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Craniosynostosis and Chiari I Malformation Managed With Middle 1/3 Calvarial Vault Expansion
Evangeline Bambakidis1, Jitsupa Sirinit2, Howard D Wang1,2,3
1Case Western Reserve University School of Medicine.
Insights
Craniosynostosis can cause Chiari malformation. The Pi-Hung Span procedure for middle 1/3 vault expansion successfully treated 3 children with craniosynostosis and Chiari malformation, improving symptoms and radiographic outcomes.
Area of Science:
- Neurosurgery
- Pediatric Neurosurgery
- Craniofacial Surgery
Background:
- Craniosynostosis, particularly multisutural syndromic forms, is linked to secondary Chiari malformation.
- Single suture synostosis can also cause Chiari malformation due to craniocephalic disproportion.
- Traditional posterior vault expansion may worsen scaphocephaly in sagittal synostosis and may be insufficient for Chiari malformation in growing children.
Purpose of the Study:
- To describe the successful use of the Pi-Hung Span procedure for middle 1/3 vault expansion in 3 pediatric cases.
- To evaluate the efficacy of this technique in treating craniosynostosis with concurrent Chiari malformation.
- To assess the impact on scaphocephaly and tonsillar herniation.
Main Methods:
- The study involved 3 pediatric patients with craniosynostosis (2 isolated sagittal, 1 sagittal with partial lambdoid) and Chiari malformation.
- Treatment utilized the Pi-Hung Span procedure for middle 1/3 vault expansion.
- Follow-up ranged from 6 to 18 months, assessing clinical symptoms and radiographic evidence of tonsillar herniation.
Main Results:
- All 3 patients showed symptomatic improvement following the Pi-Hung Span procedure.
- Radiographic evaluation demonstrated improvement in tonsillar herniation in all treated patients.
- The procedure avoided craniotomy over the torcular and transverse sinus, reducing the risk of sinus injury.
Conclusions:
- Middle 1/3 vault expansion using the Pi-Hung Span procedure is a viable surgical option for children with Chiari malformation secondary to craniosynostosis.
- This technique effectively addresses craniocephalic disproportion and prevents worsening scaphocephaly.
- It offers a comprehensive reconstruction for overall anatomic disproportion during skull growth, improving neurodevelopmental outcomes.
Abstract:
Multisutural syndromic craniosynostosis is strongly associated with secondary Chiari malformation. Although less commonly described, single suture synostosis can also be associated with secondary Chiari malformation due to craniocephalic disproportion. These children have traditionally been treated with posterior vault expansion; however, in patients with sagittal synostosis, increasing only the posterior vault volume can worsen the scaphocephalic deformity. In addition, treating a Chiari malformation with a decompression without addressing the craniocephalic disproportion during a period of continued skull growth can be insufficient for the future neurodevelopment of the patient. Our study describes 3 cases of craniosynostosis (2 isolated sagittal, 1 sagittal with partial lambdoid-upper 1/3 of the suture) with concurrent Chiari malformation that were successfully treated with middle 1/3 vault expansion using the Pi-Hung Span procedure. As this procedure avoids a craniotomy over the torcular and transverse sinus, this method also decreases the risk of sinus injury during surgery. All 3 patients, with 6 to 18 months of follow-up, experienced both symptomatic improvement and radiographic improvement of tonsillar herniation. Supratentorial vault expansion should be considered as a viable surgical treatment for children with Chiari malformation secondary to craniosynostosis to prevent worsening scaphocephaly and to provide a complete reconstruction for the overall anatomic disproportion in the setting of continued skull growth.
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