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. 2015 Jul 28;6(2):58-63.
eCollection 2015 Apr-Jun.

Treatment timing and multidisciplinary approach in Apert syndrome

Maria Teresa Fadda  1 , Gaetano Ierardo  2 , Barbara Ladniak  2 , Gianni Di Giorgio  2 , Alessandro Caporlingua  1 , Ingrid Raponi  1 , Alessandro Silvestri  3 ; Group of Apert syndrome, Policlinico Umberto I, “Sapienza” University of Rome, Italy
Affiliations

Treatment timing and multidisciplinary approach in Apert syndrome

Maria Teresa Fadda et al. Ann Stomatol (Roma). .

Abstract

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.

Keywords: Apert syndrome; congenital disorders; multidisciplinary approach.

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Figures

Figure 1
Figure 1
Orbital dystopia, hypertelorism, eyelid antimongoloid and globous nose in a patient affected by Apert syndrome.
Figure 2
Figure 2
Intraoral features in Apert syndrome. A: cleft palate, V-Shaped maxillary dental arch, shovel-shaped incisors; B: open bite, Angle class III malocclusion, crossbite, dental crowding, eruption anomalies.
Figure 3
Figure 3
A and B Severe syndactyly of a hand with complete fusion of all five digits at the level of the terminal and middle phalanges, the hand has a cup-like appearance.
Figure 4
Figure 4
Fronto-orbital advancement.
Figure 5
Figure 5
Facial bipartition.
Figure 6
Figure 6
Middle third advancement A:Le Fort III osteotomy line; B: external distractor device applied in a patients after a Le Fort III procedure. C: internal distractor device applied in a patient after Le Fort III osteotomies.
Figure 7
Figure 7
Third middle advancement in a 8 year-old child with external distraction. A: Pre surgical, B: Post surgical outcome.
Figure 8
Figure 8
Inferior Third surgery: A: Le Fort I osteotomies and advancement; B: Bilateral split osteotomy of mandible.
Figure 9
Figure 9
Orthodontic presurgical treatment. In the picture it could be noticed the presence of a palate incisor.
See this image and copyright information in PMC

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