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Cauda Equina Syndrome

CES

Cauda equina syndrome is a rare disorder characterized by a group of symptoms resulting from compression of the nerve roots of the cauda equina.


Presentation

Patients initially present with lower back pain in almost all cases, usually with sciatica and pain in the genitalia and rectum [10]. Impaired sphincter function occurs in 15-30% of patients [10].

The clinical picture of cauda equina syndrome includes [2] [13]:

  • Lower back pain 
  • Neurological deficits (distal motor weakness, sensory disturbance in the legs, numbness and pain, absent lower-limb reflexes)
  • Urological symptoms (bladder and bowel incompetence, impotence may occur with erectile dysfunction due to the lack of sensation in pubic area [14])
  • Scoliosis and foot deformities. 

Diagnosis and treatment are often delayed because the symptoms are not recognized immediately [6]. Cauda equina syndrome may present as acute, sub-acute or insidious [1] [4] [15]. The precipitating factor in the acute form may be traumatic injury or movements as simple as stooping, with or without heavy lifting [15].

Urinary manifestations include the following [4] [7]:

Bowel disturbances may include the following [7]:

Complications include [4]:

Spontaneous Hemorrhage
  • This article describes a case of subacute cauda equina syndrome resulting from spontaneous hemorrhage into an upper lumbar synovial cyst. A 65-year-old man presented with a 3-month history of intermittent bilateral lumbar pain.[ncbi.nlm.nih.gov]
Lower Extremity Pain
  • A 37-year-old woman, with history of intravenous drug abuse, hepatitis C, and hepatitis B, presented with low back pain lasting 2 months, lower extremity pain, left greater than right with increasing weakness and difficulty ambulating, and urinary and[ncbi.nlm.nih.gov]
  • Classically, the full-blown syndrome includes urinary retention, saddle anesthesia of the perineum, bilateral lower extremity pain, numbness, and weakness. Decreased rectal tone may be a relatively late finding.[ncbi.nlm.nih.gov]
Hoover's Sign
  • Abstract In the first prospective comparison of 'scan-negative' (n 11) and 'scan-positive' (n 7) patients with cauda equina syndrome (CES) we found that Hoover's sign of functional leg weakness but not routine clinical features differentiated the two[ncbi.nlm.nih.gov]
Fecal Incontinence
  • After catheter removal, the patient developed urinary retention, fecal incontinence, and perianal hypoesthesia.[ncbi.nlm.nih.gov]
  • The patient initially showed no neurological deficits after the gunshot injury but after 15 months he presented again with urinary and fecal incontinence.[ncbi.nlm.nih.gov]
  • Post-operatively, she developed urinary and fecal incontinence as the sole presenting symptom of communicating post-hemorrhagic hydrocephalus.[ncbi.nlm.nih.gov]
  • I cannot help but emphasize one how important it is to realize that it is URINARY RETENTION and FECAL INCONTINENCE that are hallmarks of this syndrome.[canadiem.org]
Pelvic Mass
  • Classical presentation includes abdominal pain or a pelvic mass in female patients with primary amenorrhea.[ncbi.nlm.nih.gov]
Lower Abdominal Pain
  • We present the case of a 32-year-old woman with CES who presented to the emergency department with gradually worsening lower abdominal pain.[ncbi.nlm.nih.gov]
Back Pain
  • Low back pain during pregnancy is a common cause of medical consultation. Although back pain is very common, the incidence of low back pain secondary to lumbar disk herniation in pregnancy is low (1: 10,000).[ncbi.nlm.nih.gov]
  • Back pain prevalence in the pediatric age group is less compared with adults. There is a wide range of possible etiologies, and tumors such as primary spinal hemangiomas are uncommon.[ncbi.nlm.nih.gov]
  • He had a 1-year history of low back pain diagnosed as disk herniation and managed conservatively but had experienced recent onset of a similar pain and new onset of nocturnal back pain causing sleep disturbance.[ncbi.nlm.nih.gov]
  • […] and represents a surgical emergency. 1 Sixty percent of patients are male with a mean age of 42 years, and 82% have a history of chronic low-back pain.[dx.doi.org]
  • A 44-year-old lady with a history of lumbar back pain presented to the emergency department complaining of severe back pain radiating to her buttocks.[ncbi.nlm.nih.gov]
Low Back Pain
  • Low back pain during pregnancy is a common cause of medical consultation. Although back pain is very common, the incidence of low back pain secondary to lumbar disk herniation in pregnancy is low (1: 10,000).[ncbi.nlm.nih.gov]
  • Aim To develop a simple cauda equina syndrome (CES) toolkit to facilitate the subjective examination of low back pain patients potentially at risk of CES. To undertake preliminary validation of the content of the toolkit.[ncbi.nlm.nih.gov]
  • This syndrome presents as low back pain, motor and sensory deficits in the lower extremities, and bladder as well as bowel dysfunction. Although various etiologies of cauda equina syndrome have been reported, a less common cause is infection.[ncbi.nlm.nih.gov]
  • He had a 1-year history of low back pain diagnosed as disk herniation and managed conservatively but had experienced recent onset of a similar pain and new onset of nocturnal back pain causing sleep disturbance.[ncbi.nlm.nih.gov]
  • A 13-year-old female, follow-up in an outer health care center due to a L1 vertebral hemangioma, characterized by 3 years of low back pain without neurologic symptoms presented to our emergency department with an acute cauda equina syndrome.[ncbi.nlm.nih.gov]
Buttock Pain
  • .  Convoy machine gunner  Prolonged periods of standing 8 hrs  Wearing equipment up to 80 lbs  4 week history of insidious onset and recent worsening of:  Low back pain  Left buttock pain  Posterior left thigh pain  Goal: Decrease pain during[slideshare.net]
Nocturnal Back Pain
  • He had a 1-year history of low back pain diagnosed as disk herniation and managed conservatively but had experienced recent onset of a similar pain and new onset of nocturnal back pain causing sleep disturbance.[ncbi.nlm.nih.gov]
Distractibility
  • This would remove the 87% of patients not requiring urgent surgery from an unnecessary and distracting referral process.[ncbi.nlm.nih.gov]
  • Thus suddenly distracts the vertebral bodies anteriorly but does not close them posteriorly. The nucleus receives a sudden suction force which tends to displace it anteriorly, i.e. away from the sensitive area of the annulus.[macdonaldpublishing.com]
Foot Drop
  • Most of the symptoms resolved within a few days, but right side foot drop persisted for 2 years after the procedure.[ncbi.nlm.nih.gov]
  • CES can lead to pain, numbness, and weakness in the lower back, pelvic area and legs; " foot drop "; problems with bowel or bladder control; sexual dysfunction; and even paralysis.[rarediseases.info.nih.gov]
  • The person may experience, 'foot drop,' where their foot drags and may cause trips and falls. In some instances, it may be necessary for the person to use walking aids or a wheelchair.[disabled-world.com]
Numbness in the Buttocks
  • He also complained of newly developed numbness in the buttocks, groins, and perineum, and difficulty with urination and defecation, after repeated caudal epidural injections during a 3-week period.[ncbi.nlm.nih.gov]
Sensory Deficit of the Lower Extremity
  • This syndrome presents as low back pain, motor and sensory deficits in the lower extremities, and bladder as well as bowel dysfunction. Although various etiologies of cauda equina syndrome have been reported, a less common cause is infection.[ncbi.nlm.nih.gov]
Sensory Deficit of the Lower Extremity
  • This syndrome presents as low back pain, motor and sensory deficits in the lower extremities, and bladder as well as bowel dysfunction. Although various etiologies of cauda equina syndrome have been reported, a less common cause is infection.[ncbi.nlm.nih.gov]
Sleep Disturbance
  • He had a 1-year history of low back pain diagnosed as disk herniation and managed conservatively but had experienced recent onset of a similar pain and new onset of nocturnal back pain causing sleep disturbance.[ncbi.nlm.nih.gov]
Urinary Retention
  • After catheter removal, the patient developed urinary retention, fecal incontinence, and perianal hypoesthesia.[ncbi.nlm.nih.gov]
  • retention with overflow incontinence.[ncbi.nlm.nih.gov]
  • Here we present a 13-year-old boy with pain in the lumbar region radiating bilaterally to the lower limbs, with asymmetrical weakness of lower the limbs, perianal hypoaesthesia and urinary retention.[ncbi.nlm.nih.gov]
  • Patients with demonstrated urinary retention are more likely to have CES. Domen et al. found that 75% of patients with CES had urinary retention of more than 500 mL after voiding.[aliem.com]
Urinary Incontinence
  • None of the patients with complete CES operated in the early stage had urinary incontinence, and also had greater motor recovery. Of the 5 patients with complete CES who underwent delayed surgery, 3 showed residual urinary incontinence.[ncbi.nlm.nih.gov]
  • Our patient underwent emergency neurosurgical spinal decompression, which resolved most symptoms, except for mild urinary incontinence.[ncbi.nlm.nih.gov]
  • Patients may present with back pain, unilateral or bilateral leg pain, paresthesias and weakness, perineum or saddle anesthesia, and rectal and/or urinary incontinence or dysfunction.[doi.org]
  • We present a 62-year-old female patient with lower-back pain, progressive left leg paresis, numbness on the both lower extremities and urinary incontinence. The patient's clinical picture made us suspect the possibility of cauda equina syndrome.[ncbi.nlm.nih.gov]
  • Thirty hours after an uneventful surgery, she complained of weakness in her lower extremities and developed fecal and urinary incontinence.[ncbi.nlm.nih.gov]
Imperforate Hymen
  • We report a unique, cauda equina syndrome-like presentation of hematometrocolpos secondary to imperforate hymen in a 13-year old, previously healthy girl with primary amenorrhea.[ncbi.nlm.nih.gov]

Workup

The diagnosis of cauda equina syndrome is usually made by history and physical findings [7]. Patients who present with bowel or bladder disturbances, leg weakness, or rectal and genital sensory deficits should be evaluated [7] [16]. Radiologic and laboratory studies help to confirm the diagnosis and identify the pathology and underlying cause [1] [13]. Magnetic resonance imaging is the most effective means of definitive diagnosis [7] [13]. Urgent magnetic resonance imaging should be obtained in all patients with lumbar back pain and new onset urinary symptoms [7] [17]. However, magnetic resonance imaging may be normal in as many as 43% of patients with suspected cauda equina syndrome [7]. Magnetic resonance imaging with gadolinium contrast has improved results [7]. 

Other examinations include [4] [7]:

  • Myelography
  • Computed tomography 
  • Bone scans can detect malignant tumors and inflammation of the vertebrae

Laboratory studies may include [1] [3]:

  • Basic blood tests, chemistries, fasting blood sugar
  • Erythrocyte sedimentation rate
  • Renal function
  • Syphilis and Lyme serology
  • Cerebrospinal fluid examination

Other procedures

  • Needle electromyography can show acute denervation and predict outcome and monitor recovery [1] [7]. 
  • Somatosensory evoked potentials can be used to rule out multiple sclerosis [1]. 
  • Inflammatory or infective diseases of the meninges or spinal cord are ruled out using lumbar puncture [1] [14].

Treatment

No proven medical treatment exists for the treatment of cauda equina syndrome. Therapy needs to be individualized and directed toward the underlying cause of the syndrome [11]. Since compression of the lower spinal column is the primary cause of cauda equina syndrome, surgical decompression is the primary treatment for the condition [17]. The major factors effecting operative outcome include [10] [17]:

  • The severity of the sphincter dysfunction
  • The extent of sensory loss
  • Early diagnosis and surgical intervention

Early diagnosis, evaluation by a neurosurgeon, and surgical decompression should be instituted before the neurological damage becomes permanent [4] [10]. The onset of bladder paralysis is the most important indication for immediate surgery [3]. Laminectomy and discectomy are the recommended treatment and should be an emergency intervention [8] [17]. Posterior decompression and stabilization offers the best neurological outcomes [11]. Surgery is recommended within 48 hours, but newer studies failed to see a correlation between the timing of surgery and the outcomes [3] [8] [15] [17].

Medications

Treatment with pharmaceuticals may include [11]:

  • Antibiotics to treat cauda equina syndrome due to infections
  • Antiinflammatories, steroids and non-steroidal anti-inflammatory drugs (NSAIDs) to reduce or prevent swelling
  • Chemotherapy for the treatment of neoplastic tumors

Other therapy

Rehabilitation post-surgery should include physical and occupational therapy. The goal is a return to independent activities of daily living and the maintenance of and improvement of strength and endurance [9] [10]. Prevention and treatment of possible complications, especially deep venous thrombosis, bladder and bowel problems, and decubitus ulcers, should also be part of any treatment plan.

Prognosis

Patients who have had cauda equina syndrome often do not return to a normal status [12]. Prognosis for recovery is improved when the cause of the disorder is identified early and appropriate treatment instituted [9]. Residual weakness, incontinence, impotence, and/or sensory abnormalities are potential problems if therapy is delayed [1] [6].

Patients with bladder incontinence or retention regain continence post-surgery in over 90% of cases [5] [8]. Outcomes postoperatively are worse in patients with bowel dysfunction [5] [10]. Morbidity and mortality rates depend on the underlying etiology [1] [8]. Increased age is also associated with poorer postoperative outcomes [6].

Etiology

Cauda equina syndrome is caused by a narrowing of the spinal canal that results in compression of the nerve roots below the spinal cord, the cauda equina [3]. The cauda equina provides sensory innervation to the saddle area, motor innervation to the urinary and anal sphincters, parasympathetic innervation to the bladder and lower bowel, and sensation and motor control to the lower extremities [3] [5].

The most common causes of caudal equina syndrome include acute disc herniation or traumatic injury at the terminal portion of the spinal cord [2] [7], tumors (malign or benign), or infection [7]. Herniated lower lumbar or sacral discs are the most common cause [1] [4] [7]. Cauda equina syndrome is an emergency [1] requiring immediate diagnosis (usually with magnetic resonance imaging) and surgical intervention [5]. The onset of symptoms in cauda equina syndrome may be acute or may have a gradual onset [7]. The gradual onset has a better prognosis. The outcome of the disorder depends on how quickly it is diagnosed and treated [8].

Individuals with congenital developmental abnormalities or degenerative conditions, such as ankylosing spondylitis, spondylosis, and spondylolisthesis, are more likely to develop cauda equina syndrome, because narrowing already exists [9].  Complications of the condition may include bowel and bladder dysfunction, lower extremity sensory loss, and lower extremity paralysis [1] [10].

Common causes of cauda equina syndrome include [3] [2] [8] [11]:

  • Disc herniation
  • Intradural disc rupture
  • Spinal stenosis
  • Traumatic injury, fracture or subluxation
  • Primary and metastatic tumors
  • Infections
  • Hemorrhage or emboli
  • Iatrogenic injury resulting from complications of spinal surgery or spinal anesthesia

Other, rare causes include the following [3] [8]:

Epidemiology

Cauda equina syndrome is rare [4] [7]. The incidence is variable and depends on the etiology of the syndrome [7]. Mean age at onset of cauda equina syndrome is 41 years of age (24-67 years) but it can occur at any age [12]. Cauda equina syndrome due to traumatic injury is not age specific. Non-traumatic forms of cauda equina Syndrome occur primarily in adults [12]. Cauda equina syndrome, secondary to disk herniation, is most common in men 40 -to50 years of age [6]. The incidence is not affected by gender or race. Males and females are equally affected [7] [12].

Sex distribution
Age distribution

Pathophysiology

Cauda equina syndrome results from injury to the lumbosacral nerve roots at the end of the spinal cord below the tip of the conus medullaris [1]. The spinal cord connects the peripheral and spinal nerves to the brain. The cauda equina provides the connection of the spinal cord to the lower peripheral nervous system [1]. It is a purely lower motor neuron injury [11].

Compression of the nerve fibers results in increased vascular permeability and subsequent diffusion of cerebral spinal fluid. This causes edema which accentuates the mechanical compression [1] [11]. The symptoms are due to compression, displacement, entrapment, or trauma to the cauda equina [9]. The level of spinal injury and the initial cause of compression affect the symptomatology and outcome [11]. Symptoms depend on which roots of the cauda equina are involved [9].

Tethered spinal cord is a cause of cauda equina syndrome seen more in children, but also occurs in adults [13]. This is due to a congenital abnormality, a tight, thickened, shortened filum terminale, intradural scar formation, or other lesion, that leads to fixation of the lower spinal cord [11] [13]. Movement of the spinal cord is impaired and leads to chronic local ischemia, swelling, and compression [1] [13].

Compression of the cauda equina can result in decreased sensation to the saddle area, paralysis of bowel and bladder sensation and function, sensory loss and motor dysfunction of the lower extremities [1] [10] [13].

Differential diagnoses include [9] [11] [13]:

Prevention

Prevention of lower spinal injury is the only way to prevent the syndrome. Prevention of cauda equina syndrome depends upon the early identification of lower spine defects that may result in compression of the nerve roots. Immediate intervention to relieve the compression improves the progression and the occurrence of complications [10].

Summary

Cauda equina syndrome is a rare disorder characterized by a group of symptoms resulting from compression of the nerve roots of the cauda equina [1] [2]. The cauda equina, Latin for ‘horse's tail’, is a collection of nerve roots located at the end of the spinal cord at the first and second lumbar vertebrae [1] [3]. The symptoms of the disorder are due to damage to nerve fibers due to compression and ischemia. Onset may be sudden or gradual. Symptoms include low back pain, sciatica, saddle sensory loss, bladder and bowel dysfunction, and lower extremity motor dysfunction [1] [3] [4]. Damage may be irreversible [1].

Cauda equina syndrome is considered a surgical emergency [1] and surgical intervention to relieve nerve compression is the most effective treatment [1] [5]. The causes of the condition are varied, but the most common causes are a displaced herniated disc or traumatic injury to the lumbar or sacral vertebrae [3] [5] [6].

Patient Information

What is cauda equina syndrome?

Cauda equina syndrome is a rare group of symptoms resulting from compression of the nerve roots located at the end of the spinal cord at the level of the first and second lumbar vertebrae. It results from injury to the spinal cord or vertebrae due to a herniated disk or trauma.

What are the symptoms of cauda equina syndrome?

Symptoms include low back pain, sciatica, saddle sensory loss, bladder and bowel dysfunction, and lower extremity motor disability. Onset may be sudden or gradual. Damage may be irreversible.

What causes cauda equina syndrome?

The symptoms of cauda equina are caused by damage to nerve fibers from compression of the nerve roots. Causes of cauda equina syndrome include:

  • Disc herniation
  • Spinal stenosis 
  • Traumatic injury, fracture or subluxation
  • Primary and metastatic tumors 
  • Infections 
  • Hemorrhage or emboli
  • Congenital anomalies of the spine
  • Iatrogenic injury resulting from complications of spinal surgery or spinal anesthesia

Herniated disk is most common cause. Cauda equina syndrome due to traumatic injury is not age specific. Non-traumatic forms occur primarily in adults.

Who gets cauda equina syndrome?

Cauda equina syndrome is rare. Mean age at onset is 41 years of age (24-67 years) but can occur at any age. The incidence of cauda equina syndrome is not affected by gender or race. Males and females are equally affected.

How is it diagnosed?

Cauda equina syndrome is diagnosed by its presentation, group of symptoms, history of onset and/or injury. Definitive diagnosis is made by magnetic resonance imaging.

How is cauda equina syndrome treated?

The only effective treatment of cauda equina is surgery to decompress the affected nerve fibers. It is considered a surgical emergency and the sooner the surgery is performed the better the prognosis for recovery.

What are the complications?

Complications of cauda e equina include:

How can it be prevented?

Prevention of lower spinal injury is the only way to prevent the syndrome. Prevention of complications depends upon the early identification of the syndrome and immediate surgery to relieve the compression.

References

Article

  1. Mauffrey C, Randhawa K, Lewis C, Brewster M, Dabke H. Cauda Equina Syndrome: an anatomically driven review. Br J Hosp Med. 2008;69(6):344-7. 
  2. Harrop JS, Hunt GE Jr, Vaccaro AR. Conus medullaris and Cauda Equina Syndrome as a result of traumatic injuries: management principles. Neurosurg Focus. 2004;16(6):e4.
  3. Dinning TA, Schaeffer HR. Discogenic compression of the cauda equina: a surgical emergency. Aust N Z J Surg. 1993;63(12):927-34.
  4. Ma B, Wu H, Jia LS, Yuan W, Shi GD, Shi JG. Cauda Equina Syndrome: a review of clinical progress. Chin Med J 2009;122(10):1214-22.
  5. Shaw A, Anwar H, Targett J, Lafferty K. Cauda Equina Syndrome versus saddle embolism. Ann R Coll Surg Engl. 2008;90(6):W6-8.
  6. Ahn UM, Ahn NU, Buchowski JM, Garrett ES, Sieber AN, Kostuik JP. Cauda Equina Syndrome secondary to lumbar disc herniation: a meta-analysis of surgical outcomes. Spine . 2000;25(12):1515-22.
  7. Bell DA, Collie D, Statham PF. Cauda Equina Syndrome: what is the correlation between clinical assessment and MRI scanning?. Br J Neurosurg. 2007;21(2):201-3.
  8. Olivero WC, Wang H, Hanigan WC, Henderson JP, Tracy PT, Elwood PW, Lister JR, Lyle L. Cauda Equina Syndrome (CES) from lumbar disc herniations. J Spinal Disord Tech. 2009;22(3):202-6.
  9. Indrieri RJ. Lumbosacral stenosis and injury of the cauda equina. Vet Clin North Am. 1988;18(3):697-710.
  10. O'Laoire SA, Crockard HA, Thomas DG. Prognosis for sphincter recovery after operation for cauda equina compression owing to lumbar disc prolapse. Br Med J (Clin Res). 1981;282(6279):1852-4.
  11. Kingwell SP, Curt A, Dvorak MF. Factors affecting neurological outcome in traumatic conus medullaris and cauda equina injuries. Neurosurgical Focus. 2008;25(5):E7.
  12. McCarthy MJ, Aylott CE, Grevitt MP, Hegarty J. Cauda Equina Syndrome: factors affecting long-term functional and sphincteric outcome. Spine. 2007;32(2):207-16. 
  13. Kothbauer K, Seiler RW. Tethered spinal cord syndrome in aTethered spinal cord syndrome in adults. Nervenarzt. 1997; 68(4):285-91.
  14. Fujisawa H, Igarashi S, Koyama T. Acute Cauda Equina Syndrome secondary to lumbar disc herniation mimicking pure conus medullaris syndrome--case report. Neurol Med Chir. 1998;38(7):429-31.
  15. Raj D, Coleman N. Cauda Equina Syndrome secondary to lumbar disc herniation. Acta Orthop Belg. 2008;74(4):522-7.
  16. Haldeman S, Rubinstein SM. Cauda Equina Syndrome in patients undergoing manipulation of the lumbar spine. Spine. 1992;17(12):1469-73.
  17. Hussain SA, Gullan RW, Chitnavis BP. Cauda Equina Syndrome: outcome and implications for management. Br J Neurosurg. 2003;17(2):164-7.

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Last updated: 2018-06-22 01:31