Large occipito-cervical encephalocele with Chiari III malformation
S Ambekar1, B I Devi, D Shukla
1Department of Neurosurgery, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Insights
Chiari type III malformation, a rare condition, often leads to severe outcomes. This study suggests cerebrospinal fluid (CSF) shunting before encephalocele repair may improve results for large cases.
Area of Science:
- Neurology
- Neurosurgery
- Developmental Biology
Background:
- Chiari type III malformation is a rare congenital disorder.
- It is characterized by significant morbidity and mortality.
- Current treatment involves encephalocele closure, potentially with cerebrospinal fluid (CSF) shunting.
Purpose of the Study:
- To evaluate an alternative treatment approach for Chiari type III malformation.
- To investigate the efficacy of prioritizing CSF shunting for large encephaloceles.
Main Methods:
- The study involved a patient with Chiari type III malformation.
- Treatment included initial ventriculoperitoneal shunt placement.
- Subsequent excision of the encephalocele was performed.
Main Results:
- The patient underwent successful ventriculoperitoneal shunting followed by encephalocele excision.
- This sequential approach was employed for a large encephalocele.
Conclusions:
- Prioritizing CSF shunting before encephalocele repair is proposed for large cases.
- This strategy may lead to more optimal outcomes in managing Chiari type III malformations.
Abstract:
Chiari type III is the rarest of the Chiari malformations and is usually associated with high morbidity and mortality. Treatment consists of primary closure of the encephalocele with or without cerebro-spinal fluid (CSF) shunting. In our case, the patient was treated with ventriculoperitoneal shunt followed by excision of the encephalocele. We propose that large encephaloceles should be treated with CSF shunting prior to repair of the sac so as to achieve optimal result.
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