Acute traumatic presentation of Chiari I malformation with central cord syndrome and presyrinx in an infant

Abstract

Background: Chiari I malformation (CM-I) typically presents in late childhood and early adulthood. Often these lesions are asymptomatic and discovered incidentally. Patients typically present with tussive headaches and focal neurological findings, especially when associated with syringomyelia. Here, an 11-month-old child with a severely symptomatic CM-I required surgery (e.g., suboccipital craniectomy and C1/2 laminectomy) within the 1st year of life. Case Description: An 11-month-old infant presented with acute bilateral upper extremity weakness following a ground-level fall. The magnetic resonance imaging of the cervical spine showed crowding at the craniocervical junction with 7 mm of cerebellar tonsillar herniation/descent, and swelling/edema of the cervical spinal cord with a presyrinx. The patient underwent an urgent suboccipital craniectomy and C1/2 laminectomy under intraoperative neuromonitoring; the motor evoked potentials in the upper and lower extremities partially recovered intraoperatively. One day postoperatively, bilateral upper extremity strength improved; 4 weeks later, he recovered full neurological function. The follow-up MR also showed complete resolution of the previously noted presyrinx. Conclusion: Acute neurological deficits may occur in infants with CM-I who, following trauma, sustain the equivalent of a central cord syndrome. Neurosurgical evaluation with MR should prompt timely/appropriate surgical decompression (e.g., suboccipital craniectomy and C1/2 laminectomy).