Apert\'s Syndrome: Report of a New Case and its Management

Abstract

Abstract:In this article, an interesting case of Apert syndrome in a 14-year-old boy with characteristic craniosynostosis, acrocephaly, midface hypoplasia, pharyngeal attenuation, ocular manifestations, and syndactyly of the hands and feet is presented.

Dental approach for Apert syndrome in children: a systematic review

“…The full-texts of the remaining 52 citations were critically reviewed. Finally, 35 relevant papers were identified for inclusion in the present systematic review; these were then classified, according to publication/topic type, into four categories ( Table 2 ) ( 1 , 2 - 6 , 9 , 12 , 14 , 18 - 20 , 22 , 23 ). The majority of these were cross-sectional studies, and one article was in Spanish language ( 19 ) ( Table 3 ) ( 7 , 8 , 10 , 11 , 13 , 15 - 17 , 21 , 35 , 36 ).…”

“…In addition, affected children with limited arm and hand mobility are considered at high risk of caries, because this restriction makes it difficult to perform and maintain proper oral hygiene ( 8 , 26 ); dental practitioners can simplify this task by suggesting floss holders, electronic brushers, or home fluoride rinses, and by asking parents to help their children when necessary ( 26 ). Taken together, these problems may affect long-term systemic/oral health, growth, and development ( 6 , 35 ); furthermore, extensive structural and functional impairments can be life-threatening ( 8 ). Therefore, the dental management of patients with AS requires an integrated care team to care for the patient from infancy to adulthood, under the guidelines from worldwide accepted current protocols designed specifically for the oral/craniofacial area, which include preventive, restorative, endodontic, orthodontic/orthopedic, and orthognathic surgical approaches.…”

“…Prior to performing any dental procedure in a patient with AS, it is essential for the dentist to be capable of recognizing the disorder early, and then, the dentist must know the child’s craniofacial and oral therapeutic needs based on his/her unique combination of features ( 6 ). To carefully develop an individualized comprehensive treatment plan, a collaborative dental team should be integrated by different specialists.…”

“…When recognized in the patient as early as 3 months of age, surgical treatment of the craniofacial region and fingers/toes may result in significant functional and cosmetic improvement ( 6 ). However, when the AS syndrome is diagnosed at later ages or treatment is delayed, many complications may arise, including the abnormal neurological development present in mental deficiency.…”

“…AS has an autosomal dominant pattern of inheritance, associated with advanced paternal age, maternal infections, maternal drug consumption, and cranial inflammatory processes ( 18 ). More than 98% of cases are caused by Fibroblast Growth FactoR (FGFR2) gene-specific missense mutations at chromosome 10q25-10q26, which are exclusively paternal in nature ( 2 , 6 , 15 , 19 , 20 ). In this regard, it has been mentioned that the probability of a second child being affected is 1%, and that a person with AS has a 50% risk of having a child with the syndrome ( 8 ).…”