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2015 ; 6
(2
): 58-63
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Treatment timing and multidisciplinary approach in Apert syndrome
#MMPMID26330906
Fadda MT
; Ierardo G
; Ladniak B
; Di Giorgio G
; Caporlingua A
; Raponi I
; Silvestri A
Ann Stomatol (Roma)
2015[Apr]; 6
(2
): 58-63
PMID26330906
show ga
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.