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This study reviews 10 years of Apert syndrome management, finding that posterior vault distraction osteogenesis (PVDO) in an updated algorithm reduces blood transfusions compared to fronto-orbital advancement (FOA). Complication rates and hospital stays were similar for both procedures.

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Area of Science:

  • Craniofacial Surgery
  • Pediatric Plastic Surgery
  • Orthognathic Surgery

Background:

  • Apert syndrome presents complex craniofacial and limb abnormalities requiring long-term surgical management.
  • A 10-year review of surgical strategies for Apert syndrome was conducted.
  • An updated treatment algorithm incorporating posterior vault distraction osteogenesis (PVDO) and hand reconstruction was developed.

Purpose of the Study:

  • To review a decade of surgical experience in managing Apert syndrome.
  • To evaluate an updated algorithm including PVDO and hand reconstruction.
  • To compare the efficacy and safety of PVDO versus fronto-orbital advancement (FOA) in Apert syndrome patients.

Main Methods:

  • Retrospective observational study of 69 Apert syndrome patients (2007-2017).
  • Analysis of craniofacial and upper/lower limb reconstruction surgeries.
  • Comparison of PVDO and FOA using the modified Clavien-Dindo complication scale and Student t test for blood transfusion rates.

Main Results:

  • 38 patients underwent craniofacial surgery; 210 limb operations were performed.
  • PVDO required significantly less blood transfusion volume compared to FOA (P<0.05).
  • Complication rates and hospital stay duration were comparable between PVDO and FOA.

Conclusions:

  • An updated surgical algorithm for Apert syndrome management was successfully implemented.
  • Incorporating PVDO into the treatment algorithm leads to reduced blood transfusion requirements.
  • PVDO offers a potentially safer alternative for posterior vault reconstruction in Apert syndrome.