Apert syndrome is a complex autosomal dominant genetic disorder, with early closure of cranial and facial sutures, midface retrusion, and limb anomalies. The syndrome is well known as acrocephalosyndactyly type I. Treatment protocols remain a topic of discussion in relation to the complexity of the deformity, the timing and nature of the surgical approach, and the functional and cognitive outcomes. Frontofacial monobloc advancement (FFMBA) is a surgical treatment option. However, posterior cranial vault decompression or frontal–orbital advancement should be considered early in surgical treatment. The benefits of FFMBA include good eye protection, increased airway dimensions, improved respiratory problems, and avoidance of corneal lesions. The facial osteotomies for FFMBA should be performed with extreme care on the cribriform plate and pterygoid plate to permit the gradual mobilization of the facial bone. External distractors, when used, can provide better vector traction for facial bones in the orbital and maxillary regions. This chapter discusses differences between internal and external distractors. Both devices have inherent complications, but the major complications found in our series were related to facial osteotomies rather than the type of distractors used.

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Monobloc Advancement with External Distraction

  • Nivaldo Alonso,
  • Cristiano Tonello

摘要

Apert syndrome is a complex autosomal dominant genetic disorder, with early closure of cranial and facial sutures, midface retrusion, and limb anomalies. The syndrome is well known as acrocephalosyndactyly type I. Treatment protocols remain a topic of discussion in relation to the complexity of the deformity, the timing and nature of the surgical approach, and the functional and cognitive outcomes. Frontofacial monobloc advancement (FFMBA) is a surgical treatment option. However, posterior cranial vault decompression or frontal–orbital advancement should be considered early in surgical treatment. The benefits of FFMBA include good eye protection, increased airway dimensions, improved respiratory problems, and avoidance of corneal lesions. The facial osteotomies for FFMBA should be performed with extreme care on the cribriform plate and pterygoid plate to permit the gradual mobilization of the facial bone. External distractors, when used, can provide better vector traction for facial bones in the orbital and maxillary regions. This chapter discusses differences between internal and external distractors. Both devices have inherent complications, but the major complications found in our series were related to facial osteotomies rather than the type of distractors used.