NSCSOB are a complex entity. They have in common a high risk of severe intracranial hypertension and rare hydrodynamic complications. Genetic screening is necessary to assert their non-syndromic nature. Prolonged follow-up of all craniosynostoses is necessary because unisutural synostoses can evolve into NSCSOB, and NSCSOB can evolve into craniofaciosynostosis.
Midface distraction using the external transfacial pin is a simple and safe procedure that allows an efficient correction of major facial retrusion. The external transfacial pin acts directly onto the maxilla and allows distraction without Le Fort osteotomy. By eliminating major osteotomies, it reduces the number of severe complications encountered in craniofacial surgery.
Surgical treatment of maxillonasal dysplasia or Binder syndrome is a challenge for surgeons. Its aim is to replace or substitute the missing or malformed anatomic structures. The authors report a comprehensive analysis for the understanding of the mechanisms resulting in the lack of maxillonasal development observed in Binder syndrome. The evolution of the surgical treatment due to facial function analysis is explained, and the surgical treatment of 20 consecutive cases over the last 20 years is reviewed, illustrated by three-dimensional reconstruction to emphasize the choice made and the results obtained. Bone grafts were always performed, and an arrow-shaped graft for the neospine reconstruction is described. This analysis allowed the authors to ameliorate patient self-image subsequently to facial contour improvement.
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