Cauda Equina Syndrome: Innovait October 2011

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Cauda Equina Syndrome

Article  in  InnovAiT · October 2011


DOI: 10.1093/innovait/inq205

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INNOVAIT inq205 VD
InnovAiT, Vol. 0, No. 0, pp. 1–5, 2011 doi:10.1093/innovait/inq205
JOURNAL NAME MS No. CE Code

Cauda equina syndrome

C
auda equina syndrome (CES) is a relatively uncommon condition typically
associated with a large space-occupying lesion within the canal of the
lumbosacral spine. It is one of the few spinal surgical emergencies and its
prompt diagnosis and surgical treatment are of paramount importance as, otherwise,
5 patients could be left with permanent and disabling neurological deficits involving
lower limb sensorimotor function, bladder, bowel and sexual function. Furthermore,
and as a result of perceived delays in diagnosis and treatment culminating in a
suboptimal functional outcome, CES carries a disproportionately high medicolegal
profile. This article aims to provide a simple overview of lumbar disc prolapse, CES
10 and its initial management in primary care.

between 1 in 33 000 and 1 in 100 000 people. The L4/5 level


The GP curriculum and cauda equina syndrome
is frequently quoted as being the most commonly affected
Curriculum statement 15.7: Neurological problems level (57%), with other involved levels being L5/S1 (30%)
requires GPs to be able to and L3/4 (13%).
 Manage neurological emergencies competently

 Manage primary contact with patients who have a Pathophysiology 30


neurological problem The intervertebral disc permits stable motion of the spine
 Practice the key competence of making appropriate while supporting and distributing loads under movement.
referrals for neurological problems It is composed of the ‘annulus fibrosus’, which is the
 Describe the indications for referral to a neurologist multilaminated ligament that encompasses the periphery of
for conditions that are irreversible without early the disc space, and the ‘nucleus pulposus’, which is the 35
treatment gelatinous central portion of the disc.
 Coordinate care with other primary care health

professionals such as occupational therapists, The majority of clinically significant disc herniations occur in
physiotherapists and district nurses to enable a posterolateral direction for two probable reasons:
rehabilitation  The nucleus pulposus is located somewhat posteriorly
 Recognize that neurological conditions can impact within the disc space 40
upon a family’s social and economic well-being  The posterior longitudinal ligament reinforces the

annulus fibrosus in the midline posteriorly

Lumbar disc prolapse The usual posterolateral herniation impinges upon the
ipsilateral nerve root at its exit from the dural sac rather
Epidemiology than in the exit foramen. Thus, a right L4/5 disc prolapse 45
Lumbar disc herniation represents one of the most compresses the right L5 nerve root. However, if the disc
common clinical problems encountered by a neurosurgeon. prolapse is more laterally placed, i.e. a far lateral disc
15 Approximately, 50% of working adults will experience back herniation, then it impinges upon the ipsilateral nerve root
pain in any given year and, of this population, many will be exiting through the adjacent exit foramen —right L4 nerve
subsequently diagnosed with a lumbar disc herniation. The root in this example. Discrete nerve root compression as 50
vast majority of cases of lumbar disc herniation are managed described here causes radicular symptoms and signs in the
effectively and successfully with a conservative approach distribution of a specific nerve root. A large central disc
20 comprising close observation, analgesia and physiotherapy. prolapse may compress several elements of the cauda equina
In a small minority of cases, where symptoms persist beyond on both sides of the midline, producing CES.
a 3–6 month period, or where they recur, microdiscectomy
should be considered in suitable patients. Aetiology of CES and differential diagnosis 55
By far, the most common cause of CES is a large centrally
Only 2% of all herniated lumbar discs result in cauda equina prolapsed lumbar disc. There are, however, other more
25 syndrome (CES), the incidence of which is estimated to be unusual aetiologies that can result in a compressive

© The Author 2011. Published by Oxford University Press on behalf of the RCGP. All rights reserved.
For permissions please e-mail: journals.permissions@oup.com
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lesion of the cauda equina with all the associated vary, but the most consistent clinical sign is urinary retention
60 symptoms and signs of CES. These include trauma, intra- (Table 1). 75
and extra-medullary spinal tumours, metastatic spinal
tumours, lymphomas, degenerative spondylotic disease, Three classic patterns of presentation have been described:
haematomas (either traumatic or associated with  Type 1: Presents acutely as the first symptom of lumbar
anticoagulation), iatrogenic , e.g. following lumbar disc disc prolapse
65 surgery, and epidural lipomatosis (Box 1).  Type 2: Presents as the endpoint of a long history of

chronic lower back pain with or without sciatica 80


 Type 3: Presents insidiously with slow progression to
Box 1. Unusual causes of CES numbness and urinary symptoms
 Trauma
Once CES has been diagnosed, it is important to differentiate Q2
 Intrinsic/extrinsic spinal tumours
between two clinical categories identified by most clinicians:
 Metastatic spinal tumours
 Incomplete cauda equina syndrome (CESI): Subjective and/ 85
 Lymphoma
or objective evidence of neurological losses such as
 Degenerative spondylotic disease
impaired bladder sensation, impaired urethral sensation,
 Haematoma
impaired rectal sensation and/or objective genital/perianal
 Iatrogenic
(S3–S5) sensory disturbance and/or reduction in anal tone,
 Epidural lipomatosis
provided the bladder is still functioning normally 90
 Complete cauda equina syndrome with retention (CESR):

Paralysis of bladder leading to established painless


Diagnosis of CES urinary retention with overflow incontinence

Clinical presentation ‘Red flag’ symptoms


The diagnosis of CES is usually made from the history and There are a group of patients who are at high risk of 95
physical examination. Combined motor and sensory deficits developing CES who can be identified by the so-called ‘red
70 are often found and usually include bilateral leg weakness, flag’ symptoms. These include:
reduced straight leg raise (SLR), decreased reflexes, saddle  Severe lower back pain

anaesthesia and bowel/bladder disturbance. The presence  Motor weakness, sensory loss or radicular pain (usually

and diagnostic value of each of these symptoms in CES can bilateral) 100

Table 1. Presenting symptoms of CES

Bladder Bladder dysfunction or difficulty with micturition can be variable. Urinary retention has a sensitivity
of 90% and a specificity of about 95% for the diagnosis of CES in those patients with the
appropriate history and physical examination. If urinary incontinence is present, it is secondary to
overflow incontinence from underlying acute urinary retention. A PVR should be obtained. If there is
greater than 100–200 ml of residual urine, then urinary retention is likely present.

Bowel Patients present with symptoms ranging from constipation to incontinence, although this is rarer in
complete CES because of an inability to appreciate filling of the rectum.

Anal tone Anal sphincter tone is diminished in up to 80% of patients. A rectal examination will assess perineal
sensation and anal sphincter tone.

Pain Approximately 70% of patients with CES have a history of chronic back pain. Lower limb pain is
implicated as a poor prognostic indicator in CES, with bilateral pain (present in only 20% of patients
with a central disc prolapsed) found to be worse than unilateral radiculopathy.

Sensation The most common sensory deficits in CES occur over the buttocks, posterior thighs and perineal
region. Approximately, three quarters of patients with CES present with anaesthesia of the perineum
and saddle area. Loss of sensation in the saddle distribution is said to be the most striking feature of
CES when found in conjunction with sphincter disturbances.

Power Weakness may be minimal if present at all in CES, and while apparently less important in the clinical
presentation than bladder or bowel dysfunction, it should nonetheless be assessed in patients with
suspected CES.

Reflexes Reflexes that may be decreased in CES include the patellar, ankle, detrusor, bulbocavernous and
cremasteric reflexes.

Sexual function General sexual dysfunction, erectile dysfunction or ejaculatory dysfunction may be present though
less commonly. Patients may describe reduced sensation during intercourse.

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 Loss of lower limb reflexes


 Saddle anaesthesia
 Recent onset of bladder dysfunction (i.e. urinary
retention or incontinence)
105  Recent onset of faecal incontinence
 Recent onset of sexual dysfunction

Key questions
Pertinent questions during history taking will help to
discern red flag symptoms and thus differentiate CES from
110 other causes of back or leg pain. It is important to establish
the precise time of onset of symptoms, particularly bladder
or bowel disturbances or saddle anaesthesia. Patients
with known disc prolapse, particularly those who have
undergone previous treatment or surgery, will also be more
115 at risk of developing CES. Other focused questions should
include:
Do you have difficulty starting to urinate? (Urinary
hesitancy)
Can you feel yourself passing urine? (Reduced urethral
120 sensation)
Do you experience dribbling after urinating? (Overflow
incontinence)
When you try to pass urine is it painful? (Painful
retention)
125 Can you feel the toilet paper and does it feel normal?
(Saddle anaesthesia)
Have you noticed any recent change in your ability to
maintain erections? (Sexual dysfunction)

It seems that no individual symptom is able to accurately


130 predict the presence of a clinically relevant disc prolapse,
and because of the difficulty in excluding the diagnosis
without magnetic resonance imaging (MRI), urgent MRI
Figure 1. Sagittal and axial T2W MR images clearly demonstrating Q4
assessment is generally recommended in all patients who a large central disc prolapse at the L5/S1 level with significant
present with new onset urinary symptoms in the context of impingement upon the cauda equina. Q5
135 lumbar back pain or sciatica.

A word of warning with and/or without contrast or lumbar myelography is


CES is a clinical area that attracts a high risk of litigation. alternative imaging options.
Although symptoms have poor predictive value on their
own for the syndrome, it is important to document Bladder ultrasonography may be used to measure post-
140 the nature and onset of bladder, bowel and sexual void residual (PVR) urine. A PVR urine of greater than
symptoms and to make a timely referral to the local spinal 100 ml is generally defined as incomplete bladder emptying. 160

surgery service for appropriate investigation and further Catheterization for residual urine volume may also help
management. reveal neurogenic urinary retention.

Investigations Management of CES


145 Plain X-ray of the lumbar spine is helpful in the presence of
trauma to rule out fracture and can help diagnose destructive The algorithm shown in Table 2 summarizes the management
changes, disk-space narrowing or spondylolisthesis. However, of suspected CES. When a patient has clinical and radiological 165
it is of little help in CES. If CES is suspected, do not delay features of CES, current consensus recommends surgical
for investigation in primary care and refer immediately to decompression. Some causes of CES such as tumour clearly
150 hospital as a same-day emergency. require detailed assessment of the nature and extent of the
pathology. However, for CES secondary to lumbar disc
In secondary care, superiority of MRI over computed herniation, emergency surgical decompression is indicated 170
tomography (CT) is well established, with far greater at the level of the herniation, usually involving discectomy
detail of soft tissue anatomy enabling more confident and sometimes also comprising laminectomy to allow
radiological diagnosis ( Fig. 1). In patients for whom MRI optimal exposure and maximize neural decompression
155 is contraindicated (e.g. pacemaker, metallic implants ), CT ( Fig. 2).

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Table 2. Algorithm for management of suspected CES

Clinical category Presenting features Management

Unlikely to have CES  Difficulty in passing urine  Admit


 Particularly in the context  Pain relief
of severe pain  MRI the following day
 No subjective or objective
neurological deficit

High risk of  ‘Red flags’  Admit


developing CES  No features of CES  Pain relief
 MRI the following day

CESI  Subjective or objective  Emergency MRI


evidence of CES  Emergency decompressive surgery prior to the
 No bladder paralysis onset of CESR
 Debatable as to whether surgery should be
carried out as an emergency out-of-hours or
on the next day list

CESR  Bladder paralysis  Due to scientific uncertainty, advise adopting


precautionary principle, i.e. patients should be
decompressed urgently
 Whether such patients should be operated as
an emergency out-of-hours remains debatable

Source: Todd, N.V. An algorithm for suspected cauda equina syndrome. Annals of The Royal College of Surgeons of
England (2009) 91(4): p. 358–9. Accessed via www.ncbi.nlm.nih.gov/pmc/articles/PMC2749433/.

175
The operation can be very technically demanding and of
course is not without risk. Further damage to nerve roots
can occur resulting in motor or sensory dysfunction, and
there is risk of dural tear leading to cerebrospinal fluid (CSF)
leak. Recurrence and reoperation are also risks and more
180
likely in patients who have already undergone previous
surgery.

Timing of surgery remains the most controversial aspect


of CES management. A meta-analysis by Ahn et al. in
2000 is the most widely quoted regarding urgency of
surgery. It suggests that intervention less than 48 hours 185
after the onset of symptoms will produce a better
outcome. However, these data have been reanalysed, and
it is suggested that the outcome for both types of CES is
better when there is surgical intervention within 24 hours
(Jerwood and Todd, 2006). Despite this, some argue that 190
patients with CESR have a poor clinical outcome
regardless of timing of surgery and so should wait until
the next morning’s elective list rather than having a
potentially difficult operation out-of-hours. A recent
meta-analysis supports the view that early surgery is 195
related to better results with CESI, but the case for early
surgery for CESR is less certain (DeLong et al., 2008).

Prognosis
In the UK, 43% of patients referred from primary care to
hospital will subsequently have normal MRI scans, and 200
Figure 2. Sagittal and axial T2W magnetic resonance (MR) images approximately 20% will be found to have lumbar disc
depicting post-operative appearances in the same patient featured
in Fig. 1. A laminectomy has been performed and the disc prolapse
prolapses requiring urgent surgery. One reason for the high
excised resulting in a satisfactory decompression as evidenced by number of false positive diagnoses being referred is that
the nerve roots seen to be freely floating in CSF. pain, regardless of aetiology, can result in difficulty with

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InnovAiT

205 micturition, possibly due to excessive sympathetic tone. In


fact, the most common cause of difficulty in passing urine in prolapse. British Journal of Neurosurgery (1990) 4:
patients with lumbar degenerative disorders is pain rather p. 205–9 255
than CES.  Henriques, T., Olerud, C., Petren-Mallmin, M., Ahl, T.
Cauda equina syndrome as a postoperative complication
Early recognition and treatment of CES is associated with
in five patients operated for lumbar disc herniation.
210 improved outcome with respect to bladder function.
Spine (Philadelphia Pa 1976) (2001) 26 (21):
Outcomes in patients treated at the time of CESI are
generally favourable, whereas outcomes following bladder p. 2404–5 Q3 260

paralysis (CESR) are less favourable. If male patients present  Jerwood, D., Todd, N.V. Reanalysis of the timing of
with erectile dysfunction, this usually has a poor prognosis. cauda equina surgery. British Journal of Neurosurgery
215 For these reasons, failure to recognize and treat this (2006) 20: p. 178–9
condition expediently may have outcome and medicolegal  Lefresne, S., Fairchild, A., Bistritz, A., Venner, P., Yee,
implications. Litigation is more common in patients with D. A case of indirect cauda equina syndrome from 265
residual symptoms. metastatic prostate cancer. Canadian Urological
Association Journal (2009) 3 (4): p. E31–5
 Liao, J.C., Fu, T.S., Chen, W.J., Jung, S.M. Dumbbell-
Key points shaped Hodgkin’s disease with cauda equina
220  The most consistent clinical sign of CES is urinary compression mimicking a herniated inter-vertebral 270
retention disc: a case report. Chang Gung Medical Journal
 Clinical diagnosis is associated with a 43% false (2007) 30 (5): p. 458–63
positive rate  Mixter, J.M., Barr, J.S. Rupture of the intervertebral
 Investigation of choice is MRI of the lumbar spine disc with involvement of the spinal canal. New
225  Surgery is highly recommended within 24 hours for England Journal of Medicine (1934) 211: p. 210–5 275
CESI
 Radulovic, D., Tasic, G., Jokovic, M., Nikolic, I. The
 Surgery is debatably recommended within 24–48
role of surgical decompression of cauda equina in
hours for complete CES
 Prognosis is worse in the presence of urinary lumbar disc herniation and recovery of bladder
230 retention function. Medicinski Pregled (2004) 57 (7–8):
 Litigation is more common in patients with residual p. 327–30 280
symptoms  RCGP Curriculum statement 15.7: Neurological
problems. Accessed via www.rcgp-curriculum.org
.uk/pdf/curr_15_7_Neurological_problems.pdf [date
last accessed 18.08.2010]
REFERENCES AND FURTHER INFORMATION  Riffaud, L., Adn, M., Brassier, G., Morandi, X. Acute 285
 Ahn, U.M., Ahn, N.U., Buchowski, J.M., Garrett, cauda equina compression revealing Hodgkin’s
235 E.S., Sieber, A.N., Kostuik, J.P. Cauda equina disease: a case report. Spine (Philadelphia Pa 1976)
syndrome secondary to lumbar disc herniation: a (2003) 28 (14): p. E270–2
meta-analysis of surgical outcomes. Spine (2000)  Schizas, C., Ballesteros, C., Roy, P. Cauda equina
25: p. 1515–22 compression after trauma: an unusual presentation of 290
 Bell, D.A., Collie, D., Statham, P.F. Cauda equina spinal epidural lipoma. Spine (Philadelphia Pa 1976)
240 syndrome: what is the correlation between clinical (2003) 28 (8): p. E148–51
assessment and MRI scanning? British Journal of  Todd, N.V. Cauda equina syndrome: the timing of
Neurosurgery (2007) 21: p. 201–3 surgery probably does influence outcome. British
 DeLong, W.B., Polissar, N., Neradilek, B. Timing of Journal of Neurosurgery (2005) 19: p. 301–6 295
surgery in cauda equina syndrome with urinary  Todd, N.V. An algorithm for suspected cauda equina
245 retention: meta-analysis of observational studies. syndrome. Annals of the Royal College of Surgeons of
Journal of Neurosurgery: Spine (2008) 8: p. 305–20 England (2009) 91 (4): p. 358–9. Accessed via www
 Gleave, J.R., MacFarlane, R. Prognosis for recovery of .ncbi.nlm.nih.gov/pmc/articles/PMC2749433/ [date
bladder function following lumbar central disc last accessed 16.12.2010] 300

Mr Kenan Deniz
250 Specialist Registrar in Neurosurgery, National Hospital for Neurology & Neurosurgery, London
Q1 E-mail: kenandeniz@doctors.org.uk
Dr Jonathan Pararajasingham
Senior House Officer in Neurosurgery, National Hospital for Neurology & Neurosurgery, London

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