Cauda equina syndrome (CES) is a condition that occurs when the bundle of
nerves
A nerve is an enclosed, cable-like bundle of nerve fibers (called axons) in the peripheral nervous system.
A nerve transmits electrical impulses. It is the basic unit of the peripheral nervous system. A nerve provides a common pathway for the e ...
below the end of the
spinal cord
The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...
known as the
cauda equina
The cauda equina () is a bundle of spinal nerves and spinal nerve rootlets, consisting of the second through fifth lumbar nerve pairs, the first through fifth sacral nerve pairs, and the coccygeal nerve, all of which arise from the lumbar enlarg ...
is damaged.
Signs and symptoms include
low back pain
Low back pain (LBP) or lumbago is a common disorder involving the muscles, nerves, and bones of the back, in between the lower edge of the ribs and the lower fold of the buttocks. Pain can vary from a dull constant ache to a sudden sharp feel ...
,
pain that radiates down the leg, numbness around the anus, and loss of bowel or bladder control.
[ Onset may be rapid or gradual.][
The cause is usually a ]disc herniation
Spinal disc herniation is an injury to the cushioning and connective tissue between vertebrae, usually caused by excessive strain or trauma to the spine. It may result in back pain, pain or sensation in different parts of the body, and physical ...
in the lower region of the back.[ Other causes include ]spinal stenosis
Spinal stenosis is an abnormal narrowing of the spinal canal or neural foramen that results in pressure on the spinal cord or nerve roots. Symptoms may include pain, numbness, or weakness in the arms or legs. Symptoms are typically gradual i ...
, cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
, trauma
Trauma most often refers to:
* Major trauma, in physical medicine, severe physical injury caused by an external source
* Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event
*Traumatic i ...
, epidural abscess, and epidural hematoma
Epidural hematoma is when bleeding occurs between the tough outer membrane covering the brain (dura mater) and the skull. Often there is loss of consciousness following a head injury, a brief regaining of consciousness, and then loss of consciou ...
.[ The diagnosis is suspected based on symptoms and confirmed by medical imaging such as ]MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
or CT scan.
CES is generally treated surgically via laminectomy
A laminectomy is a surgical procedure that removes a portion of a vertebra called the lamina, which is the roof of the spinal canal. It is a major spine operation with residual scar tissue and may result in postlaminectomy syndrome. Depending ...
.[ Sudden onset is regarded as a ]medical emergency
A medical emergency is an acute injury or illness that poses an immediate risk to a person's life or long-term health, sometimes referred to as a situation risking "life or limb". These emergencies may require assistance from another, qualified ...
requiring prompt surgical decompression In medicine, decompression refers to the removal or repositioning of any structure compressing any other structure.
Common examples include decompressive craniectomy (removal of part of the skull to relieve pressure on the brain), and spinal decomp ...
, with delay causing permanent loss of function. Permanent bladder problems, sexual dysfunction
Sexual dysfunction is difficulty experienced by an individual or partners during any stage of normal sexual activity, including physical pleasure, desire, preference, arousal, or orgasm. The World Health Organization defines sexual dysfunction a ...
or numbness may occur despite surgery.[ A poor outcome occurs in about 20% of people despite treatment.][ About 1 in 70,000 people is affected every year.] It was first described in 1934.
Signs and symptoms
Signs and symptoms of cauda equina syndrome include:
* Severe back pain
* Saddle anesthesia
Saddle anesthesia is a loss of sensation (anesthesia) restricted to the area of the buttocks, perineum and inner surfaces of the thighs.
Asymmetric saddle anesthesia is frequently associated with the spine-related injury cauda equina syndrome. It ...
(see diagram), i.e., anesthesia or paraesthesia
Paresthesia is an abnormal sensation of the skin (tingling, pricking, chilling, burning, numbness) with no apparent physical cause. Paresthesia may be transient or chronic, and may have any of dozens of possible underlying causes. Paresthesias ar ...
involving S3 to S5 dermatome Dermatome may refer to:
* Dermatome (anatomy), an area of skin that is supplied by a single pair of dorsal roots
* Dermatome (embryology), the portion of the embryonic paraxial mesoderm, the somite, which gives rise to dermis
* Dermatome (instrument ...
s, including the perineum
The perineum in humans is the space between the anus and scrotum in the male, or between the anus and the vulva in the female. The perineum is the region of the body between the pubic symphysis (pubic arch) and the coccyx (tail bone), includi ...
, external genitalia
A sex organ (or reproductive organ) is any part of an animal or plant that is involved in sexual reproduction. The reproductive organs together constitute the reproductive system. In animals, the testis in the male, and the ovary in the female, a ...
and anus; or more descriptively, numbness or "pins-and-needles" sensations of the groin and inner thighs which would contact a saddle when riding a horse.
* Bladder and bowel dysfunction, caused by decreased tone of the urinary and anal sphincters. Detrusor weaknesses causing urinary
The urinary system, also known as the urinary tract or renal system, consists of the kidneys, ureters, bladder, and the urethra. The purpose of the urinary system is to eliminate waste from the body, regulate blood volume and blood pressure, co ...
retention and post-void residual incontinence
Incontinence or Incontinent may refer to:
*Fecal incontinence, the involuntary excretion of bowel contents
*Urinary incontinence, the involuntary excretion of urine
* Lack of moderation or self-control, especially related to sexual desire - see I ...
as assessed by bladder scanning the patient after the patient has urinated
Urination, also known as micturition, is the release of urine from the urinary bladder through the urethra to the outside of the body. It is the urinary system's form of excretion. It is also known medically as micturition, voiding, uresis, ...
.
* Sciatica
Sciatica is pain going down the leg from the lower back. This pain may go down the back, outside, or front of the leg. Onset is often sudden following activities like heavy lifting, though gradual onset may also occur. The pain is often described ...
-type pain on one side or both sides, although pain may be wholly absent
* Weakness of the muscles of the lower legs (often paraplegia)
* Achilles (ankle) reflex absent on both sides.
* Sexual dysfunction
Sexual dysfunction is difficulty experienced by an individual or partners during any stage of normal sexual activity, including physical pleasure, desire, preference, arousal, or orgasm. The World Health Organization defines sexual dysfunction a ...
* Absent anal reflex and bulbocavernosus reflex The bulbocavernosus reflex (BCR), bulbospongiosus reflex (BSR) or "Osinski reflex" is a polysynaptic reflex that is useful in testing for spinal shock and gaining information about the state of spinal cord injuries (SCI). ''Bulbocavernosus'' is an ...
* Gait disturbance
Gait deviations are nominally referred to as any variation of standard human gait, typically manifesting as a coping mechanism in response to an anatomical impairment. Lower-limb amputees are unable to maintain the characteristic walking pattern ...
Severe back pain, saddle anesthesia
Saddle anesthesia is a loss of sensation (anesthesia) restricted to the area of the buttocks, perineum and inner surfaces of the thighs.
Asymmetric saddle anesthesia is frequently associated with the spine-related injury cauda equina syndrome. It ...
, urinary
The urinary system, also known as the urinary tract or renal system, consists of the kidneys, ureters, bladder, and the urethra. The purpose of the urinary system is to eliminate waste from the body, regulate blood volume and blood pressure, co ...
or fecal incontinence
Fecal incontinence (FI), or in some forms encopresis, is a lack of control over defecation, leading to involuntary loss of bowel contents, both liquid stool elements and mucus, or solid feces. When this loss includes flatus (gas), it is referre ...
and sexual dysfunction
Sexual dysfunction is difficulty experienced by an individual or partners during any stage of normal sexual activity, including physical pleasure, desire, preference, arousal, or orgasm. The World Health Organization defines sexual dysfunction a ...
are considered "red flags", i.e. features which require urgent investigation.
Causes
After the conus medullaris
''Conus'' is a genus of predatory sea snails, or cone snails, marine gastropod mollusks in the family Conidae.Bouchet, P.; Gofas, S. (2015). Conus Linnaeus, 1758. In: MolluscaBase (2015). Accessed through: World Register of Marine Species ...
(near lumbar vertebral levels 1 (L1) and 2 (L2), occasionally lower), the spinal canal contains a bundle of nerve fibers (the cauda equina
The cauda equina () is a bundle of spinal nerves and spinal nerve rootlets, consisting of the second through fifth lumbar nerve pairs, the first through fifth sacral nerve pairs, and the coccygeal nerve, all of which arise from the lumbar enlarg ...
or "horse-tail") that branches off the lower end of the spinal cord and contains the nerve roots from L1–L5 and S1–S5. The nerve roots from L4–S4 join in the sacral plexus which affects the sciatic nerve, which travels caudally (toward the feet). Compression, trauma or other damage to this region of the spinal canal can result in cauda equina syndrome.
The symptoms may also appear as a temporary side-effect of a sacral extra-dural injection.
Trauma
Direct trauma can also cause cauda equina syndrome. Most common causes include as a complication of lumbar punctures, burst fractures resulting in posterior migration of fragments of the vertebral body, severe disc herniations, spinal anaesthesia involving trauma from catheters and high local anaesthetic concentrations around the cauda equina, penetrating trauma such as knife
A knife ( : knives; from Old Norse 'knife, dirk') is a tool or weapon with a cutting edge or blade, usually attached to a handle or hilt. One of the earliest tools used by humanity, knives appeared at least 2.5 million years ago, as evidence ...
wounds or ballistic trauma.
Spinal stenosis
CES can be caused by lumbar spinal stenosis
Lumbar spinal stenosis (LSS) is a medical condition in which the spinal canal narrows and compresses the nerves and blood vessels at the level of the lumbar vertebrae. Spinal stenosis may also affect the cervical or thoracic region, in which case ...
, which is when the diameter of the spinal canal narrows. This could be the result of a degenerative process of the spine (such as osteoarthritis
Osteoarthritis (OA) is a type of degenerative joint disease that results from breakdown of joint cartilage and underlying bone which affects 1 in 7 adults in the United States. It is believed to be the fourth leading cause of disability in the w ...
) or a developmental defect which is present at birth. In the most severe cases of spondylolisthesis
Spondylolisthesis is the displacement of one spinal vertebra compared to another. While some medical dictionaries define spondylolisthesis specifically as the forward or anterior displacement of a vertebra over the vertebra inferior to it (or t ...
cauda equina syndrome can result.
Inflammatory conditions
Chronic spinal inflammatory conditions such as Paget disease, neurosarcoidosis
Neurosarcoidosis (sometimes shortened to neurosarcoid) refers to a type of sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, in this type involving the central nervous system (brain and spinal cord). Neurosarcoidosi ...
, chronic inflammatory demyelinating polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called c ...
, ankylosing spondylitis
Ankylosing spondylitis (AS) is a type of arthritis characterized by long-term inflammation of the joints of the spine typically where the spine joins the pelvis. Occasionally areas affected may include other joints such as the shoulders or hi ...
, rheumatoid disease of the spine, and chronic tuberculosis can cause it. This is due to the spinal canal narrowing that these kinds of syndromes can produce.
Risk factors
Individuals most at risk for disc herniation
Spinal disc herniation is an injury to the cushioning and connective tissue between vertebrae, usually caused by excessive strain or trauma to the spine. It may result in back pain, pain or sensation in different parts of the body, and physical ...
are the most likely to develop CES. Race has little influence with the notable exception that African Americans appear slightly less likely to develop CES than other groups.[ Middle age also appears to be a notable risk factor, as those populations are more likely to develop a herniated disc; heavy lifting can also be inferred as a risk factor for CES.][
Other risk factors include obesity and being female.]
Diagnosis
Diagnosis is first suspected clinically based on history and physical exam and usually confirmed by an MRI scan
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
or CT scan, depending on availability. Bladder scanning and loss of catheter sensation can also be used to evaluate bladder dysfunction in suspected cases of cauda equina syndrome and can aid diagnosis before MRI scanning. Early surgery in acute onset of severe cases has been reported to be important.
Prevention
Early diagnosis of cauda equina syndrome can allow for preventive treatment. Signs that allow early diagnosis include changes in bowel and bladder function and loss of feeling in groin. Changes in sensation can start as pins and needles leading to numbness. Changes in bladder function may be changes to stream or inability to fully empty the bladder. If a person progresses to full retention intervention is less likely to be successful.
Management
The management of true cauda equina syndrome frequently involves surgical decompression. When cauda equina syndrome is caused by a herniated disk early surgical decompression is recommended.
Sudden onset cauda equina syndrome is regarded as a medical/surgical emergency. Surgical decompression by means of laminectomy or other approaches may be undertaken within 6, 24 or 48 hours of symptoms developing if a compressive lesion (e.g., ruptured disc, epidural abscess, tumor or hematoma) is demonstrated. Early treatment may significantly improve the chance that long-term neurological damage will be avoided.
Surgery may be required to remove blood, bone fragments, a tumor or tumors, a herniated disc or an abnormal bone growth. If the tumor cannot be removed surgically and is malignant then radiotherapy may be used as an alternative to relieve pressure. Chemotherapy can also be used for spinal neoplasms. If the syndrome is due to an inflammatory condition e.g., ankylosing spondylitis, anti-inflammatory, including steroids can be used as an effective treatment. If a bacterial infection is the cause then an appropriate course of antibiotics can be used to treat it.
Cauda equina syndrome can occur during pregnancy due to lumbar disc herniation. The risk of cauda equina syndrome during pregnancy increases with age of the mother. Surgery can still be performed and pregnancy does not adversely affect treatment. Treatment for those with cauda equina can and should be carried out at any time during pregnancy.
Lifestyle issues may need to be addressed post-treatment. Issues could include the person's need for physiotherapy and occupational therapy due to lower limb dysfunction. Obesity
Obesity is a medical condition, sometimes considered a disease, in which excess body fat has accumulated to such an extent that it may negatively affect health. People are classified as obese when their body mass index (BMI)—a person's ...
might also need to be tackled.
Bowel and bladder control
Rehabilitation of CES depends on the severity of the injury. If permanent damage occurs, then impairment in bladder and bowel control may result. Once surgery is performed, resting is required until the bladder and bowel dysfunction can be assessed. Urinary catheterization
In urinary catheterization a latex, polyurethane, or silicone tube known as a urinary catheter is inserted into the bladder through the urethra to allow urine to drain from the bladder for collection. It may also be used to inject liquids used ...
may help with bladder control. Gravity and exercise can help control bowel movement (Hodges, 2004). Pelvic floor exercise
Kegel exercise, also known as pelvic-floor exercise, involves repeatedly contracting and relaxing the muscles that form part of the pelvic floor, now sometimes colloquially referred to as the "Kegel muscles". The exercise can be performed many t ...
s assist in controlling bowel movements
Defecation (or defaecation) follows digestion, and is a necessary process by which organisms eliminate a solid, semisolid, or liquid waste material known as feces from the digestive tract via the anus. The act has a variety of names ranging f ...
(Pelvic Floor Exercises, 2010). These exercises can be done standing, lying, or on all fours with the knees slightly separated. Full recovery of bowel and bladder control can take as long as two years.
Prognosis
The prognosis for complete recovery is dependent upon many factors. The most important of these is the severity and duration of compression upon the damaged nerve(s). Generally, the longer the time before intervention to remove the compression causing nerve damage, the greater the damage caused to the nerve(s).
Damage can be so severe that nerve regrowth is impossible, and the nerve damage will be permanent. In cases where the nerve has been damaged but is still capable of regrowth, recovery time is widely variable. Surgical intervention with decompression of the cauda equina can assist recovery. Delayed or severe nerve damage can mean up to several years' recovery time because nerve growth is exceptionally slow.
Review of the literature indicates that around 50–70% of patients have urinary retention (CES-R) on presentation with 30–50% having an incomplete syndrome (CES-I). The latter group, especially if the history is less than a few days, usually requires emergency MRI to confirm the diagnosis followed by prompt decompression. CES-I with its more favourable prognosis may become CES-R at a later stage.
Epidemiology
Various etiologies of CES include fractures
Fracture is the separation of an object or material into two or more pieces under the action of stress. The fracture of a solid usually occurs due to the development of certain displacement discontinuity surfaces within the solid. If a displa ...
, abscesses
An abscess is a collection of pus that has built up within the tissue of the body. Signs and symptoms of abscesses include redness, pain, warmth, and swelling. The swelling may feel fluid-filled when pressed. The area of redness often extends ...
, hematomas
A hematoma, also spelled haematoma, or blood suffusion is a localized bleeding outside of blood vessels, due to either disease or trauma including injury or surgery and may involve blood continuing to seep from broken capillaries. A hematoma is b ...
, and any compression
Compression may refer to:
Physical science
*Compression (physics), size reduction due to forces
*Compression member, a structural element such as a column
*Compressibility, susceptibility to compression
* Gas compression
*Compression ratio, of a ...
of the relevant nerve roots A nerve root (Latin: ''radix nervi'') is the initial segment of a nerve leaving the central nervous system. Nerve roots can be classified as:
*Cranial nerve roots: the initial or proximal segment of one of the twelve pairs of cranial nerves leaving ...
.[
] Injuries to the thoracolumbar
The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord (a flexible rod of uniform composition) found in all chordates ...
spine will not necessarily result in a clinical diagnosis of CES, but in all such cases it is necessary to consider. Few epidemiological studies of CES have been done in the United States, owing to difficulties such as amassing sufficient cases as well as defining the affected population, therefore this is an area deserving of additional scrutiny.[
]
Traumatic spinal cord injuries
A spinal cord injury (SCI) is damage to the spinal cord that causes temporary or permanent changes in its function. Symptoms may include loss of muscle function, sensation, or autonomic function in the parts of the body served by the spinal cor ...
occur in approximately 40 people per million annually in the United States, resulting from traumas due to motor vehicle accidents, sporting injuries, falls, and other factors.[
] An estimated 10 to 25% of vertebral fractures will result in injury to the spinal cord
The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...
.[ Thorough physical examinations are required, as 5 to 15% of ]trauma
Trauma most often refers to:
* Major trauma, in physical medicine, severe physical injury caused by an external source
* Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event
*Traumatic i ...
patients have fractures that initially go undiagnosed.[
]
The most frequent injuries of the thoracolumbar
The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord (a flexible rod of uniform composition) found in all chordates ...
region are to the conus medullaris
''Conus'' is a genus of predatory sea snails, or cone snails, marine gastropod mollusks in the family Conidae.Bouchet, P.; Gofas, S. (2015). Conus Linnaeus, 1758. In: MolluscaBase (2015). Accessed through: World Register of Marine Species ...
and the cauda equina
The cauda equina () is a bundle of spinal nerves and spinal nerve rootlets, consisting of the second through fifth lumbar nerve pairs, the first through fifth sacral nerve pairs, and the coccygeal nerve, all of which arise from the lumbar enlarg ...
, particularly between T12 and L2.[ Of these two syndromes, CES is the more common.][ CES mainly affects middle-aged individuals, particularly those in their forties and fifties, and presents more often in men.] It is not a typical diagnosis, developing in only 4 to 7 out of every 10,000 to 100,000 patients, and is more likely to occur proximally.[ ]Disc herniation
Spinal disc herniation is an injury to the cushioning and connective tissue between vertebrae, usually caused by excessive strain or trauma to the spine. It may result in back pain, pain or sensation in different parts of the body, and physical ...
is reportedly the most common cause of CES, and it is thought that 1 to 2% of all surgical disc herniation
Spinal disc herniation is an injury to the cushioning and connective tissue between vertebrae, usually caused by excessive strain or trauma to the spine. It may result in back pain, pain or sensation in different parts of the body, and physical ...
cases result in CES.
CES is often concurrent with congenital or degenerative disease
Degenerative disease is the result of a continuous process based on degenerative cell changes, affecting tissues or organs, which will increasingly deteriorate over time.
In neurodegenerative diseases, cells of the central nervous system stop wo ...
s and represents a high cost of care to those admitted to the hospital for surgery.[ Hospital stays generally last 4 to 5 days, and cost an average of $100,000 to $150,000.][ Delays in care for cauda equina results in the ]English NHS
The National Health Service (NHS) is the publicly funded healthcare system in England, and one of the four National Health Service systems in the United Kingdom. It is the second largest single-payer healthcare system in the world after the ...
paying about £23 million a year in compensation.
In animals
Degenerative lumbosacral stenosis (DLSS), also known as cauda equina syndrome, is a pathologic degeneration in the lumbosacral
The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord (a flexible rod of uniform composition) found in all chordates ...
disk in dogs. DLSS affects the articulation, nerve progression, and tissue and joint connections of the disk. This degeneration causes compressions in soft tissues and nerve root locations in the caudal area of the medulla
Medulla or Medullary may refer to:
Science
* Medulla oblongata, a part of the brain stem
* Renal medulla, a part of the kidney
* Adrenal medulla, a part of the adrenal gland
* Medulla of ovary, a stroma in the center of the ovary
* Medulla of t ...
, causing neuropathic pain
Neuropathic pain is pain caused by damage or disease affecting the somatosensory system. Neuropathic pain may be associated with abnormal sensations called dysesthesia or pain from normally non-painful stimuli (allodynia). It may have continuous ...
in the lumbar vertebra
The spinal column, a defining synapomorphy shared by nearly all vertebrates, Hagfish are believed to have secondarily lost their spinal column is a moderately flexible series of vertebrae (singular vertebra), each constituting a characteristi ...
e.
References
External links
*
{{Spinal cord lesions
Cerebral palsy and other paralytic syndromes
Neurotrauma
Oncological emergencies
Syndromes affecting the nervous system
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