hyperplasia, with associated trismus, has been recently described as a feature of the craniofacial abnormalities seen in these patients. However, the details of the surgical and post-operative management of these patients have not been previously documented. This case describes the successful management of severe trismus from coronoid process hyperplasia in a 14-year-old male, utilising an extra-oral approach for bilateral coronoidectomies and removal of exophytic zygomatic bone, followed by immediate and long-term physiotherapy. An improvement of mandibular opening from 8 to 45 mm has been maintained at 18 months post operation.

Publisher

Oxford University Press (OUP)

Subject

Surgery

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