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Central nervous system imaging in Crouzon's syndrome
T W Proudman1, B E Clark, M H Moore
1Australian Craniofacial Unit, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
The Journal of Craniofacial Surgery
|September 1, 1995
Summary
Crouzon syndrome patients frequently exhibit ventriculomegaly, a condition of enlarged brain ventricles, which may be a primary brain abnormality rather than a direct result of the skull suture defect. This study details central nervous system anomalies in Crouzon syndrome.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Craniofacial Surgery
Background:
- Craniosynostosis, particularly syndromic forms like Crouzon syndrome, necessitates understanding central nervous system (CNS) impacts.
- Detailed structural anatomy of the CNS in syndromal craniosynostoses is an area of growing clinical interest.
Purpose of the Study:
- To determine the frequency of CNS deformities in patients with Crouzon syndrome.
- To characterize the nature and progression of ventriculomegaly and other CNS anomalies.
- To inform the assessment and management strategies for these patients.
Main Methods:
- Clinical and radiographic review of 59 patients diagnosed with Crouzon syndrome.
- Computed tomographic (CT) scans were utilized to assess CNS structures, including ventricular size and nonventricular anomalies.
- Follow-up CT scans were analyzed to determine the progression of ventriculomegaly.
Main Results:
- Twelve percent of patients presented with decreased mental function.
- Ventriculomegaly was observed in 51% of patients, graded as mild, moderate, or severe (hydrocephalus).
- Nonventricular anomalies occurred less frequently (14%) compared to Apert syndrome, with patients showing higher mental function.
- Ventriculomegaly was often nonprogressive, even after surgical intervention to increase intracranial space.
Conclusions:
- Ventriculomegaly in Crouzon syndrome may represent a primary brain abnormality, not solely a consequence of suture defects.
- CNS findings in Crouzon syndrome differ from Apert syndrome, with fewer nonventricular anomalies and better cognitive outcomes.
- Recommendations are provided for the clinical assessment and management of CNS issues in Crouzon syndrome.