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Cited by 98Craniofacial Disorders and TreatmentsPectus Deformity Diagnosis and TreatmentFetal and Pediatric Neurological Disorders

Postoperative Mental and Morphological Outcome for Nonsyndromic Brachycephaly

Arnaud, Eric, Meneses, Pedro, Lajeunie, Elizabeth, Thorne, John A., Marchac, Daniel, Renier, Dominique·2002·Plastic and Reconstructive Surgery
DOI 10.1097/00006534-200207000-00002
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Abstract

Bilateral coronal synostosis causes functional and morphological problems that require fronto-orbital advancement in infancy to correct the brachycephalic deformity and to prevent mental impairment caused by the intracranial hypertension. In this study, 99 children with isolated cases of brachycephaly were prospectively followed to study their preoperative and postoperative mental outcome, which was evaluated using developmental or intelligence quotients. Several factors were analyzed: age before treatment, age at the time of surgery, and the correlation between mental assessments before and after surgery. In a subgroup or patients tested for the FGFR3 P250R mutation (n = 48), mental and morphological assessments were analyzed. Before surgery, mental status was better in the patients tested before 1 year of age (p < 0.001). The preoperative mental assessment always correlated with the postoperative assessment (p < 0.0001). The postoperative mental outcome was better when surgery was performed before the patient reached 1 year of age (p < 0.02). Although both the morphological and functional outcomes were better in the subgroup of noncarriers of the mutation, the differences were not statistically significant. Prominent bulging of the temporal fossae was frequently responsible for poor morphological outcome in carriers of the mutation. This study confirms the need for early corrective surgery before 1 year of age in brachycephalic patients to prevent impairment of their mental development. Suboptimal morphological and mental outcomes can be expected in patients with nonsyndromic brachycephaly who carry the FGFR3 P250R mutation. Primary correction of the temporal bulging should be performed in conjunction with fronto-orbital advancement to improve the morphological outcome in patients with the mutation.

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Crossref · 10.1097/00006534-200207000-00002

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  • Coronal synostosis has a worse prognosis-more repeat surgery and persistent deformity-when the Muenke syndrome mutation is present.

    “coronal synostosis has a worse prognosis (higher risk of repeat surgery and persistent deformity) in the presence of the Muenke syndrome mutation; 48,49”

    From Craniosynostosis (2011) · cites this paper as [49]

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Authors
Arnaud, E., Meneses, P., Lajeunie, E., Thorne, J. A., Marchac, D., & Renier, D.
Affiliations
Hôpital Necker-Enfants Malades (FR)
Venue
Plastic and Reconstructive Surgery
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2002-07
Volume
110
Issue
1
Pages
6-12
ISSN
0032-1052 (ISSN-L)
DOI
10.1097/00006534-200207000-00002
OpenAlex
W2011023825
Cited by
98
References
19
Citations by year
2026: 1 · 2025: 3 · 2024: 4 · 2023: 2 · 2022: 8 · 2021: 8 · 2020: 5 · 2019: 6 · 2018: 5 · 2017: 5 · 2016: 5 · 2015: 1 · 2014: 4 · 2013: 5 · 2012: 6
FWCI
1.59
Type
journal article
Language
en
Open access
closed
Locations
  • Plastic & Reconstructive Surgery (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Craniofacial Disorders and Treatments
  • Health Sciences › Medicine › Surgery › Pectus Deformity Diagnosis and Treatment
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
Keywords
coronal synostosis, fronto-orbital advancement, intellectual development, intracranial hypertension, timing of surgery, craniofacial deformities
MeSH terms
Cephalometry, Child, Child, Preschool, Craniosynostoses, Craniotomy, DNA Mutational Analysis, Esthetics, Female, Follow-Up Studies, Frontal Bone, Genetic Carrier Screening, Humans, Infant, Intelligence, Intelligence Tests, Male, Mental Status Schedule, Orbit, Postoperative Complications, Protein-Tyrosine Kinases, Receptors, Fibroblast Growth Factor, Facies, Intracranial Hypertension, Receptor, Fibroblast Growth Factor, Type 3

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