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Spontaneous CSF leak


Introduction

Spontaneous intracranial hypotension is often caused by structural defects in the dura within the spinal canal or along the nerve root sleeve. Although rare, cerebrospinal fluid (CSF) venous fistula has also been recognized recently as a cause of spontaneous intracranial hypotension. This condition has an estimated annual incidence of 5 per 100,000 persons, has a female predilection, and can occur in any region along the spine, but most often occurs in the thoracic levels. Symptoms classically include orthostatic headaches that improve with recumbency; however, the headache symptoms may be variable in location, onset, and quality. Cranial imaging typically demonstrates enhancement of the dura mater, empty sella, sagging of the cerebellar tonsils, and, in more severe cases, subdural hematomas. A computed tomography (CT) myelogram may be useful in pinpointing the exact location of a spontaneous leak. In many cases, there are multiple offending locations. CSF opening pressure is not a reliable diagnostic tool because most of the patients show pressures within the normal range. Autologous epidural blood patching may be employed as a first-line treatment and can be repeated if necessary. Persistent symptoms with identified leaks may require surgical intervention and direct closure. In this chapter, we present a case of a male patient presenting with the classic symptoms of spontaneous intracranial hypotension.

Example Case

  • Chief complaint: postural headaches

  • History of present illness: This is a 50-year-old male nonobese patient with a 3-month history of worsening postural headaches. He has not had any spinal procedures. Magnetic resonance image (MRI) of the thoracic spine showed epidural fluid collection consistent with spontaneous CSF leak ( Fig. 61.1 ).

    Fig. 61.1, Preoperative magnetic resonance imaging (MRI). (A) Sagittal T2 thoracic MRI demonstrating a dorsal cervicothoracic epidural collection of cerebrospinal fluid (CSF) intensity with flow voids (possibly from enlarged internal vertebral venous plexus of Batson) and ventral displacement of the spinal cord. (B) Sagittal T2 cervical MRI demonstrating absence of cerebellar tonsil ectopia (no Chiari malformation). There is no evidence of a nerve root cyst, pseudomeningocele, intervertebral disc herniation, or transdural osteophyte.

  • Medications: none

  • Allergies: no known drug allergies

  • Past medical and surgical history: none

  • Family history: noncontributory

  • Social history: lawyer, no smoking, occasional alcohol

  • 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: 2+ in bilateral biceps/triceps/brachioradialis with negative Hoffman; 2+ in bilateral patella/ankle; no clonus or Babinski; sensation intact to light touch

  • Ignacio Barrenechea, MD

  • Neurosurgery

  • Grupo Gamma

  • Rosario, Santa Fe, Argentina

  • Selby Chen, MD

  • Neurosurgery

  • Mayo Clinic

  • Jacksonville, Florida, United States

  • Fernando Hakim, MD

  • Hospital Universitario Fundacion Santafe de Bogota

  • Bogota, Colombia

  • Daniel C. Liu, MD, PhD

  • Neurosurgery

  • University of California at Los Angeles

  • Los Angeles, California, United States

Preoperative
Additional tests requested

  • MRI brain

  • CT myelogram

  • Anesthesia evaluation

  • Interventional radiology for blood patch

CT myelogram

  • MRI brain

  • CT myelogram

  • Interventional radiology for blood patch

  • MRI brain

  • CT myelogram

  • Interventional radiology for blood patch

Surgical approach selected If blood patch did not work, thoracic laminectomy and repair of CSF leak Thoracic laminectomy and repair of CSF leak If blood patch did not work, thoracic laminectomy and repair of CSF leak If blood patch did not work, thoracic laminectomy and repair of CSF leak
Goal of surgery Repair CSF leak Repair CSF leak, improve postural headaches Repair CSF leak Repair CSF leak
Perioperative
Positioning Prone on Wilson frame Prone Prone on gel rolls, no pins Prone on Jackson table, no pins
Surgical equipment

  • Fluoroscopy

  • Surgical microscope

  • Tubular retractor

  • Fluoroscopy

  • Surgical microscope

  • Castro-Viejo needle driver

  • Fluoroscopy

  • Piezoelectric drill

  • Ultrasound

  • Surgical microscope

  • Fluoroscopy

  • IOM (MEP/SSEP)

  • Surgical navigation

  • Lumbar drain

Medications None None Maintain MAP Fluorescein
Anatomical considerations Thoracic facet joint, ligamentum flavum, dura, spinal cord Thecal sac, spinal cord Nerve roots Spinal cord, facets
Complications feared with approach chosen Spinal cord injury, CSF leak, spinal instability Spinal cord injury Recurrence of CSF leak Spinal cord injury, recurrence of CSF leak
Intraoperative
Anesthesia General General General General
Exposure One level above and below area of CSF leak on myelogram One level above and below area of CSF leak on myelogram Hemilaminectomy at area of CSF leak on myelogram One level above and below area of CSF leak on myelogram
Levels decompressed Level of leak on myelogram Level of leak on myelogram Level of leak on myelogram Level of leak on myelogram
Levels fused None None None Only if needed
Surgical narrative Position prone, x-ray to plan incision with true PA levels, incision made between upper and lower pedicular line of target level, fascia incision and opened under skin edge with monopolar cautery, sequential dilation with tubular retractors, dock 25 mm tube and secure to table, monopolar cautery to remove remaining muscle and soft tissue attached to the bone inside the tube, bring surgical microscope in, hemilaminotomy with drill, keep ligamentum flavum until bone work is done to protect the dura, resect ligamentum with Kerrison rongeurs, dissect dura and locate site of CSF Position prone, midline incision over target level after confirmation with fluoroscopy, dissect down to level of spinous process, localize level with fluoroscopy, insert retractors, dissect soft tissue off of lamina, laminectomy with high-speed bur and rongeurs, yellow ligament resected with curette and Kerrison rongeurs, site of CSF leak identified, dural defect repaired under microscope with 6-0 Prolene, muscle pledget if defect large, Position prone, fluoroscopy to localize level, midline long incision where neuroradiologist identified leakage, subperiosteal dissection exposing posterior elements, hemilaminectomy at level selected, expose dura, Valsalva to confirm area of leakage, attempted primary repair of defect under microscopic visualization, also use muscle patch and fibrin sealant, Valsalva to confirm proper closure, layered closure, upright in bed for 24 hours Position prone, fluoroscopy to localize level based on rib counting in AP view, mark midline incision, posterior midline incision, dissect to posterior elements, x-ray to confirm level, laminectomy or laminectomies over relevant level, identify CSF leak and can inject intrathecal fluorescein with small-gauge needle if needed or intraoperative myelogram with x-ray or CT, primary closure of leak if possible with dural sealant, dural substitute and sandwich closure if cannot primary repair, can use muscle to help
leak, ligate if clear leak seen, Valsalva to identify leak if not clearly seen, if no leak seen then fat and fibrin glue, remove tube, layered closure Surgery on dural repair site, wound closed in anatomical layers with absorbable sutures closure, multilayer closure with subfascial drain to gravity, vancomycin powder epifascially, head of bed at 0 degrees for at least 24 hours, fusion if concern of instability
Complication avoidance Minimally invasive tubular retractor, keep ligamentum flavum until bone work is done to protect the dura, Valsalva to identify leak, pack with fat and fibrin glue if no leak seen Myelogram to confirm leak, muscle pledget if needed Myelogram to confirm leak, hemilaminectomy, confirm presence and repair with Valsalva maneuver Rib counting to determine level, intraoperative fluorescein or myelogram to identify CSF leak, attempt primary repair
Postoperative
Admission Floor Floor Floor Floor
Postoperative complications feared CSF leak, spinal instability CSF leak, neurological injury CSF leak CSF leak
Anticipated length of stay 2 days 3 days 2 days 2–5 days
Follow-up testing MRI brain and complete spine within 48 hours, 2 months, 6 months, 12 months after surgery None None None
Bracing None None None None
Follow-up visits 2 weeks, 6 weeks, 3 months, 6 months, 12 months after surgery 4–6 weeks after surgery 2 weeks after surgery 2 weeks, 6 weeks after surgery
AP , Anteroposterior; CSF , cerebrospinal fluid; CT , computed tomography; IOM , intraoperative monitoring; MAP , mean arterial pressure; MEP , motor evoked potential; MRI , magnetic resonance imaging; PA , posteroanterior; SSEP , somatosensory evoked potential.

Differential diagnosis

  • ▪

    Traumatic chronic subdural hematoma

  • ▪

    Aseptic meningitis

  • ▪

    Ventriculoperitoneal shunt (overshunting)

  • ▪

    Abnormalities of connective tissue

  • ▪

    Meningeal diverticula

  • ▪

    CSF leak

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