Dental approach for Apert syndrome in children: a systematic review.

BACKGROUND: Apert Syndrome (AS), or type I acrocephalosyndactyly, is a rare, congenital craniosynostosis condition resulting from missense mutations in the gene encoding fibroblast growth factor receptor 2. It is characterized by three specific clinical features: brachycephalic skull; midface hypoplasia, and limb abnormalities (syndactyly of hands and feet). The disorder exhibits variable presentations in bones, brain, skin, internal organs, and in the oral/maxillofacial region. The aim of the present paper was to show the main results from a systematic review of AS. MATERIAL AND METHODS: A search of the literature was performed from April to June 2016 in five electronic databases. Clinical interventional or observational studies, reviews, and case reports were included. The present systematic review was carried out strictly following PRISMA and Cochrane Collaboration criteria. RESULTS: A total of 129 potential references were identified. After reviewing titles and abstracts, 77 of these did not meet the desired criteria and were discarded. The full text of the remaining 52 manuscripts was critically screened. Finally, 35 relevant papers were identified for inclusion in the present systematic review and classified according to topic type. CONCLUSIONS: According to the information gathered, dentistry practitioners must be able to supply an early diagnosis through the recognition of AS clinical features and provide correct oral management. Additionally, they should be integrated in a multidisciplinary medical care team in order to improve the quality of life of the affected patients.

Rhabdomyosarcoma in children: report of two cases.

Rhabdomyosarcoma of soft tissue is the commonest sarcoma in pediatric medicine and comprises 15% of the solid tumors treated in pediatric oncology. It has an ample variety of clinical presentations in diverse organs and also presents a gamut of histopathological patterns, which sometimes make diagnosis difficult. We report two cases of rhabdomyosarcoma: one of the embryonic histological variety and the second one of the alveolar variety, which presented themselves in the preauricular and masseteric areas respectively. Both patients were male, diagnosed and treated.