Dental Manifestations in a Female Patient with Apert's Syndrome

Year 2018, Volume: 1 Issue: 1, 9 - 12, 01.01.2018

Ayşe Feyda Nursal Kemal Tümer Serbülent Yiğit Akın Tekcan

Abstract

Viral
Apert's syndrome (AS) is a rare congenital disorder with autosomal dominant
inheritance and is characterized by irregular craniosynostosis, syndactylia of
hands and feet, mid-face hypoplasia, hypertelorism and anomalies of central
nervous system, heart and kidneys. AS has been associated with mutations in
Fibroblast growth factor receptor 2 (FGFR2) located on chromosome 10q (10q26).
Dental anomalies are common in AS. We report on a 6-year-old AS patient with
complex dental anomalies. A 6 year-old female patient with AS was presented to
the dental clinic with complaints of teeth decay and embedded teeth. She had
dysmorphic facial symptoms including mid-face hypoplasia, low-set ears,
hypertelorism, prognathic mandible, steep wide forehead, down-slanting lateral
canthi and palpebral fissures. She had syndactyly of third and fourth digits of
both hands. Arachnoidal cyst was diagnosed previously. She had intellectual
disability. Radiography showed that there were more than one embedded teeth.
Upper first premolar and canine teeth were displaced. She had teeth agenesis of
the maxillary lateral incisor. Her maxilla and mandible were narrow. The
maxillary dental arch was v-shaped. Orthodontic treatment was planned for the
future because the patient was too young. The aim of the present report is to
show the dental manifestations in case with AS. The treatment and management of
AS require a multidisciplinary approach.

Keywords

Apert's syndrome, Dental anomalies, Orthodontic treatment

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