BACKGROUND: @entity2168 is caused by mutations predominantly in the @entity7991 gene leading to @entity91 and @entity2094 . Monobloc distraction aims to correct both functional and aesthetic disharmony as a result of @entity2094 . This study evaluates the corrective effects and effectiveness of monobloc distraction in @entity2168 @entity1 . METHODS: Preoperative and postoperative scans were collected from 20 Crouzon and two Pfeiffer @entity1 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 @entity1 skulls. Color maps were created to visualize differences among the average normal, preoperative, and postoperative @entity2168 @entity1 . RESULTS: In the studied @entity1 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 @entity1 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.

Evaluating the Efficacy of Monobloc Distraction in the XXXX Using Geometric Morphometrics.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity2168 :: ('MESH:D003394', 'Disease') :: ['Crouzon-Pfeiffer syndrome', 'Crouzon-Pfeiffer'] @entity7991 :: ('2263', 'Gene') :: ['FGFR2'] @entity2094 :: ('MESH:C537559', 'Disease') :: ['midfacial hypoplasia'] @entity91 :: ('MESH:D003398', 'Disease') :: ['syndromic craniosynostosis']
@entity2168 :: (MESH:D003394,Disease) :: ['Crouzon-Pfeiffer Craniofacial Deformity']
