Posterior fossa craniectomy and C1/C2 laminectomy for Arnold-Chiari II decompression of syrinx

Abstract

This patient was diagnosed shortly after birth with right-sided facial palsy as a result of lower motor neuron facial nerve involvement, together with spinal abnormalities and hearing impairment (patient currently uses hearing aids). He also has 13 ribs on his left side and 11 ribs on the right, hemi-vertebra at T2/T3 and T8/T9 and fused vertebral bodies at C2/C3 and C6/C7. As a result, clinically he has a short neck with restriction of all neck movements, particularly rotation. Patient is also known to have situs inversus. From the MRI, it showed he had an Arnold-Chiari malformation. Therefore, the cerebellar tonsils herniated through the foramen magnum which resulted in disruption of the CSF flow. This lead to the formation of a syrinx within the spinal cord, the condition being known as Syringomyelia. The syrinx can expand and elongate over time, destroying part of the spinal cord so that the damage will cause the symptoms felt by the patient. Symptoms vary between patients and also depending on the location of the syrinx; in this case, the patient experienced the gait disturbances, balance problems, paraesthesia, pain and bowel control issues. His symptoms suggest a cape-like distribution of paraesthesia and sensory disturbance attributable to syringomyelia.