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Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe

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Evolution of tonsillar ectopia associated with frontal encephalocoele.

Dharmendra Ganesan¹, Richard D Hayward, Dominic N Thompson

¹Department of Neurosurgery, Great Ormond Street Children's Hospital, Great Ormond Street, London, WC1N 3JH, UK.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery

|February 25, 2009

Summary

Chiari I malformation may be acquired, not congenital. An infant with a frontonasal encephalocoele developed tonsillar ectopia due to impaired cranial growth, suggesting an acquired cause for this condition.

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Area of Science:

Neuroscience
Developmental Biology
Pediatric Neurology

Background:

Chiari I malformation is traditionally viewed as a congenital condition.
Emerging evidence suggests acquired mechanisms contribute to its development.
This case presents a unique example supporting an acquired etiology.

Observation:

Fetal MRI revealed a frontonasal encephalocoele at 28 weeks gestation.
Post-natal MRI showed cerebellar tonsillar ectopia in a 7-week-old infant.
No hydrocephalus was present; tonsillar herniation occurred during the third trimester/neonatal period.

Findings:

A hypothesized mechanism involves the encephalocoele dampening brain growth and cerebrospinal fluid pulsations.
This leads to diminished cranial growth and a small posterior fossa.
Cerebellar growth spurt post-third trimester results in tonsillar ectopia due to cephalocranial disproportion.

Implications:

This case challenges the congenital view of Chiari I malformation.
It highlights the potential for acquired factors in its pathogenesis.
Understanding these mechanisms may inform future diagnostic and therapeutic approaches.