Parry Romberg Syndrome. An unusual case with dental features.
Creators
- 1. Orthodontic Department, Leeds Dental Institute, Leeds, UK
- 2. Oral and Maxillofacial Surgery Department, Leeds Dental Institute, Leeds, UK Parry Romberg Syndrome. An unusual case with dental features. Citation: Tk Mittal, H Safaei, L Carter and Cj Bates. (2020). Parry Romberg Syndrome. An unusual case with dental features. Journal of Oral Care and Dentistry 2(1). Parry-Romberg syndrome was first described by Parry (Parry, 1825) and Romberg (Romberg, 1846). It is also known as progressive hemifacial atrophy, progressive orofacial hemiatrophy, and has been described as having clinical overlap with Morphea en coup de sabre or linear scleroderma (Toussaint et al., 2010; Maletic et al., 2010; Tollefson and Witman, 2007; De Somer et al., 2015). The aetiology of this rare disorder is unknown but thought to be of autoimmune nature due to favourable response to immunosuppressive drugs (Korkmaz et al., 2005). The presence of the disease in one of two twins reduces the likelihood of a genetic aetiology (Hulzebos et al., 2004) although some familial association has been reported in two first cousins, whose fathers were non-identical twins and mothers were sisters (Anderson et al., 2005). The disease is self-limiting and although a skin and subcutaneous disease, will progress to the underlying muscles, cartilage and hard tissues. Parry-Romberg Syndrome typically presents in children or adolescents and has a slow, progressive nature until it stabilises(Rogers, 1964). Following initial clinical features it may become apparent that there are areas of hyper or hypopigmented skin which may be present on the ipsilateral or contralateral side of the facial deformity. Another early clinical feature may be the presence of a depressed vertical scar along the centre of the forehead known as an "en coup de sabre". (Stone, 2006; Cory et al., 1997) The facial deformity as a result of the syndrome can vary in severity, dependant on when the disease begins. If during early childhood, during rapid growth, the asymmetry can be marked and severe. The atrophy can result in restriction of growth of hard tissues in all three dimensions. The mandible and maxilla are commonly affected, but up to 20% of individuals suffering with Parry Romberg Syndrome have disorders of other limbs or the contralateral craniofacial area Abstract
Description
Abstract
Parry-Romberg syndrome, also known as progressive hemi facial atrophy has been described as having clinical overlap with Morphea en coup de sabre or linear scleroderma. The aetiology of this rare disorder is believed to be of auto-immune origin and presents with both intra-oral and extra-oral deformities in the craniofacial region.There is currently little information available in the literature discussing the dental features and orthodontic management of patients with Parry-Romberg syndrome. Here we hope to raise awareness in the dental community regarding the presenting features and management of Parry-Romberg syndrome. We have outlined the treatment and management the patient received from both Maxillofacial and Orthodontic departments over a six year period.
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JOCD-02-0013.pdf
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