Resolution of Chiari malformation after repair of a congenital thoracic meningocele: case report and literature review.

OBJECTIVE AND IMPORTANCE

Many theories have been proposed regarding potential causative factors for Chiari malformations. An unusual case is described in which regression of a congenital Chiari malformation was observed after repair of a thoracic meningocele without direct surgical intervention to decompress the craniocervical junction. This supports the importance of an in utero craniospinal pressure gradient as a potential cause for congenital, but reversible, cerebellar herniation.

CLINICAL PRESENTATION

A newborn baby was observed to have a thoracic meningocele. Magnetic resonance imaging scan revealed a concomitant Chiari malformation. No neurological deficits were present at initial examination.

INTERVENTION

The patient underwent surgical closure of the thoracic meningocele and untethering of the spinal cord at the site of the dural defect. A postoperative magnetic resonance imaging scan obtained 3 months after the operation revealed complete resolution of the cerebellar herniation.

CONCLUSION

The resolution of the Chiari malformation in this child may have resulted from restoration of normal cerebrospinal fluid flow and elimination of the meningocele-related cerebrospinal fluid pressure gradient between the intracranial and intraspinal compartments.