Treatment timing and multidisciplinary approach in Apert syndrome.
Apert syndrome is a rare congenital disorder characterized by craniosynostosis, midface hypoplasia and symmetric syndactyly of hands and feet. Abnormalities associated with Apert syndrome include premature fusion of coronal sutures system (coronal sutures and less frequently lambdoid suture) resulting in brachiturricephalic dismorphism and impaired skull base growth. After this brief explanation it is clear that these anatomical abnormalities may have a negative impact on the ability to perform essential functions. Due to the complexity of the syndrome a multidisciplinary (respiratory, cerebral, maxillo-mandibular, dental, ophthalmic and orthopaedic) approach is necessary in treating the psychological, aesthetic and functional issues. The aim of this paper is to analyse the different functional issues and surgical methods trying to enhance results through a treatment plan which includes different specialities involved in Apert syndrome treatment. Reduced intellectual capacity is associated to the high number of general anaesthesia the small patients are subject to. Therefore the diagnostic and therapeutic treatment plan in these patients has established integrated and tailored surgical procedures based on the patients' age in order to reduce the number of general anaesthesia, thus simplifying therapy for both Apert patients and their family members.
Department of Oral and Maxillofacial Sciences, Maxillofacial Surgery Unit, Policlinico Umberto I, "Sapienza" University of Rome, Italy.
Maria Teresa
MT
Fadda
Department of Oral and Maxillofacial Sciences, Pediatric Unit, "Sapienza" University of Rome, Italy.
Gaetano
G
Ierardo
Department of Oral and Maxillofacial Sciences, Pediatric Unit, "Sapienza" University of Rome, Italy.
Barbara
B
Ladniak
Department of Oral and Maxillofacial Sciences, Pediatric Unit, "Sapienza" University of Rome, Italy.
Gianni
G
Di Giorgio
Department of Oral and Maxillofacial Sciences, Maxillofacial Surgery Unit, Policlinico Umberto I, "Sapienza" University of Rome, Italy.
Alessandro
A
Caporlingua
Department of Oral and Maxillofacial Sciences, Maxillofacial Surgery Unit, Policlinico Umberto I, "Sapienza" University of Rome, Italy.
Ingrid
I
Raponi
Department of Oral and Maxillofacial Sciences, Orthognathodontic Unit, "Sapienza" University of Rome, Italy.
Alessandro
A
Silvestri
Apert syndrome; congenital disorders; multidisciplinary approach