Cauda Equina Syndrome

Definition

Cauda equina syndrome (CES) is a rare but serious neurological condition affecting the
bundle of nerve roots at the lower end of the spinal cord. The CES provides innervation to the
lower limbs, and sphincter,controls the function of the bladder and distal bowel and sensation
to the skin around the bottom and back passage[1].

CES occurs when the nerves below the spinal cord are compressed causing compromise to
the bladder and bowel. The most common cause of CES is a prolapse of a lumbar disc but
other conditions such as metastatic spinal cord compression can also cause CES[1].

There is no agreed definition of CES but the British Association of Spinal Surgeons (BASS)
present a definition that is useful in clinical practice;

'A patient presenting with acute back pain and/or leg pain...... with a suggestion of a
disturbance of their bladder or bowel function and/or saddle sensory disturbance should be
suspected of having a CES. Most of these patients will not have critical compression of the
cauda equina. However, in the absence of reliably predictive symptoms and signs, there
should be a low threshold for investigation with an emergency scan'[2].

Classification
4 groups of patients have been classified according to their presentation :[3]

CESS- Suspected

Patients who do not have CES symptoms but who may go on to develop CES. It is important
that patients understand the gravity of the condition and the importance of the time frame to
seeking urgent medical attention. The use of a *credit card style patient information or a
leaflet explaining what to look for and what to do should they develop symptoms is
recommended.

CESI- Incomplete

Patients who present with urinary difficulties with a neurogenic origin, including loss of
desire to void, poor stream, needing to strain to empty their bladder, and loss of urinary
sensation. These patients could develop CESR and are a medical emergency and should have
a surgical opinion urgently.

CESR -Retention

Patients who present with painless urinary retention and overflow incontinence; the bladder is
no longer under executive control. An urgent surgical opinion is necessary
CESC-Complete

Patients who have objective loss of the cauda equina function, absent perineal sensation, a
loose anus and paralysed bladder and bowel.
[4]

Clinically Relevant Anatomy

The spinal cord ends around L1, consequently, the caudal nerve roots below the first lumbar
root, form the cauda equina. The roots descend at an almost vertical angle to reach their
corresponding foramina, gathered around the filum terminale within the spinal theca[5]. The
proximal portion of the cauda equina is said to be hypovascular hence more vulnerable if
compressed [6]. The cauda equina roots have both a dorsal and ventral root. The ventral root
provides motor fibres for the efferent pathway along with sympathetic fibres. The dorsal root
is composed of afferent fibres for the transmission of sensation. The functions of those nerves
are:

 Sensory and motor fibres to the lower limbs.

 Sensory innervation to the saddle area.
 Voluntary control of the external anal and urinary sphincters.

Aspects of anatomical features relating to saddle sensation, bladder, bowel and sexual
function are discussed below;

The first three sacral nerves, S1,2 and 3 supply multifidus and lateral cutaneous branches to
the skin and fascia over the sacrum and part of the gluteal region. The 4th and 5th sacral
nerves, S4 and 5, along with posterior primary ramus of the coccygeal nerve supply the skin
and fascia around the coccyx. The pelvic splenic nerves to the pelvic viscera composed of
parasympathetic fibres, travel in the ventral rami of S2,3 and 4. They then leave these nerves
as they exit the anterior sacral foramina and pass to the pre-sacral tissue. Some pass to the
pelvic viscera alongside the pelvic sympathetic supply and supply the urogenital organs and
distal aspect of the large intestine. Others pass immediately into retroperitoneal tissue and
into the mesentry of the sigmoid and descending colon [5]. The pudendal nerve supplies the
perineum and arises from S2,3 and 4 with its terminal branches including the dorsal nerve of
the penis or clitoris[7].

Epidemiology
CES occurs as a consequence of compression of the cauda equina and can be caused by a
number of pathologies. The prevalence among the general population has been estimated
between 1:100 000 and 1:33 000. The most common cause of CES is herniation of a lumbar
intervertebral disc[8] and accounts for 2% of all herniated lumbar discs.[9] It commonly
affects the discs at the L4/5 and L5/S1 level . However disc prolapse at any lumbar level can
cause CES. Patients may be predisposed to CES if they have a congenitally narrow spinal
canal or have acquired spinal stenosis. [10]The prevalence among patients with low back pain
is approximately four in 10 000[11].
CES affects males and females equally and can occur at any age but primarily in adulthood.
[12]
Other pathologies which can cause CES include spinal stenosis, haematoma, trauma
tumour, infection, fracture and inflammatory conditions. [1][13][14]

Other rare causes such as abdominal aortic dissection, and complications after surgery,
anesthetic procedures, spinal manipulation or epidural injections are possible causes of
CES[1].

Clinical Presentation
5 characteristic features of CES are consistently described in the literature and should form
the basis of questions related to diagnosis[3];

1. Bilateral neurogenic sciatica - Pain associated with the back and/ or

unilateral/bilateral leg symptoms maybe present.
2. Reduced perineal sensation - Sensation loss in the perineum and saddle region is the
most commonly reported symptom.
3. Altered bladder function leading to painless retention - Bladder dysfunction is the
most commonly reported symptom and can range from increased frequency ,
difficulty in micturition, change in stream, incontinence and retention.
4. Loss of anal tone - loss or reduced anal tone may be evident if a patient reports bowel
dysfuntion. Bowel dysfunction may include incontinence, inability to control motions,
inability to feel when the bowel is full and consequently overflow.
5. Loss of sexual function - Sexual dysfunction is not widely mentioned in the literature
but is an important aspect that should be discussed with patients.

Examination
Subjective examination

The difficulty with diagnosing serious spinal conditions early and the catastrophic outcomes
of delayed diagnosis are widely documented [15][16]. The subjective history is the most
important aspect of the examination early in the disease process as the subtle and vague
symptoms related to early Cauda Equina Syndrome need to be identified using clear methods
of communication. Good communication skills allow us to gain an understanding of the
patient’s world by achieving an understanding of what patients perceive is happening to
them[17] . The important items to screen within the subjective history are Red Flags. It is well
recognized that the presence of Red and Yellow Flags are not mutually exclusive [18]. The
clinical reasoning process essentially combines a biopsychosocial assessment alongside this
Red Flag screening to get a full true picture of the patient’s story and current clinical
presentation. Establishing the history of the present condition in detail is key as timing is of
paramount importance in this condition.

 When the back and or leg pain started is significant but precisely when symptoms
relating to parasympathetic supply began is vital; one hour, one day, one week, 15
years? There is no way of predicting who will progress from CESS to CESR and how
quickly this may happen and so precise recording of the timing of chronology cannot
be underestimated.
  Establish if things are changing, better, episodic, worse or the same. Improving pain
does not necessarily mean the condition is improving. Checking Red Flags and
neurological status is important before this improved status can be assumed. Constant
pain and night pain must be viewed along with all Red Flags with caution.
 Establish the pattern of pain through 24 hours. Reference of pain and precise area of
pins and needles and numbness must be identified and clearly documented.
Aggravating and easing factors should be explored. Establish if these symptoms have
been experienced before or are they different?
 Has an MRI been performed with these current symptoms? This seems so obvious but
can help with the clinical reasoning process.
 What treatments have been tried including medication is helpful on a variety of
levels. Many medications cause symptoms that masquerade as CES[19]. This does not
mean that symptoms can be ignored and attributed to drugs, however, medication
could be contributing to the bladder, bowel and sexual dysfunction. Similarly, pain
can cause retention.
 Explore the patient’s medication regime and escalation up the analgesic ladder? Is
medication being used appropriately and titrated correctly? This can give an
indication of the severity of pain and its control. Establish the quality and intensity of
pain e.g VAS.
 What is the past medical history status; previous diagnosis of disc pathology or spinal
stenosis for instance may be significant. Previous history of serious conditions such
as cancer must be noted and may be important. Similarly many co-morbidities could
masquerade as CES e.g. Diabetes, Multiple Sclerosis, Benign prostatic hyperplasia,
pregnancy.
 Has there been any recent or past spinal surgery and any history of osteoporosis; a
retropulsed vertebral insufficiency fracture could cause CES.

If CES/CES risk is suspected the subjective history must explore symptoms in even more
detail. Tools and questions to use are covered in the next Research section. It is important that
these questions are framed to highlight their gravity. The patient needs to recognise that the
next questions are vital and accurate response of the utmost importance.

Communication

A Qualitative research study has identified that clear communication plays a pivotal role in
identifying Cauda Equina Syndrome patient’s early to facilitate bringing these patients to the
surgical team in a timely manner [20]. Through this study it emerged that in order to identify
CES patients early in the disease process to facilitate a timely surgical opinion one of the key
problems was the use of language that reflected the patient’s own voice. The patient
participants emphasised the need for clinicians to use language that they could understand
during a clinical consultation, especially in the context of severe pain. A CES cue card for
clinicians to use in the clinical consultation to enable the patient to focus on important
questions was developed. It enables clinicians to frame the questions as important. The
clinical cue card maps against a patient credit card using the same questions. This highlights
symptoms to look out for and crucially timely action to take should symptoms develop. *The
credit card could be used by the patient particularly in an emergency setting to help express
the change in embarrassing and sensitive symptoms.

*Download the patient credit card

Physical examination

The physical examination should include a full neurological assessment to determine

dermatomal sensory loss, myotomal weakness and reflex change. Where a patient reports
bilateral leg pain, signs of upper motor neuron involvement should be examined (babinski
and clonus). For a comprehensive overview of neurological integrity testing the reader is
referred to the following book 'Neuromusculoskeletal examination and assessment' [21].

Where a patient reports sensory changes in the perineal area this should be tested to evaluate
any sensory loss. A digital rectal examination should be performed to assess any loss of anal
sphincter tone. This should only be performed by an appropriately trained clinician. Reduced
sensation of the perineum and/or anal tone is objective evidence of CESI and CESR but are
likely to be normal in CESS[3].

Diagnostic Procedures
The diagnosis of cauda equina syndrome is based on the patients reported subjective history.
Physical examination findings may help to confirm the diagnosis but should not be solely
relied on. If CES is suspected the patient must undergo an MRI urgently to confirm the
diagnosis. It is important to understand your locally agreed pathway to make sure there is no
delay to diagnosis and where CES is confirmed, there is no delay to surgical intervention.

While MRI, coupled with patient history and examination, remains the diagnostic gold
standard, it comes at a high cost with many patients demonstrating no concordant
pathology.[22]

Key Evidence
Cauda equina syndrome is a grey area and there is no consensus on which signs and
symptoms should be acted on. However it can have life changing consequences and it is
important to act quickly if it is suspected.

Litigation

The scale and impact of claims for negligence against clinicians treating people with CES is
significant, and rising. Whilst it is difficult to accurately collate international statistics, there
are robust data for the UK, which are presented below. These are taken from national
agencies dealing with litigation against medical professionals (Medical Defence Union-
MDU, and the National Health Service Litigation Authority-NHSLA) [23].

Taylor [24] analysed claims made to the MDU between 2005 and 2016 related to CES. In that
period there were 150 claims made-92% against GPs. The majority of these were successfully
defended (70%) though the MDU paid out 350 000 pounds ($456,340) in legal costs. Over
the same time period, £8 million ($10.4m) was paid out on settled claims, most of which
were under £100 000 ($130 000). 4.5m of this was in solicitors’ fees. Around 12% of claims
were for more than 500 000 pounds ($650 000).
The NHSLA (2016) examined claims for CES from 2010-2015. Of the 293 cases identified,
232 were still under investigation and unsettled; 20 had settled with agreed damages; 41 had
concluded with no damages awarded. Overall £25 million had been paid out. The survey
identified that 70% of patients involved in claims were aged between 31-50.

Other data suggests that average payouts for CES claims in the UK are around £336 000
($436 800), with around £133 000 of that going to the patient and the remainder on legal
costs. US data suggests average payouts are $549 427 (£422 636)

Although not specifically focused on CES, a study by Taylor in 2014 of litigation cases in the
USA against neurosurgeons, found that they were more likely to be sued following spinal
surgery than cranial surgery, with the average claim being around $278 362. A similar study,
relating to neurosurgical litigation in the UK [25], found that the highest number of claims
related to spinal surgery (44%) and that 87.5% of claims relating to CES were successful.

It is clear that litigation for CES is only likely to increase, and equally clear that as treating
healthcare professionals, we need to ensure that we examine patients fully and appropriately,
that we warn, or “safety net” them where we have concerns, and that we have robust
pathways in place to ensure rapid access to MRI scanning and spinal surgical specialists.

Clinical Bottom Line

Cauda equina syndrome is rare but can have life changing consequences if not acted upon in
a timely manner. If surgical intervention is delayed irreversible damage can occur to the
bladder, bowel and sexual function.

Relevant symptoms include unilateral or bilateral radicular pain and/or dermatomal reduced
sensation and/or myotomal weakness with any suggestion of change in bladder or bowel
function however minor should be investigated[3].

Nothing is to be gained by delaying surgery and should be carried out as soon as is practically
possible[2][3].

Safety netting

Not all patients with back pain will develop CES and it is not necessary to warn all patients.
Those patients whom you suspect may go onto develop CES should be given the appropriate
information and know what to do should they go on to develop symptoms.

Communication

Patients need to understand the relevance of the questions you ask as they may not fully
appreciate the importance and subsequent consequences if not explained properly.

Documentation

It is important that a patients signs and symptoms are fully documented in accordance to your
governing bodies standards of practice so there is a clear record of the patients journey.
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