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Lumbosacral Spinal Cord Compression

Management and Treatment

Created by:
BILGIS BILADI
1102013059

Advisory Board:
Dr. Donny. H. Hamid, SpS

Clinical Science of Neurology


General Hospital Pasar Rebo East Jakarta
19 MAY – 30 JUNE 2018
1. Definition
Conus medullaris and cauda equina syndromes are spinal cord injuriesthat involve
compression and injury to the lumbosacral segment of the spinal cord. 1

Conus medullaris syndrome is a constellation of signs and symptoms indicating


underlying injury to the lower end of the spinal cord at the level of first or second
lumbar vertebra in adults (conus medullaris/ conus terminalis). They are majorly due
to traumatic events and present with features of lower motor neuron and upper motor
neuron lesions involving the bladder, bowel, nearby skin, and muscles. 1

2. Anatomy

The spinal cord tapers and ends at the level between the first and second lumbar
vertebrae in an average adult. The most distal bulbous part of the spinal cord is called
the conus medullaris, and its tapering end continues as the filum terminale. Distal to
this end of the spinal cord is a collection of nerve roots, which are horsetail-like in
appearance and hence called the cauda equina (Latin for horse's tail).2

The conus medullaris (Fig. 1), also known as the medullary cone, is the distal end of
the spinal cord. Its location varies, and in adults it tapers at approximately the first or
second lumbar vertebra, ranging from T11 and L3. Derived from the neural tube, the
structure ascends in the vertebral canal because the growth rates of the spinal cord and
the vertebral column differ during development. Injury to the conus can cause
bladder, bowel, and sexual dysfunction as well as sensorimotor deficits. 3

Figure 1-2 Schematic drawing of the conus medullaris and distal nerve roots in relation to the sacrum.
Conus medullaris is consisting of sacral spinal cord segments. The upper border of the
conus medullaris is often not well defined. The fibrous extension of the cord, the
filum terminale, is a nonneural element that extends down to the coccyx.2

The blood supply to the conus medullaris includes the anterior spinal artery and the
right and left posterior spinal arteries, with smaller radicular contributions. The
anterior spinal artery traverses the anterior median sulcus of the spinal cord, while the
posterior spinal arteries descend posterior to the spinal cord and medial to the
posterior nerve roots. These anterior and posterior spinal arteries join at the lower
aspect of the conus to form a “conus basket” complex with extensions along the filum
terminale.1

The cauda equina (CE) is a bundle of intradural nerve roots at the end of the spinal
cord, in the subarachnoid space distal to the conus medullaris. Cauda is Latin for tail,
and equina is Latin for horse (ie, the "horse's tail"). The CE provides sensory
innervation to the saddle area, motor innervation to the sphincters, and
parasympathetic innervation to the bladder and lower bowel (ie, from the left splenic
flexure to the rectum).

The nerves in the CE region include lower lumbar and all of the sacral nerve roots.
The pelvic splanchnic nerves carry preganglionic parasympathetic fibers from S2-S4
to innervate the detrusor muscle of the urinary bladder. Conversely, somatic lower
motor neurons from S2-S4 innervate the voluntary muscles of the external anal
sphincter and the urethral sphincter via the inferior rectal and the perineal branches of
the pudendal nerve, respectively. Hence, the nerve roots in the CE region carry
sensations from the lower extremities, perineal dermatomes, and outgoing motor
fibers to the lower extremity myotomes.

The conus medullaris obtains its blood supply primarily from 3 spinal arterial vessels:
the anterior median longitudinal arterial trunk and 2 posterolateral trunks. Less
prominent sources of blood supply include radicular arterial branches from the aorta,
lateral sacral arteries, and the fifth lumbar, iliolumbar, and middle sacral arteries. The
la4tter contribute more to the vascular supply of the cauda equina, although not in a
segmental fashion, unlike the blood supply to the peripheral nerves.2,3

3. Epidemiology

Internationally about 10 to 80 people per million of the population will suffer from
spinal cord injuries. In the United States, more than 450,000 people live with spinal
cord injuries and about 120,000 new cases are reported every year. Most of the spinal
injury cases are secondary to traumatic events such as accidents and violence. Thus,
they are more common in younger men. Of all spinal cord injuries, 82% are men and
the average age of all patients is 47 years.
Spinal cord injuries involving thoracolumbar region make up 25 % of all spinal cord
injuries.1 Thus, conus medullaris and cauda equina syndrome are infrequent, it is a
diagnosis that must be considered in patients who complain of low back pain coupled
with neurologic complaints, especially urinary symptoms.2

4. Etiology

Spinal cord injuries occur in association with vertebral injuries but may also occur in
isolation and with no imaging changes especially in non-traumatic causes of spinal
cord injuries a situation known as spinal cord injury without radiographic
abnormality.1

The most common causes of cauda equina and conus medullaris syndromes are the
following:
• Lumbar stenosis (multilevel)
• Spinal trauma including fractures
• Herniated nucleus pulposus (cause of 2-6% of cases of cauda equina syndrome)
• Neoplasm, including metastases, astrocytoma, neurofibroma, and meningioma;
20% of all spinal tumors affect this area.
Tumors that compress the conus medullaris or impinge on the nerves at the
neuroforamina

• Spinal infection/abscess (eg, tuberculosis, herpes simplex virus, meningitis,


meningovascular syphilis, cytomegalovirus, schistosomiasis)
• Idiopathic (eg, spinal anesthesia ): these syndromes may occur as complications
of the procedure or of the anesthetic agent (eg, hyperbaric lidocaine, tetracaine)
• Iatrogenic injury to the nerves such as improperly placed screws or lamina
hooks

• Spina bifida and subsequent tethered cord syndrome


• Infections, inflammation, or hemorrhage that causes irritation to the bundles of
nerves which are not well myelinated
• Trauma to the spina causing fractures or subluxation birth defects such as AV
malformation

Other, rare causes include the following:


• Spinal hemorrhage, especially subdural and epidural hemorrhage causing
compression within the spinal canal
• Intravascular lymphomatosis
• Congenital anomalies of the spine/filum terminale, including tethered cord
syndrome
• Conus medullaris lipomas
• Multiple sclerosis
• Spinal arteriovenous malformations
• Late-stage ankylosing spondylitis
• Neurosarcoidosis
• A vascular lesion that compromises blood supply to the spinal cord to induce
ischemic injury
• Deep venous thrombosis of the spinal veins (propagated)
• Inferior vena cava thrombosis 1,2

5. Pathophysiology
The central event to the pathophysiology of the syndromes is the presence of a narrow
spinal canal that compresses the terminal part of the spinal cord or emerging spinal
nerves. The nervous structures are susceptible to injury i.e. the spinal nerves are
poorly myelinated compared to peripheral nerves. The narrowing of the canal may be
congenital or acquired such as in spondylosis and trauma to the lumbosacral region or
from space occupying lesions near the region.

The epidural space is made up of a structure such as fat and blood vessels that may
enlarge to cause compression of the nerves. Growths may also arise from the bony
vertebra as primary tumors or metastatic tumors that seed into the bones. Moreover,
the presence of inflammation, infection or any irritation is likely to trigger damage to
the nerves and induce a cauda equina syndrome.

The involved spinal nerves are responsible for Bladder and bowel function Sensory
innervation of the perineal area and extremities Motor function of the lower limbs

Thus, impingement on the spinal cord or spinal nerve roots leads to altered function in
the bladder, bowel or lower limbs and patients present with features of both upper and
lower motor neuron lesion due to injury to the spinal cord and spinal nerves
respectively.

6. Clinical Manifestation
Conus medullaris syndrome are a combination of LMN and upper motor neuron
(UMN) effects.2 this can be distinguished with the symptomps of Cauda Equina
Syndrome.1 but the two conditions require different treatment. Conus medullaris
typically produces sudden symptoms on both sides of the body, while cauda equina
syndrome usually develops over time, producing uneven symptoms concentrated on
one side of the body. the symptoms and signs of cauda equina syndrome tend to be
mostly lower motor neuron (LMN). 2,3

• Back pain that does not follow any dermatomal distribution and less severe
compared to the pain in cauda equina syndrome
• Absent ankle jerk reflex but present knee jerk reflex
• Lower limb weakness
• Loss of perineal sensations (Saddle anesthesia) involvement of the sacral
nerves S2-S4
• Absent bulbocavernosus reflex
• Poor rectal tone
• Sexual dysfunction
• Bladder and bowel incontinence

Cauda equina syndrome takes the form of a pure lower motor neuron lesion since the
spinal cord is intact and only spinal nerves are involved. It may vary in onset as:

• Acute onset: there is a rapid development of signs and symptoms that include
severe back pain and loss of bladder and bowel control.
• Gradual onset: symptoms may come and go and usually develops over months
or weeks. The loss of bladder and bowel function is usually intermittent.

The specific signs and symptoms include:

• Severe and sudden loss of sexual dysfunction


• Numbness, weakness, and pain in both legs due to the transaction of motor
fibers
• Altered sensation in both legs, buttocks, and thighs. This is due to injury of the
sacral nerves S2 -S5 (saddle anesthesia)
• Bladder and bowel retention due to loss of voluntary control and eventually
overflow incontinence
• Severe lower back pain that follows dermatomal distribution such as sciatica
• Reduced deep tendon reflexes.

Low back pain can be divided into local and radicular pain. Local pain is generally a
deep, aching pain resulting from soft-tissue and vertebral body irritation. Radicular
pain is generally a sharp, stabbing pain resulting from compression of the dorsal nerve
roots. Radicular pain projects in dermatomal distributions. Low back pain in cauda
equina syndrome may have some characteristic that suggests something different
from the far more common lumbar strain.

Severe pain is an early finding in 96% of patients with cauda equina syndrome
secondary to spinal neoplasm. Later findings include lower extremity weakness due to
involvement of the ventral roots. Patients generally develop hypotonia and
hyporeflexia. Sensory loss and sphincter dysfunction are also common.
Urinary manifestations of cauda equina syndrome include the following:
• Retention
• Difficulty initiating micturition
• Decreased urethral sensation
• Typically, urinary manifestations begin with urinary retention and are later
followed by an overflow urinary incontinence.
Bowel disturbances may include the following:
• Incontinence
• Constipation
• Loss of anal tone and sensation
7. Physical Examination

The anatomical proximity of the conus medullaris, the epiconus, and the cauda equina
can lead to 2 of these anatomical structures being involved via a single lesion,
resulting in an overlap of symptomatology.

Features Cauda Equina Syndrome Conus Medullaris

Vertebral level L2-sacrum L1-L2

Injury to the lumbosacral Injury of the sacral cord segment


Spinal level
nerve roots (conus and epiconus) and roots

Severity of
symptoms and Usually severe Usually not severe
signs

Symmetry of
symptoms and Usually asymmetric Usually symmetric
signs

Prominent, asymmetric, Usually bilateral and in the


Pain
and radicular perineal area

Weakness to flaccid Normal motor function to mild or


Motor
paralysis moderate weakness

Symmetric saddle distribution,


Saddle anesthesia, may be sensory loss of pin prick, and
Sensory
asymmetric temperature sensations (Tactile
sensation is spared.)

Areflexic lower Areflexic lower extremities


extremities; (If the epiconus is involved,
Reflexes bulbocavernosus reflex is patellar reflex may be absent,
absent in low CE (sacral) whereas bulbocavernosus reflex
lesions may be spared.)

Sphincter and Usually late and of lesser Early and severe bowel, bladder,
sexual function magnitude; and sexual dysfunction that
lower sacral roots results in a reflexic bowel and
involvement can cause bladder with impaired erection in
bladder, bowel, and sexual males
dysfunction

Multiple root level Mostly normal lower extremity


EMG involvement; sphincters with external anal sphincter
may also be involved involvement

May be favorable The outcome may be less


Outcome compared with conus favorable than in patients with
medullaris syndrome CES

• Cauda equina syndrome


In cauda equina syndrome, muscle strength in the lower extremities is diminished.
This may be specific to the involved nerve roots as listed below, with the lower
lumbar and sacral roots more affected, leading to diminished strength in the glutei
muscles, hamstring muscles (ie, semimembranosus, semitendinosus, biceps femoris),
and the gastrocnemius and soleus muscles.
Sensation is decreased to pinprick and light touch in a dermatomal pattern
corresponding to the affected nerve roots. This includes saddle anesthesia (sometimes
including the glans penis or clitoris) and decreased sensation in the lower extremities
in the distribution of lumbar and sacral nerves. Vibration sense may also be affected.
Sensation of the glans penis or clitoris should be examined.
Muscle stretch reflexes may be absent or diminished in the corresponding nerve roots.
Babinski reflex is diminished or absent.
Bulbocavernosus reflexes may be absent or diminished. This should always be tested.
Anal sphincter tone is patulous and should always be tested since it can define the
completeness of the injury (with bulbocavernosus reflex); it is also useful in
monitoring recovery from the injury.
Urinary incontinence could also occur secondary to loss of urinary sphincter tone; this
may also present initially as urinary retention secondary to a flaccid bladder.
Muscle tone in the lower extremities is decreased, which is consistent with an LMN
lesion.
• Conus medullaris syndrome
Patients may exhibit hypertonicity, especially if the lesion is isolated and primarily
UMN.Signs are almost identical to those of the cauda equina syndrome, except that in
conus medullaris syndrome signs are more likely to be bilateral; sacral segments
occasionally show preserved bulbocavernosus reflexes and normal or increased anal
sphincter tone; the muscle stretch reflex may be hyperreflexic, especially if the conus
medullaris syndrome (ie, UMN lesion) is isolated; Babinski reflex may affect the
extensors; and muscle tone might be increased (ie, spasticity).
Other signs include papilledema (rare, occurs in lower spinal cord tumors), cutaneous
abnormalities (eg, cutaneous angioma, pilonidal sinus that may be present in dermoid
or epidermoid tumors), distended bladder due to areflexia, and other spinal
abnormalities (noted on lower back examination) predisposing the patient to the
syndrome.
Muscle strength
Physical examination for cauda equina or conus medullaris syndromes would be
incomplete without tests for sensation of the saddle and perineal areas,
bulbocavernosus reflex, cremasteric reflex, and anal sphincter tone, findings for all of
which are likely to be abnormal.
Muscle strength of the following muscles should be tested to determine the level of
lesion:
• L2 - Hip flexors (iliopsoas)
• L3 - Knee extensors (quadriceps)
• L4 - Ankle dorsiflexors (tibialis anterior)
• L5 - Big toe extensors (extensor hallucis longus)
• S1 - Ankle plantar flexors (gastrocnemius/soleus)

ASIA impairment scale


In defining impairments associated with a spinal cord lesion, the American Spinal
Cord Injury Association (ASIA) impairment scale is used in determining the level and
extent of injury.
This scale should also be used in defining the extent of conus medullaris
syndrome/cauda equina syndrome. The scale is as follows:
• A - Complete; no sensory or motor function preserved in sacral segments S4-S5
• B - Incomplete; sensory, but not motor, function preserved below the neurologic
level and extends through sacral segments S4-S5
• C - Incomplete; motor function preserved below the neurologic level, and the
majority of key muscles below the neurologic level have a muscle grade less
than 3
• D - Incomplete; motor function preserved below the neurologic level, and the
majority of key muscles below the neurologic level have a muscle grade greater
than or equal to 3
• E - Normal; sensory and motor function normal
The injury should be described using this scale, for example, ASIA class A. Most
patients with cauda equina/conus medullaris syndrome are in ASIA class A or B
initially and gradually improve to class C, D, or E.2,3

8. Management Work Up

• CT scan of the spine

An excellent method of imaging the bone in the case of suspected tumors or in


traumatic causes. Delineates the structural pathology and its position. It is the
preferred imaging in older children and adults.

• MRI scan of the spine

MRI has superior soft tissue images that are desired in the assessment of the spinal
cord. Thus, it is best for analyzing congenital malformations, spondylosis,
subluxation, and other forms of spinal root compression.
MRI with gadolinium contrast of the lumbosacral area is the diagnostic test of choice
to define pathology in the areas of the conus medullaris and cauda equina (see the
images below). It provides a more complete radiographic assessment of the spine than
other tests; plain x-rays and CT scan may be normal. [85, 82] . Gadolinium contrast MRI
also may be able to rule out abdominal aneurysm, which could be the source of
emboli causing conus medullaris infarction. See the following images for
representative MRIs.

• Plain radiography

It is unlikely to be helpful in cauda equina syndrome but may be performed in cases


of traumatic injury or in search of destructive changes, disk-space narrowing, or
spondylolysis. For example, plain radiographs of the lumbosacral spine may depict
early changes in vertebral erosions secondary to tumors and spina bifida. Chest
radiography is indicated to rule out a pulmonary source of pathology that could affect
the lumbosacral spine (eg, malignant tumor, tuberculosis). Follow-up chest CT may
be required.

• Lumbar puncture and CSF analysis

They are analyzed when infection is suspected especially in the assessment of cauda
equina syndrome.

• Blood studies (CBC, UECs)

Additional workup for patients with infective causes suspected. CBC count, blood
glucose, electrolytes, blood urea nitrogen (BUN), and creatinine - As part of the
workup to rule out associated anemia, infection, and renal dysfunction, especially in
associated retroperitoneal mass, Erythrocyte sedimentation rate (ESR) – Elevation
may point to an inflammatory pathology, Syphilis serology to rule out
meningovascular syphilis

• Assessment of bladder function

Collection or identification of the significant amount of urine with little or no urge to


urinate indicates dysfunction.

• Needle electromyography (EMG)

It is an objective test to grade the denervation for more accurate classification of the
injury. To determine the type of bladder dysfunction present. 1,2,3

9. Differential Diagnosis
Table 2: Differential Diagnosis of Lumbosacral Spinal Cord Compression

Malignancies are some of the most common causes of cauda equina


syndrome and other forms of spinal cord injury. Identification of the
Metastatic
specific tumor is necessary for definitive management if possible.
malignancies
Tumors that metastasize to the spinal column include breast cancer,
lung cancer, renal cell carcinoma, prostate carcinoma.

Tethered cord The symptoms of pain worse with taking a bent position due to
syndrome stretching of the tethered cord.

Ankylosing A special form of arthritis that affects the spine but may also involve
spondylitis other joints of the body.

Presents with peripheral motor and sensory deficits that mimic a


Guillain-
spinal injury. However, there is no history of trauma preceding the
Barré-
symptoms and the disease progresses rapidly over hours compared
Syndrome
to spinal cord injury that rarely worsens after the presentation.

Spinal cord A cause of spinal cord injury that should be differentiated from other
infections. causes since the appropriate treatment is mainly medical.

Is defined as the complete loss of all neurologic functions, including


Spinal shock reflexes and rectal tone below a specific level that is associated with
autonomic dysfunction. Recovery in 24 hours

Refers to the hemodynamic triad of hypotension, bradycardia, and


peripheral vasodilation resulting from autonomic dysfunction and
Neurogenic
the interruption of sympathetic nervous system control in acute
shock
spinal cord injuries. It does not usually occur with spinal cord
injuries below the level of T6.

10. Treatment

Immediate Treatment

• Specific treatment is directed at the primary cause. The general treatment


goals are to minimize the extent of injury and to treat ensuing general
complications.
• Other medical treatment options are useful in certain patients, depending on
the underlying cause of the syndrome. Anti-inflammatory agents and steroids
can be effective in patients with inflammatory processes, including ankylosing
spondylitis.
• Patients with syndrome secondary to infectious causes should receive
appropriate antibiotic therapy. Patients with spinal neoplasms should be
evaluated for the suitability of chemotherapy and radiation therapy.
• Methylprednisolone should be administered. It treatment must be started
within 8 hours of injury. No evidence exists of any benefit if it is started more
than 8 hours after injury; on the contrary, late treatment may have detrimental
effects.
• Administration of ganglioside GM1 sodium salt beginning within 72 hours of
injury may be beneficial; the dose is 100 mg IV qd for 18-32 days.
• Tirilazad mesylate (a nonglucocorticoid 21-aminosteroid) has been proven to
be of benefit in animals and is currently under investigation. It inhibits lipid
peroxidation and hydrolysis in the same manner as glucocorticoids.
• Caution should be used in all forms of medical management for cauda equina
syndrome. Any patient with true cauda equina syndrome with symptoms of
saddle anesthesia and/or bilateral lower extremity weakness or loss of bowel
or bladder control should undergo no more than 24 hours of initial medical
management. If no relief of symptoms is achieved during this period,
immediate surgical decompression is necessary to minimize the chances of
permanent neurologic injury.2

Surgical decompression

In acute compression of the conus medullaris or cauda equina, surgical


decompression as soon as possible becomes mandatory. The goal is to relieve the
pressure on the nerves of the cauda equina by removing the compressing agent and
increasing the space in the spinal canal. Traditionally, cauda equina syndrome has
been considered a surgical emergency, with surgical decompression considered
necessary within 48 hours after the onset of symptoms, and preferably performed
within 6 h of injury.

For patients in whom a herniated disk is the cause of cauda equina syndrome, a
laminotomy or laminectomy to allow for decompression of the canal is recommended,
followed by gentle retraction and discectomy. In a more chronic presentation with less
severe symptoms, decompression could be performed when medically feasible and
should be delayed to optimize the patient's medical condition; with this precaution,
decompression is less likely to lead to irreversible neurological damage.

Surgical treatment may be necessary for decompression or tumor removal, especially


if the patient presents with acute onset of symptoms. Surgical treatment may include
laminectomy and instrumentation/fusion for stabilization or discectomy. Other
surgical care may entail wound care (eg, debridement, skin graft, and skin
flap/myocutaneous flap).2

A Discectomy is a type of surgery used to remove the part of the disc that is putting
pressure on the spinal cord or nerves. A Discectomy may be required if a herniated
disc is pressing directly on a nerve or the spinal cord and causing considerable pain.
Surgery may also be necessary to remove broken pieces of a disc or surrounding
bone. In select cases, a Discectomy can be performed arthroscopically. Arthroscopic
surgery uses small specialized tools and a small incision. It can be performed under
local anesthesia and has a shorter recovery time than traditional surgery. However, an
Open Discectomy is the type of surgery most frequently used for a lumbar herniated
disc. This is often done through a small incision and with a microscope and is called a
microdiscectony.

In some cases, the lamina on the vertebrae and ligaments need to be removed to allow
the surgeon to see and gain access to the disc. If all of the lamina is removed, the
procedure is called a Laminectomy. A Laminotomy involves removing only part of
the lamina.6

Figure 3-4. Lumbar Discestomy6

Many clinical and experimental reports have presented data on the functional outcome
based on the timing of surgical decompression. Several investigators have reported no
significant differences in the degree of functional recovery as a function of the timing
of surgical decompression. Even with these findings, however, most investigators
recommend surgical decompression as soon as possible after the onset of symptoms
to offer the greatest chance of complete neurologic recovery.
On discharge from the surgical ward, patients often are transferred to an acute
rehabilitation unit, from which they may be discharged, transferred to a subacute unit,
or transferred to long-term care, depending on the level of long-term disability.2

10. Complication

• Bladder and bowel dysfunction if the injury is not reversed and left to progress
• Back pain
• Pressure ulcers due to loss of sensation and immobilization impotence
• Gait changes and residual weakness.1,3

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