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Cavernous malformation
Introduction
Cavernous malformations (CMs) rarely occur in the spinal cord and account for less than 10% of all CMs. However, since the broad use of high-resolution magnetic scans, CMs of the spinal cord are found more often and currently represent 20% of all the intramedullary spinal tumors. They generally present in younger populations (second and third decade) and do not appear to have a sex predilection. CMs represent low flow vascular malformations but may have hemorrhagic events leading to neurological injury. The annual hemorrhage rate appears to be approximately 1% to 3% and is comparable to intracranial lesions, but this metric is difficult to assess and is reliant on the appearance of new or worsened patient symptoms. CMs may be managed with conservative observation or surgical intervention. The natural history of spinal cord CMs is not well defined, and the neurological outcomes after surgical intervention are not well understood. In this chapter, we present the case of a young female patient with a spinal CM with mild symptomatology and no evidence of spinal cord hemorrhage.
Example case
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Chief complaint: bilateral upper extremity paresthesias
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History of present illness: This is a 38-year-old female with a 4-week history of sudden onset numbness and tingling in her bilateral upper extremities. She describes an event that occurred 1 month prior in which she had sudden onset of weakness in her bilateral upper extremities that had improved, but since that time she has had persistent paresthesias. A magnetic resonance image was advocated as part of her evaluation for her current condition. The study demonstrated an intramedullary lesion at the level of C5 ( Fig. 65.1 ).
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Medications: oral contraceptives
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Allergies: no known drug allergies
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Past medical and surgical history: none
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Family history: none
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Social history: none
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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
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Reflexes: 3+ in bilateral biceps/triceps/brachioradialis with negative Hoffman; 3+ in bilateral patella/ankle; clonus in bilateral feet, positive Babinski; sensation decreased in both upper and lower extremities
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| Preoperative | ||||
| Additional tests requested | None |
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MRI C-spine with contrast C-spine flexion-extension x-rays |
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| Surgical approach selected | C4-5 laminectomy for resection of cavernous malformation | C4-5 laminoplasty for resection of cavernous malformation | C4-5 laminectomy for resection of cavernous malformation | C4-5 laminectomy for resection of cavernous malformation |
| Goal of surgery | Gross total resection | Gross total resection | Gross total resection, preservation of neurological function | Lesion removal with resolution of syrinx |
| Perioperative | ||||
| Positioning | Prone with Mayfield pins | Prone with Mayfield pins | Prone with Mayfield pins in Jackson table | Prone with Mayfield pins |
| Surgical equipment |
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| Medications | Steroids | Mannitol, maintain MAP | Steroids, maintain MAP >75 | Steroids, MAP >100 |
| Anatomical considerations | Spinal cord, dorsal midline | Spinal cord | Rexed lamina of spinal cord, midline dorsal raphe between dorsal columns | Spinal cord |
| Complications feared with approach chosen | Spinal cord injury | Spinal cord injury, spinal instability | Spinal cord injury | Spinal cord injury, CSF leak |
| Intraoperative | ||||
| Anesthesia | General | General | General | General |
| Exposure | C4-5 | C4-5 | C4-5 | C4-5 |
| Levels decompressed | C4-5 | C4-5 | C4-5 | C4-5 |
| Levels fused | None | None | None | None |
| Surgical narrative | Position prone, IOM, posterior midline incision, C4-5 laminectomy, dural opening and tack up, under microscope identify midline with anatomy, careful midline myelotomy with micro dissectors, resection of tumor, dural closure, layered closure | Position prone on transverse rolls, fluoroscopy to identify level, midline incision, subperiosteal dissection exposing posterior elements, laminoplasty at C4-5 using piezoelectric drill after x-ray confirmation, midline durotomy with tenting sutures, ultrasound to locate malformation and to guide midline myelotomy, midline myelotomy, identify malformation and gliotic plane, evacuate hematoma and resect malformation under microscopic visualization, gross total resection dependent on cleavage plane and SSEP/MEP change, watertight dural closure with fibrin sealant, laminoplasty with titanium plates, layered closure | Position prone on Mayfield with slight flexion, skin incision localized with fluoroscopy, dissection carried in the midline avascular plane, lamina exposed and correct level confirmed with fluoroscopy, standard laminectomy, microscope brought into the field, dura opened and tacked up, point of entry most superficial to the dorsal surface, midline myelotomy if not superficial, dorsal column separated until lesion encountered, capsule dissected from surrounding tissue, lesion removed in piecemeal, cavity inspected for complete removal, dura closed with fibrin glue, layered closure | Position prone, standard laminectomy minimizing removal of articular processes, open dura, dissect arachnoid, inspect spinal cord to see if there are any exophytic components, dissection from extramedullary toward intramedullary portion if there is an exophytic component, look for presence or absence of hemosiderin ring to guide entry, ultrasound to guide entry if no visual area to enter based on lesion or hemorrhage, perform midline myelotomy under IOM if no clear entry seen, careful dissection, spatula to remove lesion from white matter, watertight dural closure, layered closure |
| Complication avoidance | Identify the midline based on anatomy, enter into the cord through the dorsal midline | Laminoplasty, ultrasound to locate malformation and to guide midline myelotomy, resection guided by availability of plane and IOM changes | Point of entry most superficial to the dorsal surface, midline myelotomy if not superficial, lesion removed in piecemeal | Minimize removal of articular processes, attempt to enter spinal cord through exophytic component, ultrasound to guide entry if no visual area to enter |
| Postoperative | ||||
| Admission | ICU | ICU | ICU | ICU |
| Postoperative complications feared | Spinal cord injury | Spinal cord injury | Spinal cord injury, CSF leak, infection, cervical instability | Spinal cord injury |
| Anticipated length of stay | 4–7 days | 2 days | 2–3 days | 7 days |
| Follow-up testing | MRI C-spine 6 weeks after surgery | MRI C-spine within 48 hours of surgery |
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MRI C-spine 3 months after surgery |
| Bracing | None | None | None | None |
| Follow-up visits | 4 weeks, 3 months, 12 months after surgery | 2 weeks after surgery | 10–14 days, 6 weeks, 6 months after surgery | 2 weeks, 3 months after surgery |
CSF , Cerebrospinal fluid; ICU , intensive care unit; IOM , intraoperative monitoring; VHL , von-Hippel Lindau; MAP , mean arterial pressure; MEP , motor evoked potential; MRI , magnetic resonance imaging; SSEP , somatosensory evoked potential.
Differential diagnosis
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Cavernous malformation
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Spinal cord AVM
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Intramedullary spinal cord tumor
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Spinal cord infarction
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