Normocephalic pancraniosynostosis.
Elizabeth A Killion1, Thomas W McEwan, Arshad R Muzaffar
1From the Division of Plastic Surgery, University of Missouri, Columbia, MO.
The Journal of Craniofacial Surgery
|September 17, 2013
Summary
Normocephalic pancraniosynostosis, a rare craniosynostosis variant, often presents late with increased intracranial pressure. This report details two pediatric cases, outlining their typical clinical, radiographic, and surgical findings.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Medical Genetics
Background:
- Normocephalic pancraniosynostosis is a rare craniosynostosis variant.
- It is characterized by premature fusion of multiple cranial sutures.
- This condition is often associated with delayed diagnosis and elevated intracranial pressure.
Purpose of the Study:
- To describe the clinical course, radiographic features, and intraoperative findings in normocephalic pancraniosynostosis.
- To enhance understanding of this rare condition in pediatric patients.
- To provide insights for clinicians managing similar cases.
Main Methods:
- Case series presentation of two pediatric patients.
- Review of clinical history, physical examinations, and diagnostic imaging (e.g., CT scans).
- Analysis of intraoperative findings during surgical correction.
Main Results:
- Both cases presented with delayed diagnosis and signs of elevated intracranial pressure.
- Radiographic findings included premature fusion of multiple cranial sutures.
- Intraoperative findings correlated with the extent of synostosis and intracranial pressure.
Conclusions:
- Normocephalic pancraniosynostosis requires early recognition due to potential for increased intracranial pressure.
- Comprehensive evaluation including imaging and surgical assessment is crucial.
- Understanding common presentations aids in timely management and improved outcomes.
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