NUMERO: #20230562

Título: Clinical and imaging changes in the craniofacial region in patients with pycnodysostosis: systematic review

Nome do Apresentador: Julliany Taverny SOUSA

Categoria do Trabalho: Painel de Revisões narrativas ou sistemáticas (PRN)

Área Temática: Imaginologia dentomaxilofacial

Resumo: Objective: To conduct a systematic review of the available data on pycnodysostosis (PYCD), determining clinical and imaging craniofacial characteristics. Study Design: Systematic review in specific search engines with the research question: "What are the craniofacial oral and imaging alterations in patients with pycnodysostosis?". Results: A total of 84 published articles were included, totaling 179 cases. PYCD showed a slight preference for female (55.3%), with a mean age of 14.7 years at the time of diagnosis. Clinically, there was a predominance of short stature (86.1%), brachydactyly (66%), presence of frontal cranial bossing (82.1%), exophthalmos (49.2%), and prominent nose (57.5%). Regarding the craniofacial imaging characteristics, acroosteolysis (58.1%), open cranial sutures (53.6%), and open fontanelles (67.1%), increased bone density (56.4%), and obtuse mandibular angle (84.3%) predominated. With regard to oral alterations, malocclusion (59.2%), micrognathia (62.6%), dental crowding (54.2%), narrow and grooved palate (54.2%), and dental anomalies such as impacted teeth, agenesis, delayed eruption, and enamel hypoplasia predominated. Conclusion: PYCD is a rare osteosclerotic dysplasia, which, despite genetic tests for diagnosis, reinforces the knowledge of the most common clinical and imaging characteristics to thus raise suspicion of PYCD.

Autor 1:  Julliany Taverny SOUSA

E-mail 1:  [email protected]

Autor 2:   Amanda Katarinny Goes GONZAGA

E-mail 2:  [email protected]

Autor 3 :  Hannah Gil de Farias MORAIS

E-mail 3:  [email protected]

Autor 4:  Carla Samily de Oliveira COSTA

E-mail 4:  [email protected]

Autor 5:  Roseana Almeida de FREITAS

E-mail 5:  [email protected]

Autor 6:  Lélia Maria Guedes QUEIROZ

E-mail 6:  [email protected]