Successful management of severe coronoid process hyperplasia in a patient with mucopolysaccharidosis VI: a case report. Issue 1 (13th January 2020)

• Record Type: Journal Article
• Authors: Khadembaschi, Darius; Arvier, John; Beech, Nicholas; Dolan, David; McGill, Jim
• Is Part Of: Journal of surgical case reports. Volume 2020:Issue 1(2020)
• Publication Date: 2020-01-13
• Subjects: mucopolysaccharidosis -- coronoid process hyperplasia -- coronoidectomy -- trismus -- physiotherapy; Surgery -- Periodicals; Surgery -- Case studies -- Periodicals; 617.005
• DOI: 10.1093/jscr/rjz388
• Languages: English
• ISSNs: 2042-8812

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.