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![Algorithm of Early Le Fort III Distraction in Craniosynostosis. [25].](profile/Kecia-Damasceno/publication/276161574/figure/fig1/AS:614147813298183@1523435657749/Algorithm-of-Early-Le-Fort-III-Distraction-in-Craniosynostosis-25.png)
Source publication
Treatment the Apert Syndrome is unique, therefore, divergences exist in the priority of the procedures performed by each team. The Apert Syndrome occurs in approximately 1/65,000 births and accounts for 4.5% of all craniosynostosis. Some changes in Apert Syndrome stand out, such as craniosynostosis and polydactyly.
Context in source publication
Context 1
... present study used three types of devices in patients according to an algorithm that we develo- ped ourselves (Figure 1). [25] Distraction techniques with a variety of external and internal distraction systems have been suc- cessfully used to advance the midface. However, external distraction devices generally cannot deliver adequate distraction forces for lengthening; as a re- sult, the distraction achieved is limited. On the other hand, internal distraction devices do not allow con- trol of the post fixation distraction vector. We have developed a hybrid facial distraction system (HFDS) leveraging the advantages of external and internal distractiondevices. However, when the advanced maxilla undergoes excessive clockwise rotation and is shifted more downward vertically than the plan- ned position, it might be impossible to pull it back into the planned position ( Figure 2). ...
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Radial polydactyly or thumb duplication is a relatively common congenital malformation of the hand, whereby the surgical techniques can be broadly divided into simple excisions, reconstructions and a Bilhaut-Cloquet procedure. The aim of this study was to identify the appropriate surgical procedures and to present the clinical outcomes that can be...
Citations
... However, the translation of this basic science research into the clinical setting remains distant and is complicated by the need for candidate therapeutics to be administered during foetal gestation. Current research applications also predominantly focus on the craniofacial aspects of Aperts Syndrome and further work is required that specifically targets hand abnormalities(Gonçalves Leite et al., 2015). ...
Introduction:
Apert Syndrome is a congenital condition characterised by primary craniosynostosis, midfacial malformations and complex symmetrical malformations of the hands and feet. The hands demonstrate one of the most complex collections of congenital upper limb deformities, posing a significant challenge for the paediatric hand surgeon. This study examines the extant literature and current practice of the four UK specialist craniofacial units regarding the management of Apert hands in order to provide a basis for guideline development.
Methods:
The current literature was reviewed. Survey-type questionnaires were distributed to the four UK specialist craniofacial units and responses analysed.
Results:
Management of the Apert hand is largely dictated by the degree of malformation present. Although all units aim to achieve a five digit hand, variation in the timing of surgery, operative protocols and mobilisation policies exist.
Conclusion:
The results of this study provide an interesting snapshot of the current management of Apert hands across four UK craniofacial surgery units. The four UK units remain congruent on most areas surrounding the management of Apert hands although some minor inter-unit variation exists. A multidisciplinary approach to management remains fundamental in optimising the regain of function and aesthetically acceptable hands.
