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Postoperative mental and morphological outcome for nonsyndromic brachycephaly
Eric Arnaud1, Pedro Meneses, Elizabeth Lajeunie
1Groupe d'Etudes des Malformations Craniofaciales, Service de Neurochirurgie, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France.
Plastic and Reconstructive Surgery
|June 28, 2002
Summary
Early fronto-orbital advancement surgery before age one is crucial for children with brachycephaly to improve mental development and prevent cognitive impairment. Genetic factors like the FGFR3 P250R mutation may influence outcomes.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Developmental Pediatrics
Background:
- Bilateral coronal synostosis leads to brachycephaly, causing functional and morphological issues.
- Intracranial hypertension in these cases can result in mental impairment.
- Fronto-orbital advancement is the standard surgical correction in infancy.
Purpose of the Study:
- To prospectively evaluate the preoperative and postoperative mental outcomes in children with isolated brachycephaly.
- To analyze factors influencing mental outcomes, including age at treatment and surgery.
- To assess the impact of the FGFR3 P250R mutation on mental and morphological results.
Main Methods:
- Prospective follow-up of 99 children with isolated brachycephaly.
- Mental status evaluation using developmental or intelligence quotients.
- Analysis of preoperative and postoperative assessments, including correlation and subgroup analysis for FGFR3 P250R mutation carriers.
Main Results:
- Preoperative mental status was significantly better in patients treated before one year of age (p < 0.001).
- Preoperative mental assessments strongly correlated with postoperative outcomes (p < 0.0001).
- Surgery before one year of age led to better postoperative mental outcomes (p < 0.02).
- While non-carriers of the FGFR3 P250R mutation showed better outcomes, differences were not statistically significant.
- Temporal bulging was a common cause of poor morphological outcomes in mutation carriers.
Conclusions:
- Early surgical intervention (before age one) is essential for optimal mental development in brachycephalic patients.
- Nonsyndromic brachycephaly patients with the FGFR3 P250R mutation may experience suboptimal mental and morphological outcomes.
- Addressing temporal bulging concurrently with fronto-orbital advancement can enhance morphological results in mutation carriers.