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Hydrocephalus in craniosynostosis: a review
H Collmann1, N Sörensen, J Krauss
1Section of Pediatric Neurosurgery, Würzburg University, Josef-Schneider-Strasse 11, 97080 Würzburg, Germany. Collmann_H@nch.uni-wuerzburg.de
Summary
Ventricular dilatation in craniosynostosis, particularly syndromic forms like Apert syndrome, often requires monitoring. True hydrocephalus in these cases is best treated with ventriculo-peritoneal shunting.
Area of Science:
- Neuroscience
- Pediatric Neurosurgery
- Medical Genetics
Background:
- Ventricular dilatation is a notable feature in syndromic craniosynostosis, affecting over 40% of patients with conditions like Crouzon's, Pfeiffer's, and Apert syndrome.
- While common in syndromic cases, it's rare in nonsyndromic craniosynostosis and often linked to other disorders.
- Hydrocephalus requiring shunting is more prevalent in Crouzon or Pfeiffer syndromes, whereas Apert syndrome typically presents with nonprogressive ventriculomegaly.
Purpose of the Study:
- To elucidate the pathogenesis and clinical significance of ventricular dilatation in primary craniosynostosis.
- To highlight diagnostic challenges and the importance of monitoring intracranial pressure and ventricular size.
- To establish effective treatment strategies for hydrocephalus associated with craniosynostosis.
Main Methods:
- Review of literature on the pathogenesis of hydrocephalus in craniosynostosis.
- Discussion of clinical evaluation methods, including challenges in diagnosing progressive hydrocephalus.
- Emphasis on pre- and postoperative monitoring of intracranial pressure and ventricular size.
Main Results:
- Pathogenesis involves factors like hypoplastic posterior fossa and venous outlet occlusion.
- Ventriculomegaly can stem from primary brain maldevelopment or increased cerebrospinal fluid (CSF) outflow resistance.
- Clinical signs of hydrocephalus may be absent, and diagnosis is often confirmed post-surgery.
Conclusions:
- Ventriculo-peritoneal shunting is the primary effective treatment for true hydrocephalus in these patients.