Severe High Cervical Cord Compression Due to Large Bilateral Neurofibromas in a Patient With Neurofibromatosis Type 1: A Case Report and Review of Literature
By Admin | July 26, 2022
Spinal neurofibromas are rare benign lesions associated with neurofibromatosis Type 1. They can often cause compression on nerve roots and the spinal cord. In this rare case, there are bilateral large neurofibromas with severe cord compression presenting as progressive myelopathy. We illustrate the surgical management as well as post-operative care along with a detailed literature review of similar cases. To our knowledge, this is the first report, in English literature, of spinal neurofibroma with large size, bilateral high cervical cord compression.
Spinal neurofibromas are considered rare benign tumors of the spine. They comprise about 2-5% of all primary spinal neoplasms [1, 2, 3]. These lesions can occur sporadically or in conjunction with neurofibromatosis type 1 (NF1 or Von Recklinghausen’s disease). In patients with NF1 neurofibromas are not uncommon. These benign growths most often affect the spinal nerve roots and peripheral nerves; in fact, the involvement of the plexiform network (i.e the plexus of peripheral nerve and their sheath) is pathognomonic for NF1 [3, 4, 5, 6]. A small group of patients with NF1 develops the spinal neurofibromatosis subtype which results in scattered neurofibromas at almost all levels of the spine with various stages of growth. Nevertheless, symptomatic neurofibromas of the spine are rare. Patients usually present with radicular pain, numbness, and weakness which are usually localized to a specified nerve root as these lesions are commonly extradural and intra-foraminal [2, 7, 8, 9].
Neurofibromas can extend intramedullary (i.e invading the parenchyma of the spinal cord) [2,9] and pose a surgical challenge for resection. Due to their indolent course, patients often present with progressive and chronic changes in neurological status and other symptoms. Although rare, they can grow very large, causing cord compression and upper motor neuron signs [10, 11, 12].
In this report, we present a case of a patient with NF1 with multiple scattered spinal neurofibromas with spinal neurofibromas involving high cervical spinal cord bilaterally in the setting of myelopathy. As the reports of such presentation and extent of spinal neurofibromas remain sparse in the scientific literature, we aim to focus on the clinical and surgical aspects of the management of neurofibromas causing severe bilateral spinal cord compression.
A 51-year-old man with a past medical history of NF1 presented to our clinic with chief complaints of progressive worsening gait, difficulty in maintaining balance, weakness in both hands, and pain in the neck over the course of one year. His symptoms progressed to a point that he required a cane for ambulation. On his neurologic examination, he had intact function of all cranial nerves and normal bilateral visual fields. His strength exam was significant for intrinsic hand muscle, hip flexion, and knee flexion weakness. He had positive Hoffman’s on the left upper extremity, bilateral sustained clonus, and was...(More)
For more info please read, Severe High Cervical Cord Compression Due to Large Bilateral Neurofibromas in a Patient With Neurofibromatosis, by Cureus