White Cord Syndrome after Thoracic Cord Decompression in a Pediatric Patient

White cord syndrome (WCS) is a rare phenomenon which causes acute neurologic deterioration after a chronically compressed spinal cord is surgically decompressed, resulting in a T2-hyperintense signal to appear in the cord. We present the first case of pediatric WCS in the thoracic spine and the first to show complete resolution of symptoms.

A 3-year-old girl presented with difficulty ambulating due to pain caused by T4-8 spinal cord compression from a mass of ganglioneuromatous tissue, consistent with her previously treated mediastinal neuroblastoma. She underwent laminoplasty and microsurgical debulking of the mass. She developed severe bilateral leg weakness on postoperative day 1. Magnetic resonance imaging (MRI) showed T2 hyperintensity in the spinal cord from T1 to T10 without enhancement, concerning for WCS. She was started on high-dose steroids and monitored. Her strength improved to 3/5 in both legs prior to discharge to inpatient rehabilitation. On the latest follow-up, 4 months after surgery, she did not demonstrate any neurologic deficits and was ambulating well. Follow-up MRIs have showed continued improvement in the T2 hyperintensity.

Most cases in the literature occur in older adults with chronic cervical compression. Only 1 pediatric case, caused by a cervical arachnoid cyst, has been reported and only resulted in partial improvement. Our case is the first pediatric case of WCS to occur in the thoracic spine, the first pediatric case to demonstrate complete resolution, and the first case of any age to start to establish a length of time the compression is needed to be at risk for WCS.