Spinal canal decompression for hypertrophic neuropathy of the cauda equina with chronic inflammatory demyelinating polyradiculoneuropathy: A case report

BACKGROUND: Hypertrophic neuropathy of the cauda equina (HNCE) is a rare disease, especially in children. It can be caused by different etiological agents such as inflammation, tumor or hereditary factors. Currently, there is no uniform standard for clinical treatment of HNCE. Furthermore, it is unclear whether spinal canal decompression is beneficial for patients with HNCE. CASE SUMMARY: We report the case of a 13-year-old boy with enlargement of the cauda equina. The onset of the disease began at the age of 6 years and was initially marked by radiating pain in the buttocks and thighs after leaning over and weakness in the lower limbs when climbing a ladder. The child did not receive any medical treatment. As the disease slowly progressed, the child needed the help of others to walk, and he had a trendelenburg gait. He underwent spinal canal decompression and a nerve biopsy during his hospital stay. A diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was made based on electrophysiological findings and pathological examination results. Immunoglobulin or hormone therapy was recommended during hospitalization, but his mother refused. After discharge, the boy’s mother helped him carry out postoperative rehabilitation training at home. His lower-limb muscle strength gradually increased, and he could stand upright and take steps. Six mo after surgery, the child was readmitted and began immunoglobulin therapy. Long-term oral steroid treatment was initiated after discharge. The movement and sensation of the lower limbs were further improved, and the boy could walk normally 1 year after surgery. CONCLUSION: Spinal canal decompression can improve the clinical symptoms of HNCE caused by inflammation, even in children. When combined with specific etiological interventions, spinal cord decompression can lead to optimal outcomes.