Presentations 2020: Ortho&Paeds

434 - A rare case of Stoneman Syndrome

M Ezzeldin S Bhatia M Collard
Presented by: Maryam Ezzeldin
Cardiff Dental Hospital

An anxious 9-year old patient with Fibrodysplasia Ossificans Progressiva (FOP) presented to Paediatric Dentistry with a history of pain and infection associated with grossly carious first permanent molars. FOP is an extremely rare autosomal dominant condition also known as Stoneman Syndrome. It is a disorder in which muscle tissue and connective tissue such as tendons and ligaments are gradually replaced by bone, forming bone outside the skeleton which progressively restricts the patient's ability to move. Ossifications can occur spontaneously or during tissue damage or trauma, therefore having significant implications on dental management. In view of the patient’s severe anxiety, comprehensive treatment was undertaken under general anaesthetic. However as a result of her limited mouth opening, anaesthetic intubation proved challenging with subsequent trauma to the patient’s anterior teeth and the need for further treatment. This case aims to raise awareness of FOP and its dental implications along with the challenges of holistic treatment as a result of social factors; highlighting the role and responsibilities of paediatric dentists and the importance of inter-disciplinary liaison.
Consent Statement: Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient/parent/guardian. A copy of the consent form is available for review by the the meeting organisers..

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