Presentations 2020: Ortho&Paeds

404 - Clinical presentation and dental management of a child with primordial dwarfism

T Roberts M Ezzeldin C Williams
Presented by: Tristan Roberts
Cardiff Dental Hospital

Primordial dwarfism is a rare form of dwarfism, an autosomal recessive condition, with features manifesting at the earliest stages of development and continuing throughout growth. Its clinical presentation is highly heterogenous, with several subgroups existing. This case focuses on a boy with Microcephalic Primordial Dwarfism Type 1 (MOPD-1) which is characterised by: intrauterine growth restriction (IUGR), which continues postnatally; microcephaly; distinctive facial features; and osteodysplastic skeletal changes. The 4-year old patient initially presented to Paediatric Dentistry, with his mother expressing concern over his mobile and tender teeth. Clinical examination revealed microdontia with peg shaped teeth and protruding incisors. He was recently reviewed at 9 years of age, complaining of spontaneous pain arising from his teeth and refusal to brush as a result. His mandibular incisors were mobile as a result of periodontal clinical attachment loss, and his first permanent molars were hypoplastic. Treatment is supportive, involving reassurance, enhanced preventive care and restoration of the hypoplastic molars. This case presentation aims to increase awareness of this rare genetic condition, as well as the challenges in maintaining oral health of patients who live with primordial dwarfism.
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..

Poster