脳障害と治療

概要

Extradural Decompression for the Treatment of Oculomotor and Oropharyngeal Symptoms in Chiari I Malformation: A Case Report

Elvira A. Allakhverdieva, Ryan A. Grant, Jennifer L. Quon, Adele S. Ricciardi and Michael L. Diluna

Background and importance: Chiari I malformation (CIM) represents a subset of neurological disorders characterized by herniation of cerebellar tonsils below the foramen magnum. Importantly, it is associated with various motor and sensory dysfunctions. Extradural decompression is one of the most promising surgical approaches for treating CIM. Compared to more invasive techniques, a purely extradural decompression allows for the timely resolution of clinical symptoms as well as reduces the risk of postoperative complications.

Clinical presentation: We report the case of an 8-month old patient presenting with nystagmus, esotropia, laryngomalacia, and sleep apnea secondary to CIM. The patient underwent an extradural decompression, which involved removal of the posterior ring of C1 in addition to the outer dural layer. The patient had a gradual resolution of his symptoms over a several month period. After 4 months, the patient`s esotropia and laryngomalacia resolved and his motor nystagmus and sleep apnea also improved significantly. His delayed visual maturation continues to improve.

Conclusion: Extradural decompression has the potential to be used as a first-line treatment for symptomatic CIM. Our case exemplifies how extradural decompression can be used, in a pediatric patient, to successfully treat visual and oropharyngeal deficits associated with CIM.