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Pattern of Closure of Skull Base Synchondroses in Crouzon Syndrome
Guillaume Coll1, Laurent Sakka2, Céline Botella3
1Unité de Chirurgie Craniofaciale, Service de Neurochirurgie Pédiatrique, Centre de Référence National des Dysostoses Crâniofaciales, Hôpital Necker-Enfants Malades, APHP, Paris, France; Service de Neurochirurgie, Hôpital Gabriel Montpied, Clermont-Ferrand, France; Image-Guided Clinical Neurosciences and Connectomics (IGCNC), Axe thérapies guidées par l'image (TGI), CNRS, Sigma, Institut Pascal, Université Clermont Auvergne, Clermont-Ferrand, France.
Insights
Skull base synchondrosis closure occurs earlier in children with Crouzon syndrome compared to controls. This premature fusion follows a specific pattern across different skull base synchondroses.
Area of Science:
- Craniofacial development
- Pediatric genetics
- Skull base surgery
Background:
- The age of skull base synchondroses closure in children with Crouzon syndrome has not been previously studied in a uniform group.
- Crouzon syndrome is associated with Fibroblast Growth Factor Receptor type 2 mutations.
Purpose of the Study:
- To analyze the age of skull base synchondroses closure in children with Crouzon syndrome.
- To compare synchondroses closure in Crouzon syndrome patients with healthy controls.
Main Methods:
- A retrospective case-control study involving 30 children with Crouzon syndrome (aged 1 month to 12.48 years) and 235 healthy children.
- Analysis of 11 synchondroses using millimetric computed tomodensitometry before surgery.
Main Results:
- Synchondrosis closure in Crouzon syndrome occurs globally earlier than in controls (P ≤ 0.002).
- Fusion begins around 10 months with posterior intraoccipital synchondroses and lambdoid sutures.
- Spheno-occipital and petro-occipital synchondroses fuse last, around 3 years of age.
Conclusions:
- Synchondrosis closure in children with Crouzon syndrome is premature.
- The timing of synchondrosis fusion is distinct for each specific synchondrosis.
Background:
The age of closure of skull base synchondroses has never been analyzed in a homogenous population of children with Crouzon syndrome.
Methods:
A retrospective case-control study was performed on 30 Crouzon children (17 male, 13 female) aged 1 month to 12.48 years with Fibroblast Growth Factor Receptor type 2 mutation. Eleven synchondroses were analyzed on millimetric computed tomodensitometric slices before surgery. Syndromic patients were compared with a series of 235 healthy children previously published.
Results:
Synchondrosis closure follows a global pattern that occurs earlier in Crouzon syndrome than in controls (P ≤ 0.002). Synchondrosis fusion starts at 10 months of age with posterior intraoccipital synchondroses and lambdoid sutures, followed by occipitomastoid synchondroses between 1.85 (right) and 2.27 years (left) and anterior intraoccipital synchondroses at approximately 2.80 years. Time to complete fusion varies considerably according to the synchondroses. Spheno-occipital and petro-occipital synchondroses fuse last, at approximately 3 years old.
Conclusions:
In children with Crouzon syndrome, synchondrosis closure occurs prematurely, with a time course specific to each synchondrosis.