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Evaluating the Efficacy of Monobloc Distraction in the Crouzon-Pfeiffer Craniofacial Deformity Using Geometric Morphometrics.
Visser, Richard; Ruff, Clifford F; Angullia, Freida; Ponniah, Allan J T; Jeelani, N U Owase; Britto, Jonathan A; Koudstaal, Maarten J; Dunaway, David J.
Afiliación
  • Visser R; London, United Kingdom; and Rotterdam, The Netherlands.
  • Ruff CF; From the Department of Craniofacial Surgery, Great Ormond Street Hospital for Children; the Department of Medical Physics and Bioengineering, University College London Hospital; and the Department of Oral and Maxillofacial Surgery, Erasmus Medical Centre.
  • Angullia F; London, United Kingdom; and Rotterdam, The Netherlands.
  • Ponniah AJT; From the Department of Craniofacial Surgery, Great Ormond Street Hospital for Children; the Department of Medical Physics and Bioengineering, University College London Hospital; and the Department of Oral and Maxillofacial Surgery, Erasmus Medical Centre.
  • Jeelani NUO; London, United Kingdom; and Rotterdam, The Netherlands.
  • Britto JA; From the Department of Craniofacial Surgery, Great Ormond Street Hospital for Children; the Department of Medical Physics and Bioengineering, University College London Hospital; and the Department of Oral and Maxillofacial Surgery, Erasmus Medical Centre.
  • Koudstaal MJ; London, United Kingdom; and Rotterdam, The Netherlands.
  • Dunaway DJ; From the Department of Craniofacial Surgery, Great Ormond Street Hospital for Children; the Department of Medical Physics and Bioengineering, University College London Hospital; and the Department of Oral and Maxillofacial Surgery, Erasmus Medical Centre.
Plast Reconstr Surg ; 139(2): 477e-487e, 2017 Feb.
Article en En | MEDLINE | ID: mdl-28121883
BACKGROUND: Crouzon-Pfeiffer syndrome is caused by mutations predominantly in the FGFR2 gene leading to syndromic craniosynostosis and midfacial hypoplasia. Monobloc distraction aims to correct both functional and aesthetic disharmony as a result of midfacial hypoplasia. This study evaluates the corrective effects and effectiveness of monobloc distraction in Crouzon-Pfeiffer patients. METHODS: Preoperative and postoperative scans were collected from 20 Crouzon and two Pfeiffer patients aged 7 to 20 years. Fifty-six normal skulls were used as a control group. Geometric morphometrics using 52 frontofacial landmarks were used to analyze the normal skull and preoperative and postoperative patient skulls. Color maps were created to visualize differences among the average normal, preoperative, and postoperative Crouzon-Pfeiffer patients. RESULTS: In the studied patient population, monobloc distraction with the use of an external distractor advanced the upper half of the midface more than the lower half of the midface. There was an anteroinferior rotation in the monobloc segment. The zygomatic arch length improved on average to 88 and 90 percent of normal (right and left, respectively), whereas globe protrusion was corrected from 134 percent to 84 percent and from 131 percent to 87 percent of normal (right and left, respectively) in the studied patient population. Compared with a normal skull, the maxillary region remained retruded. CONCLUSIONS: The advancement achieved by monobloc distraction is effective in the upper half of the midface; the lower half of the midface is advanced but remains retruded in comparison with the normal population. The midface is rotated anteroinferiorly. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Acrocefalosindactilia / Osteogénesis por Distracción Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Plast Reconstr Surg Año: 2017 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Acrocefalosindactilia / Osteogénesis por Distracción Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Plast Reconstr Surg Año: 2017 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Estados Unidos