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Cervico-medullary junction lesion


Introduction

Intramedullary spinal cord lesions represent a diagnostic challenge. Lesions occupying the cervico-medullary junction are rare entities, and the actual prevalence has not been reported. Patients who present with myelopathic symptoms and findings on magnetic resonance imaging (MRI) typically exhaust conservative measures before undergoing open biopsy for histological diagnosis and/or resection due to the associated neurological morbidity of the procedure. The location of the lesion with respect to the spinal cord cross section may assist in narrowing the differential diagnosis, as certain conditions preferentially affect certain areas of the spinal cord. In addition, lesions that affect multiple spinal cord segments in a rostral to caudal direction may also narrow the differential diagnosis with respect to possible causes. Open biopsy is reserved as a last diagnostic resort, and resection may be performed if intraoperative pathology demonstrates neoplastic tissue. The extent of resection is limited by the type of tumor, location, and results of intraoperative monitoring. In this chapter, we present a case of a young patient with a history of walk instability and lack of coordination in both upper extremities.

Example case

  • Chief complaint: neck pain and weakness

  • History of present illness: A 29-year-old female with a 2-month history of difficulty walking and coordination in her arms. She has been having progressive difficulty writing her name and holding items in her right hand. She has a history of recent travel to the Caribbean where she regularly performs work. She has not had any constitutional symptoms. She has had multiple MRIs, which have shown progression of an enhancing lesion at the cervico-medullary junction ( Figs. 54.1–54.2 ).

    Fig. 54.1, Preoperative magnetic resonance T1 with contrast images. (A) Sagittal view demonstrating a hyperintense rounded lesion at the cervicomedullary junction surrounded by a hypointense signal extending from the medulla to the C2-C3 level. The caliber of the cord appears increased at the level of the lesion. (B) Axial view demonstrating the lesion spans most of the cross-sectional area of the spine.

    Fig. 54.2, Preoperative magnetic resonance T2 images. (A) Sagittal view demonstrating abnormal enhancement extending from the upper medulla to approximately the C6 level. (B) Axial view demonstrating a diffuse high T2 signal spanning most of the cross-sectional area of the spine.

  • Medications: antidepressants

  • Allergies: no known drug allergies

  • Past medical and surgical history: depression, anxiety

  • Family history: noncontributory

  • Social history: none

  • Physical examination: awake, alert, and oriented to person, place, and time; cranial nerves II–XII intact; bilateral deltoids/triceps/biceps 5/5; interossei 5/5; iliopsoas/knee flexion/knee extension/dorsi, and plantar flexion 5/5

  • Reflexes: 3+ in bilateral biceps/triceps/brachioradialis with positive Hoffman; 3+ in bilateral patella/ankle; no clonus or Babinski; sensation intact to light touch

  • Maziyar A. Kalani, MD

  • Neurosurgery

  • Mayo Clinic

  • Phoenix, Arizona, United States

  • Jorge Navarro Bonnet, MD

  • Neurosurgery

  • Medica Sur, Mexico City, Mexico

  • Rodrigo Navarro-Ramirez, MD

  • Neurosurgery

  • McGill University

  • Montreal, Quebec, Canada

  • Jean-Paul Wolinsky, MD

  • Neurosurgery

  • Northwestern University

  • Chicago, Illinois, United States

Preoperative
Additional tests requested

  • MRI brain and complete spine

  • Ophthalmology evaluation

  • CT abdomen/pelvis

  • Genetic evaluation for VHL

  • NCS/EMG/SSEP

  • MRI complete spine

  • Antiaquaporin antibody test

  • Neurology evaluation

CT C-spine

  • Neurology evaluation, possible repeat CSF studies

  • Spinal angiogram

  • MRI STIR C-spine

  • PET

  • Follow-up MRI to assess growth

Surgical approach selected C1-2 laminectomy and resection of intramedullary spinal cord tumor C1 laminectomy and C2 laminoplasty and resection of intramedullary spinal cord tumor C1-2 laminectomy and resection of intramedullary spinal cord tumor Pending workup, C1-3 laminectomy vs. laminoplasty and resection of intramedullary spinal cord tumor
Goal of surgery Spinal cord decompression, diagnosis, tumor resection Spinal cord decompression, diagnosis, restoration of CSF flow Tumor resection Gross total resection, preservation of neurological function
Perioperative
Positioning Prone, in Mayfield pins Prone, in Mayfield pins Prone on Jackson table, in Mayfield pins Prone, in Mayfield pins
Surgical equipment

  • IOM (MEP/SSEP/spinal cord mapping)

  • Fluoroscopy

  • Ultrasound

  • IOM (lower cranial nerve monitoring)

  • Ultrasonic bone scalpel

  • Surgical microscope

  • Ultrasonic aspirator

  • IOM (MEP/SSEP)

  • Surgical microscope

  • Ultrasound

  • IOM (MEP/SSEP/D-wave)

  • Ultrasonic bone scalpel

  • Surgical microscope

  • Ultrasound

Medications Steroids, MAP >85 Maintain MAP MAP >90 Steroids, MAP >80
Anatomical considerations Vertebral arteries Posterior median sulcus, central canal Spinal cord Spinal cord, spinal cord vasculature
Complications feared with approach chosen Ondine’s curse, posterior column injury, pseudomeningocele, wound dehiscence Spinal cord injury, namely posterior columns Spinal cord injury Spinal cord injury, spinal cord stroke
Intraoperative
Anesthesia General General General General
Exposure Occiput-C3 C1-2 C1-2 C1-3
Levels decompressed C1-2 C1-2 C1-2 C1-3
Levels fused None None None None
Surgical narrative Position prone, incision planned based on palpation of first bifid process or preoperative x-ray, subperiosteal dissection from base of skull to C3, avoid dissecting more than 1 cm lateral to posterior tubercle of arch of C1 to avoid vertebral artery injury, piecemeal C1 laminectomy and en bloc C2 laminectomy for possible laminoplasty, intraoperative ultrasound to confirm exposure, dural opening, tack dura up with arachnoid, bipolar neuromonitoring probe to identify midline or by identifying confluence of veins, midline myelotomy, send small specimen to pathology to assess goals of surgery, remove lesion via circumferential dissection leaving draining vein for last if identified, dural closure with Gore-tex suture, Valsalva to assess for leakage, fibrin glue, potential laminoplasty of C2, layered closure with subfascial drain to half suction gravity, head at 30 degrees Position prone, IOM baseline, midline skin incision following avascular line to expose C1-2, remove C1 posterior arch, C2 laminotomy with bone scalpel, linear dura openings, open posterior median sulcus under microscopic visualization, send samples for histopathological diagnosis, continue lesion resection attempting maximal safe resection guided by IOM, careful hemostasis with mild compression, dural closure reinforced with dural substitute, C2 laminoplasty, closure in layers Position prone with slight flexion, midline incision from C1-2, subperiosteal dissection of muscles from C1 posterior arch and spinous process of C2, leave muscles attach to C3, subperiosteal dissection of C1 and C2 lamina with curette, C1-2 laminectomy with Kerrison rongeur preserving C1-2 and C2-3 joints, resect ligamentum flavum, ultrasound to confirm exposure and if laminectomy needed for more lateral exposure or if more rostral/caudal extension is needed, right paramedian dural opening and tacked up, myelotomy over most translucent area, sequential clockwise resection with limiting bipolar cautery under constant communication with IOM, watertight dural closure, layered closure with subfascial drain off suction, keep head elevated postop for 24 hours Preflip IOM, midline incision from C1-3, subperiosteal exposure of posterior arch of C1 and spinous processes and lamina of C2-C3, C1-3 laminectomies with ultrasonic bone scalpel, intraoperative ultrasound to confirm sufficient exposure at both rostral and caudal ends, placement of caudal epidural electrode under C4 to establish baseline D-wave monitoring capability, midline dural opening keeping arachnoid intact, tack dura up laterally, open and dissect arachnoid under microscope, open pia at tumor-spinal cord interface, identify arterial feeders and venous drainage, dissection of tumor capsule from spinal cord, deliver tumor, hemostasis with gentle irrigation, dural closure with fibrin glue, laminoplasty, layered closure with subfascial drain, skin closure with skin glue
Complication avoidance Avoid dissecting more than 1 cm lateral to posterior tubercle of arch of C1 to avoid vertebral artery injury, en bloc C2 laminectomy for laminoplasty, intraoperative ultrasound to confirm exposure, spinal cord mapping to identify midline, intraoperative pathology to determine goals of surgery Bone scalpel to perform laminotomy, identify posterior median sulcus, send frozen, resection guided by IOM, C2 laminoplasty Leave muscles attach to C3, preserve joints, ultrasound to confirm exposure, myelotomy over most translucent area, sequential clockwise resection with limiting bipolar cautery under constant communication with IOM Preflip IOM, ultrasound to assess exposure, D-wave assessments, attempt to keep arachnoid intact to minimize CSF loss and decrease epidural bleeding, attempt to work tumor capsule, laminoplasty
Postoperative
Admission ICU Intermediate care ICU ICU
Postoperative complications feared Ondine’s curse, posterior column injury, pseudomeningocele, wound dehiscence Posterior column injury, edema, expansion of syrinx, dysphagia, respiratory issues Weakness/paralysis, sensory deficit, spinal cord edema, CSF leak Weakness/paralysis, CSF leak, wound infection, cervical kyphosis
Anticipated length of stay 2–3 days 2 days 7–10 days 7 days
Follow-up testing MRI C-spine 3 months after surgery MRI within 48 hours of surgery MRI C-spine prior to discharge MRI C-spine 6 weeks after surgery (<48 hours if subtotal resection), 6 months, 12 months, 24 months, 36 months after surgery
Bracing None None None None
Follow-up visits 2 weeks, 6 weeks, 3 months after surgery 2 weeks after surgery 2 weeks, 1 month, 3 months after surgery 2 weeks, 6 weeks, 3 months, 6 months, 12 months, and annually after surgery
CSF , Cerebrospinal fluid; CT , computed tomography; EMG , electromyography; ICU , intensive care unit; IOM , intraoperative monitoring; MAP , mean arterial pressure; MEP , motor evoked potential; MRI , magnetic resonance imaging; NCS , nerve conduction study; PET , positron emission tomography; SSEP , somatosensory evoked potential; STIR , short T1 inversion recovery; VHL , von-Hippel Lindau.

Differential diagnosis

  • ▪

    Demyelinating disease

  • ▪

    Intrinsic glial spinal cord tumor

  • ▪

    Metastatic lesion

  • ▪

    Neurosarcoidosis

  • ▪

    Hemangioblastoma

  • ▪

    Lipoma

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