Article: Volume 11 Number 2 Page 67 - April 2018

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  Orth Update 2018; 11: 67-73

Orthodontics:  Nemaline Myopathy and Severe Dentofacial Deformity - a Case Report

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Abstract: Nemaline myopathy is one of the congenital muscular weakness disorders that are associated with dentofacial deformity and malocclusion. This case report describes the clinical and radiographic features of an individual with a severe form of the disease. A review of the published literature surrounding the condition helped us understand more about the aetiology of the condition and the various medical complications that are associated with it. It is hoped that this case report and our discussion will help the future management of other cases that present with a congenital myopathy or similar condition.

Clinical relevance: Muscular weakness disorders can cause severe dentofacial deformity and malocclusions. The orthodontist must understand the aetiology of the malocclusion and associated dentofacial deformity in order to offer effective management. All the clinical team needs to understand the underlying disease process and its prognosis for the patient and any treatment options considered.

Author notes: Jamie Deans, BDS, MScD, MFDS, MOrth, StR (post CCST) Orthodontics, Cardiff University Dental Hospital and Peter Durning, BDS, MDS, FDS RCS, Consultant/Senior Lecturer in Orthodontics, Cardiff University Dental Hospital, Heath Park, Cardiff CF14 4XY, UK.

Objective: To highlight the importance of reading up on medical conditions that affect many of our patients with severe malocclusions and of working with the multidisciplinary team to plan and deliver optimal care.

Kavo Kerr