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Craniosynostosis following hemispherectomy in a 2.5-month-old boy with intractable epilepsy
Mohammad-Ali Jazayeri1, John N Jensen, Sean M Lew
1Department of Neurosurgery, Medical College of Wisconsin/Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA.
Insights
This case study details a rare instance of craniosynostosis following hemispherectomy in an infant. The patient achieved seizure freedom, highlighting the importance of minimizing brain volume loss in epilepsy surgery.
Area of Science:
- Pediatric Neurosurgery
- Epilepsy Surgery
- Craniofacial Surgery
Background:
- Infantile spasms are a severe form of epilepsy in infants.
- Hemispherectomy is a radical surgical procedure for intractable epilepsy, involving removal or disconnection of one cerebral hemisphere.
- Craniosynostosis is the premature fusion of cranial sutures, affecting skull shape and brain growth.
Observation:
- A 6-week-old boy with infantile spasms underwent a right hemispherectomy at 2.5 months.
- Three months post-surgery, the patient developed left coronal craniosynostosis.
- Cranial vault remodeling successfully corrected the cosmetic deformity.
Findings:
- This is the first reported case of iatrogenic craniosynostosis secondary to hemispherectomy.
- Two potential mechanisms for the development of this complication are proposed.
- The patient remained seizure-free for four years post-surgery without medication.
Implications:
- Minimizing brain volume loss during hemispherectomy may help prevent iatrogenic craniosynostosis.
- Surgical techniques like disconnection should be considered to preserve brain volume.
- This case underscores the complex interplay between epilepsy surgery and craniofacial development in infants.
Abstract:
The authors report on the case of a 6-week-old boy who presented with infantile spasms. At 2.5 months of age, the patient underwent a right hemispherectomy. Approximately 3 months postoperatively, the patient presented with left coronal craniosynostosis. Subsequent cranial vault remodeling resulted in satisfactory cosmesis. Four years after surgery, the patient remains seizure free without the need for anticonvulsant medications. The authors believe this to be the first reported case of iatrogenic craniosynostosis due to hemispherectomy, and they describe 2 potential mechanisms for its development. This case suggests that, in the surgical treatment of infants with intractable epilepsy, minimization of brain volume loss through disconnection techniques should be considered, among other factors, when determining the best course of action.
