The cauda equina
(CE) is a collection of intradural nerve roots, beginning at the end of the
spinal cord. "Cauda" is Latin for tail, and "equina" is Latin for horse, ie, the
"horse's tail".
In this article, the primary focus is cauda equina syndrome (CES). However,
as back pain is a major presentation of the syndrome, it is discussed briefly.
Back pain is among the most common of human conditions, approaching a 15-20%
incidence in the population. Most causes of both acute and chronic back pain are
benign. In a review of back pain cases in primary care practice, 4% were
considered to result from compression fracture, 3% from spondylolisthesis, 0.7%
from malignancy, 0.3% from ankylosing spondylitis (AS), and 0.1% from vertebral
osteomyelitis.
Three basic approaches to the diagnostic evaluation of back pain are used.
Most diagnostic decision making is based on the history and clinical
presentation. The first approach could be termed the red-flag approach, in which
certain historical and clinical clues are elicited to assess the probability of
serious disease. This method is very useful because it can help to detect
patients needing additional tests and can distinguish them from patients who may
benefit (or at least not be harmed by) a trial of conservative care. This
approach has been advocated for low back pain because it is efficient, and in
part, because it is cost-effective.
The second approach to diagnosis could be termed the pattern recognition
approach. This requires the clinician to match the patient's history and
findings with a clinical picture of the various diagnostic possibilities. Based
on the probabilities determined from such an analysis, additional testing may be
suggested to document the existence of the condition. It also may lead to a
trial of therapy for the specific disorder that, if successful, further
validates the diagnosis.
The third approach to diagnosis is an algorithmic approach. Algorithms have
proved very effective for making the differential diagnosis of many clinical
syndromes and have been advocated by several authors for the investigation of
low back pain. However, the utility of this approach may be less effective in
the evaluation of low back pain than in other conditions because it rarely leads
to a specific diagnosis. Algorithms may be helpful in determining the utility of
particular diagnostic tests, with a goal of ruling out the presence of more
serious causes of low back pain. In this regard, most of the algorithms that
have been put forward for the diagnosis of low back pain consist of a screen for
the presence of red flags, which are then used as decision points to determine
which additional testing might be indicated.
Potential red flags
History
- History of cancer
- History of a disorder with a predilection for infection or hemorrhage
- Older patient who has new onset of back pain
- History of metabolic bone disorder
- Unintended weight loss
Present complaint
- Precipitation by significant trauma
- Pain that is worse at night or not relieved by any position
- Bilateral radiculopathy
- Numbness or paresthesia in the perianal region
- Change in bladder or bowel function
- Writhing pain
- Unexplained, significant, lower limb weakness
- Progressive neurologic deficit
Physical examination and laboratory findings
- Pulsatile abdominal mass
- Fever
- Neurologic deficit not explained by monoradiculopathy
- Elevated sedimentation rate
Pattern of symptoms not compatible with benign mechanical disease
Lack of response to conservative measures
Red flags
Several symptoms and signs rarely encountered in benign forms of back pain
exist that are useful for stratifying the risk of more serious underlying
disease. These red flags can be divided into 3 basic types The first is based on history, the second on the characteristics of
the presenting complaint, and the third on physical examination findings and
simple laboratory investigations.
Several elements in the patient's medical history lead to some concern and,
therefore, may be thought of as red flags. Among these historical elements, a
history of cancer is the most important. Patients with cancer, even those
without a history of metastasis, who have a new onset of back pain require a
high index of suspicion for metastatic disease because neurologic symptoms can
develop quite rapidly. Also, treat patients who have a history of a disorder
that makes them prone to infection or hemorrhage or patients who have been using
immunosuppressive or anticoagulant medications with a high index of suspicion.
Similarly, new back pain in patients older than 50 years who lack a prior
history of similar back pain requires additional investigation because a higher
incidence of serious medical causes of back pain exists in older individuals
(eg, cancer, metabolic disorders of the spine) and most benign back pain
initially presents in younger patients.
The second type of red flag may be raised by the characteristics of the
present complaint. These characteristics include precipitation of the back pain
by significant trauma; pain that is worse at night or not relieved by a position
change; bilateral radiculopathy; numbness or paresthesias in the buttocks or the
perianal region; a change in bladder or bowel habits; writhing pain; and
significant lower limb weakness not explainable by pain or any progressive
neurologic deficit. Any of these elements should result in consideration of more
detailed imaging of the spine because of the increased likelihood of more
serious disorders.
Simple physical and laboratory studies that can be considered red flags
include a pulsatile abdominal mass or an enlarged shadow of the aorta on lumbar
radiograph. Focus neurologic examination on the lower limbs, and determine any
abnormality on motor or sensory examination. Static neurologic deficits
explainable by the involvement of a single lumbar nerve root cannot be
considered a red flag because this is a common presentation of benign disease
(disk herniation, lateral recess stenosis). However, treat patients who have
bilateral neurologic deficits, deficits involving multiple root levels, or
progressive neurologic deficits as if they had red flags.
Of all of the simple laboratory tests that may be of use in low back pain,
the erythrocyte sedimentation rate (ESR) is the most important. Not only is it
generally elevated in metastatic disease, but it also elevates in infectious
disorders of the spine (eg, osteomyelitis, discitis, epidural abscess). It may
be the only abnormal test in epidural abscess because this may present without
fever or an elevated white blood cell count. The ESR also may provide a clue as
to the inflammatory nature of the complaint in spondyloarthropathies. Back pain
with fever, as well as back pain with an elevated white blood cell count,
requires additional consideration.
The lack of a pattern of symptoms compatible with benign disease and the lack
of response to the usual measures of conservative care also may be considered
types of red flags. Reconsideration of the diagnosis is important in such cases
after a reasonable period of several weeks of conservative management.
Neoplastic disease
Neoplasms of the spine may be either primary or metastatic. Primary tumors
include neoplasms of the marrow, especially multiple myeloma, or tumors of the
bone or the cartilage of the spine. These neoplasms usually produce progressive
indolent back pain, which typically is unresponsive to rest and actually may be
most severe at night. Some of these neoplasms are benign, such as osteoid
osteoma (with pain that usually is remarkably responsive to aspirin); however,
most of these tumors can result in neurologic compromise by direct involvement
from the tumor mass or by compromising the structural integrity of the spine.
Multiple myeloma is the most common primary malignancy of the bone in adults.
It is very rare in people younger than 40 years, and back pain is the presenting
symptom in 35% of cases. Initially, this pain may be relieved by rest. Various
laboratory tests, including ESR, white blood cell, serum calcium, and uric acid
levels, frequently are elevated. Electrophoresis of serum and urine (for light
chains) is diagnostic for the condition by the demonstration of paraproteins
produced by myeloma cells.
Metastatic spinal disease is 25 times more common than primary tumors. In
part, this results from the large volume of blood that courses slowly through
bidirectional venous channels (Batson plexus) in the epidural space, which
communicates with blood spaces in the vertebral marrow. Spinal metastasis is
found in as many as 70% of patients who have primary tumors on autopsy. The most
common tumors to metastasize to the spine include tumors of the breast, lung,
prostate, kidney, lymphoma, melanoma, and GI tract. Back pain is the presenting
symptom in 90% of patients who have spinal metastasis. Frequently, early
metastasis is not visualized on plain radiographs, though bone scans are
positive in 85% of patients who have spinal metastasis. MRI may identify spinal
metastasis in patients who have normal radiographs and bone scans.
In a survey of 1975 patients presenting for the evaluation of low back pain,
Deyo and Diehl found that an age greater than or equal to 50 years, a previous
history of cancer, duration of pain greater than one month, failure to improve
with conservative therapy, an elevated ESR, and anemia were significantly
associated with the presence of neoplastic disease. The pain produced by
neoplasm often is worst while the patient is at rest, and it may even awaken
patients from a sound sleep. Both of these symptoms are atypical for benign
disease. The back pain usually is focal and worsens with percussion.
Assume new back pain is neoplastic in any patient who has a history of cancer
or unexplained weight loss, and there should be a low threshold for radiographic
studies in such patients. Plain radiographs may be reassuring; however, if
neurologic compromise occurs, if the patient with cancer is not responding to
conservative care, or if a compelling story for benign mechanical disease does
not exist, perform and MRI or CT scan. The best predictors of the spinal level
of the abnormality are location of the focal pain, location of percussive
tenderness, or location of origin of any nerve roots or lower motor neurons that
may be clinically affected.
Problem: The CE is formed by nerve roots caudal to the level
of spinal cord termination. CES has been defined as low back pain; unilateral
or, usually, bilateral sciatica; saddle sensory disturbances; bladder and bowel
dysfunction; and variable lower extremity motor and sensory loss.
Lesions involving the termination of the spinal cord (conus medullaris) and
the CE are not discussed here. Please refer to Spinal Cord
Injuries.
Frequency: In the United States, CES is uncommon, both
atraumatically and traumatically, accounting for approximately 1-3% of patients
who undergo spinal surgery. No racial or sexual predilection exists for CES.
Traumatic CES is not age specific. Atraumatic CES occurs primarily in adults.
Etiology:
- Trauma
- Lumbar disk disease
- Abscess
- Spinal anesthesia
- Tumor, metastatic, or CNS elements
- Late-stage Atherosclerosis
- Idiopathic
Pathophysiology: CES may result from any lesion that
compresses the nerve roots of the CE. These nerve roots are particularly
susceptible to injury because they have a poorly developed epineurium. When they
are well developed, as in peripheral nerves, they protect against compressive
and tensile stresses. The microvascular systems of nerve roots have a region of
relative hypovascularity in their proximal third. Increased vascular
permeability and subsequent diffusion from the surrounding cerebrospinal fluid
(CSF) supplement the nutritional supply. This property of increased permeability
may be related to the nerve root tendency toward edema formation, which may
result in the edema compounding initial and, occasionally, seemingly slight
injury.
Clinical: Radicular pain is a frequent presentation of the
cauda equina syndrome, usually in association with radicular sensory loss
(saddle anesthesia), asymmetrical paraplegia with loss of the tendon reflexes,
muscle atrophy, and bladder dysfunction. The principal causes of this syndrome
are tumors, lumbar spinal stenosis, ruptured lumbar disk, arachnoiditis, and
spinal fracture.
When the conus medullaris is impaired, radicular pain is less prominent. The
principal and early disturbances are urinary retention and constipation. In
addition, there may be loss of pinprick sensation in the perianal region,
impotence, and sometimes muscle weakness of the lower limb.
Table 1. Cauda Equina versus Conus Medullaris Syndrome
|
Cauda Equina |
Conus Medullaris Syndrome |
Pain |
Severe radicular pain |
Back pain, less severe radicular pain |
Hypesthesia |
Radicular sensory loss (saddle hypesthesia) |
Usually restricted to perianal region |
Motor deficits |
Asymmetrical areflexic paraplegia |
Distal paresis of lower limbs |
Sphincters |
Urinary retention |
Urinary retention plus atonic anal Sphincter |
Sexual dysfunction |
Sometimes impotence |
Impotence frequent |
History
- Low back pain, usually in the form of common symptoms of bilateral
sciatica
- Acute or chronic radiating pain
- Unilateral or bilateral lower extremity motor and/or sensory abnormality
- Bowel and/or bladder dysfunction
- Usually with associated saddle (perineal) anesthesia
- The examiner can inquire if toilet paper feels different when
wiping.
Physical
- Pain often is localized to the low back; local tenderness on palpation or
percussion may be present.
- Reflex abnormalities may be present; they typically include loss or
diminution of reflexes. Hyperactive reflexes may signal spinal cord
involvement and exclude the diagnosis of CES.
- Pain in the legs (or radiating to the legs) is characteristic of CES.
- A sensory abnormality may be present in the perineal area or lower
extremities.
- Muscle weakness may be present in muscles supplied by affected roots.
Muscle wasting may occur if CES is chronic.
- Poor anal sphincter tone is characteristic of the syndrome.
- Babinski sign or other signs of upper motor neuron involvement suggest a
diagnosis other than CES, possibly spinal cord compression.
- Anesthetic areas may show skin breakdown.
- Laxity of anal sphincter tone, on very rare occasions, may lead to rectal
prolapse.
- Back point tenderness is remarkable in patients with large disk
herniations.
INDICATIONS
Lumbar laminectomy
(for removal of the cause of nerve root compression) is indicated for patients
with a history, physical examination, and diagnostic imaging (magnetic resonance
[MR] lumbar spine stenosis) consistent with a spinal pathology explaining the
presentation with CE.
RELEVANT ANATOMY AND CONTRAINDICATIONS
Relevant
Anatomy: The spinal cord is the downward continuation of the medulla,
starting at the foramen magnum. It descends to about the level of the second
lumbar vertebra, tapering to a structure called the conus medullaris. A thin
threadlike continuation of the conus, the filum terminale, extends as far as the
coccyx and is composed of nonnervous tissue.
The cord serves as a conduit for the ascending and descending fiber tracts
that connect the peripheral and spinal nerves with the brain. The cord projects
31 pairs of spinal nerves on either side (8 cervical, 12 thoracic, 5 lumbar, 5
sacral, 1 coccygeal) that are connected to the peripheral nerves. A cross
section of the spinal cord demonstrates a butterfly-shaped gray matter in the
middle surrounded by white matter. As in the cerebrum, the gray matter is
composed of cell bodies. The white matter consists of various ascending and
descending tracts of myelinated axon fibers with specific functions.
Contraindications: An uncertain diagnosis or an unclear
clinical presentation is a definite contraindication to the surgery. The same
contraindications as for any other surgery (eg, fever) also apply.
Observation for a variable period of time may be a better treatment option
for patients with high surgical risk and/or mild neurologic dysfunction.
Patients in whom biopsy reveals malignant neoplasm may be best treated with
radiotherapy since they are expected to have a rapid downhill course.
WORKUP
Lab Studies:
- ESR: Of all the laboratory tests, a high ESR should raise the suspicion of
cancer or infection.
- Postvoiding residual (PVR): Catheterization for residual urine volume may
reveal urinary retention, suggesting a neurogenic bladder. More urine than 100
cc should raise suspicion of CES.
Imaging Studies:
- Plain radiographs: These are unlikely to be helpful; however, obtain
radiographs to search for destructive changes, disk-space narrowing, or
spondylolysis.
- Lumbar myelogram: This is no longer often performed since the MRI has
greater advantages. Obstruction of the flow of the dye over the area of
compression confirms the location of pathology.
- CT scan with or without contrast: This is better and easier to obtain than
the lumbar myelogram but is still not superior to MRI in terms of findings and
quality. It is the study of choice in patients with contraindications to MRI
(eg, presence of body metal).
- MRI: Because case reports suggest a superiority of MRI over CT scan, MRI
is the criterion standard for the initial evaluation of patients with CES. MRI
also is superb in showing the plexus and the nerve foramina. Early
consultation with appropriate subspecialty is encouraged to guide imaging
studies.
TREATMENT
Medical
therapy: No proven medical treatment exists. Generally, therapy is
directed at the underlying cause of CES. Some might suggest methylprednisolone
in a regimen similar to that administered for traumatic spinal cord injury or
another regimen of steroids for the acute syndrome.
For penetrating trauma, steroids have not shown significant benefit. Surgery
is controversial. The timing of decompression is controversial, with immediate,
early, and late surgical decompression showing varying results.
Consultations
Early neurosurgical, neurological, or orthopedic consultations are
recommended, depending on the suspected etiology of CES.
Medication
Steroids may be recommended in acute or traumatic CES. Early consultation
regarding the use of steroids and any specific regimen is encouraged. Steroids
have anti-inflammatory properties and cause profound and varied metabolic
effects. In addition, these agents modify the body's immune response to diverse
stimuli. They may decrease edema around nerve root segments.
A regimen of methylprednisolone (Solu-Medrol, Depo-Medrol) may be used. One
possible regimen may be the dose employed in traumatic spinal cord injuries, but
no studies exist to support this over any other regimen.
Surgical therapy:
Surgical objectives
The lumbar laminectomy is one of the simplest neurosurgical operations
performed with the patient under general anesthesia. Another simple
neurosurgical procedure, insertion of a ventriculoperitoneal shunt, is like
lumbar laminectomy in that it is fraught with a number of untoward outcomes and
complications disproportionate to the ease with which it is performed. The
objective of lumbar laminectomy is to open up the spaces that nerve roots travel
through on their way from inside to outside the vertebral canal. The site along
the course of the nerve root most liable to compression is where it passes
through the opening from the canal to the surrounding paraspinous back muscles;
this opening is known as the neural foramen.
Lesion considerations
Degenerative osteoarthritis causes foraminal narrowing by increasing the
amount and extent of inflamed arthritic bone at and immediately adjacent to the
facet joint, the site of movement in the spine. Also, as a consequence of
movement, friction and irritation generate inflammation, which ultimately leads
to the changes in joints and bones called arthritis. Relieving pressure on
nerves at and near the neural foramen requires removal of some of the material
pathologically narrowing the neural foramen. To reach the neural foramen and
remove some of the pathological bony material, the surgeon must remove the bones
and ligaments of the posterior vertebral canal, laminectomy. Once the lumbar
dura has been exposed, the surgeon needs to inspect the spaces on the sides
(lateral) of the inside the vertebral canal, ie, the so-called recesses, along
which the nerve root travels just prior to entry into the neural foramen on its
way out of the canal.
To evaluate for compression in the lateral recesses and on through the neural
foramen, the surgeon needs to tactilely and visually inspect these structures.
The thecal sac must be pulled (retracted) medially to create a space between it
and the inner wall of the vertebral canal to allow the surgeon to inspect and
work. A variety of probes are used to feel for spurs and other excrescences of
bone that can be removed using rongeurs and curettes designed to address the
anatomic particularities of surgical access and manipulation in the posterior
lumbar vertebral canal.
With curettes and rongeurs, excess bone, inflamed joint material, and
hypertrophied ligamentum flavum are removed. (Hypertrophy [abnormal thickening]
of the ligamentum flavum is a frequent accompaniment to lumbar stenosis.)
Laminectomy facetectomy
Once, by feeling with probes, the surgeon is convinced that the space of the
lateral recesses and neural foramina is sufficiently open to allow for
unrestricted passage of the exiting nerve root, the surgeon allows the dura to
return to its position up against the wall (except for an intervening layer of
epidural fat and the epidural venous plexus) of the vertebral canal.
Hemostasis usually is simple as long as muscles have not been divided during
the laminectomy. Closure is the same as for laminectomy. For lumbar stenosis, a
frequent occurrence during lumbar laminectomy is violation of the dura mater (a
tear) associated with escape of CSF from the subarachnoid space into the
epidural space; occasionally, this occurs in such volume and escapes rapidly
enough that it begins to fill the laminectomy wound.
A persistent CSF leak can become a significant complication of lumbar
laminectomy associated with formation of pseudomeningoceles and, possibly, even
wound dehiscence. The yellow ligament in elderly patients with lumbar stenosis,
facet hypertrophy, lateral recess stenosis, and neural foraminal stenosis, also
is frequently hypertrophic and closely adherent (stuck) to the underlying dura.
To avoid tearing the dura, the surgeon must carefully develop a plane between
the dura and the yellow ligament (ligamentum flavum). This is usually the most
difficult and time-consuming part of a lumbar laminectomy for decompression of
degenerative arthritic lumbar stenosis (vertebral canal narrowing due to lumbar
spondylitic [degenerative arthritic] disease).
Surgical considerations include the following:
- Exposure: Laminectomy is used to gain exposure to the posterior
lumbosacral dura, the facet joints, and the nerve roots.
- Approach
- Hazards: Structures (dura and the blood vessels) exist along the course of
the approach that might be injured during exposure or decompressive lumbar
laminectomy.
- Instruments
- Anesthesia: General anesthesia usually is used for lumbar laminectomies
because the extent of soft-tissue disruption causes patients who are awake or
only sedated intolerable pain.
- Monitoring: Monitoring is not usually performed during decompressive
lumbar laminectomy.
- Position: The patient is positioned prone during surgery. (A small number
of neurosurgeons perform lumbar laminectomy with the patient positioned
laterally.)
- Preparation and drape: Sterile drapes are placed running parallel to the
midline, with 2 drapes oriented transversely and resulting in a rectangular
sterile field.
- Problems: Epidural bleeding is less during lumbar laminectomy than during
microdiscectomy.
- Closure: Closure of a lumbar laminectomy is the same as for any
laminectomy.
- Duration: A lumbar laminectomy takes approximately one and one half hours.
- Induction - 10 minutes
- Positioning - 10 minutes
- Preparation - 5 minutes
- Exposure - 15 minutes
- Target - 30 minutes
- Closure - 15 minutes
- Extubation - 10 minutes
Preoperative details: Stabilize acute life-threatening
conditions. Immobilize the spine if the injury is traumatic.
Postoperative details: After a decompressive lumbar
laminectomy, the patient usually is taken to the recovery room. Immediately
postoperatively and preoperatively, patients are checked to make sure they are
moving and can feel their legs. The back also may be inspected to make sure no
seepage of blood occurs through the dressings. Once recovered, the patient
usually is taken to a regular ward bed.
In the recovery phase after decompressive lumbar laminectomy, rehabilitative
physical therapy or rehabilitation is not indicated in patients without
preoperative neurologic deficit.
Follow-up care: After discharge following a lumbar
laminectomy, the patient should be seen in the outpatient (ambulatory) clinic in
7-10 days. Perform a follow-up MRI.
Admit patients to appropriate service (usually neurology, neurosurgery, or
orthopedic surgery) with frequent neurologic checks. Ideally, the admitting
physician or service should examine the patient at the time of admission.
Patients in whom acute CES is being considered should not be managed or
investigated on an outpatient basis without evaluation by a consultant and/or
appropriate imaging specialist.
Consider patients with CES for transfer if appropriate subspecialty care is
not available.
COMPLICATIONS
If therapy is
delayed, potential problems include residual weakness, incontinence, impotence,
and sensory abnormalities. However, these problems can persist even with prompt
decompression.
Risks and complications of lumbar laminectomy include dural tear
(occasionally with pseudomeningocele), "failed back syndrome" (scar, residual
stenosis), and instability.
OUTCOME AND PROGNOSIS
CES is
not fatal. Morbidity from CES is variable, depending on the etiology of the
syndrome.
The prognosis for CES improves if a definitive cause is identified and
appropriate management occurs early in the course. Surgical decompression may be
performed emergently, or, in some cases, delayed, depending on the etiology.
The prognosis following decompressive lumbar laminectomy is related to the
length of time that the patient was symptomatic or with deficit.
FUTURE AND CONTROVERSIES
Reoperation may
be necessary for recurrence of stenosis or to extend the levels of decompression
after a patient fails to improve after an initial limited procedure. Reoperation
is usually more difficult than the first procedure because of scar tissue.