Epidural spinal cord compression in a patient with blue rubber bleb nevus syndrome.

ABSTRACT

Blue rubber bleb nevus syndrome (BRBNS) can present with vascular malformations throughout the body, especially in the gastrointestinal tract. Spinal cord compression from these lesions is rare, particularly in the pediatric population. The authors report a case of BRBNS involving an 18-year-old female patient who presented with back pain and an epidural thoracic mass with cord compression. She underwent an uncomplicated thoracic laminectomy and decompression, with removal of what appeared to be a venous malformation. Postoperatively her pain improved, and imaging revealed resolution of cord compression. Pathological analysis highlighted dilated venous channels with myxoid degeneration in the wall with clot, characteristic of BRBNS. The early age of presentation and location are unique based on the literature search of BRBNS. The present report highlights the multiplicity of venous malformations in BRBNS, and the management of this case.