RESUMEN
PURPOSE: Congenital craniocervical segmentation anomaly associated with malalignment of the odontoid process causes progressive spinal cord compression with myelopathy. Recent literatures have reported that ventral decompression could be achieved with posterior craniocervical realignment through posterior instrumented reduction. The purpose of the present study is to show the efficacy of intraoperative electrophysiological monitoring during craniocervical realignment through screws and rod system for congenital craniocervical segmentation anomaly. METHODS: Consecutive ten patients with a congenital craniocervical segmentation anomaly and myelopathy due to a malaligned odontoid process, who underwent posterior craniocervical realignment, were included. Klippel-Feil syndrome (KFS) was combined in four patients. Gait disturbance and motor weakness were the main presentation. Craniocervical realignment was achieved by intraoperative distraction through a rod and screw system. Intraoperative neuromonitoring was performed with transcranial motor-evoked potential (TcMEP) and somatosensory-evoked potential (SSEP). RESULTS: Significant change in TcMEP occurred in two patients with KFS during surgery, but the change was reversed with release of distraction. All patients were awakened without neurological deficit. Motor weakness and gait disturbance were normalized within 6 months in all patients. Postoperative computed tomography scan and/or magnetic resonance imaging showed that the reduction was complete in all patients. CONCLUSIONS: Craniocervical realignment through screws and rod system may be safe and efficacious surgical technique for the treatment of congenital craniocervical anomaly with the help of intraoperative neuromonitoring. However, distraction should be cautiously monitored, especially when Klippel-Feil syndrome is combined.