Cauda equina syndrome

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Cauda equina syndrome
Cauda equina and filum terminale seen from behind.
ICD-10 G83.4
ICD-9 344.6
DiseasesDB 31115
MeSH C10.668.829.800.750.700

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Joanna Ekabua, M.D. [2]

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Overview

The cauda equina is a collection of nerves at the end of the spinal cord. These nervesconsist of the spinal nerves L2-L5, S1-S5 and the coccygeal nerve. Cauda equina syndrome first described by Hutchinson in 1889 is due to compression of the cauda equina in the lumbosacral region of the spinal canal. It is an emergency medical condition requiring acute intervention in the form of acute decompression surgery to prevent permanent neurological damage to the urinary bladder, Intestine, sex organs and lower limbs. The most common cause of cauda equina syndrome is lumbar disc herniation. It can be classified into two major groups, cauda equina syndrome complete with urinary retention and cauda equina syndrome incomplete. Prognosis of cauda equina syndrome depends on time from onset of symptoms to decompression and the degree of nerve damage at the time of surgery.

Historical perspective

  • Cauda equina was named by the French anatomist Andreas Lazarius (André du Laurens) in the 17th century after its resemblance to a horse's tail (Latin: cauda equina).
  • Cauda equina syndrome was first discovered by Jonathan Hutchinson, a British dermatologist and surgeon in 1889, following a hemorrhoidectomy in a 42-year-old man in which general anesthesia of ether and a crushing clamp was used. Postop, the patient had painless urinary retention and constipation. During catheterization, he felt no pain, by postop day 3, he was fecal incontinent without knowledge. The patient was seen by Hutchinson 6 months later, where examination showed the anus to be patulous and acontractile. An enema or manual evacuation had to be used to empty bowel. The patient was unaware of the passage of feces. When patient self-catheterized three times a day, he had no sensation on catheter passage. He, however, could empty his bladder by straining. The patient had partial anesthesia around the anus and buttocks. He had no problems with his bladder or bowels before to the operation, but he did have a past medical history of alternating sciatica bilaterally which, was not very common. During the sciatica attacks, he felt numb on the buttocks. There is no record of the state of the muscles of his lower limbs. Hutchinson diagnosed a form of ascending neuritis induced by crushing of his pile. He was unhappy with these findings since there was no interval between the operation and the development of the urinary retention. Hutchinson could not establish a diagnosis. Evidence is presented to suggest that this was the first case of disc prolapsed, causing a cauda equina syndrome because of anesthesia and manipulation.[1]
  • In 1977/1978, MRI was developed by Raymond Damadian to diagnose cancer. It has since been used to diagnose other pathologies and is the gold standard for the diagnosis of cauda equina syndrome.[2][3][4]

Classification

Cauda equina syndrome may be classified into complete and incomplete.[5][3]

  • Cauda equina syndrome complete with urinary retention
  • Cauda equina syndrome incomplete
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cauda equina syndrome
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Complete with urinary retention
 
 
 
 
 
 
 
 
 
 
 
Incomplete
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lumbar +/- leg pain, sensory and motor deficency in lower extremities, painless urine retention with overflow incontinence, total perianal sensory loss, and fecal incontinece.
 
 
 
 
 
 
 
 
 
 
 
Lumbar +/- leg pain, sensory and motor deficency in lower extremities, loss of micturition reflex, altered urinary sensation and hesitancy, partial saddle anesthesia, and decreased anal sphinter tone.
 
 
 
 
 
 
 
 
 
 

Pathophysiology

The exact pathogenesis of cauda equina syndrome is not fully understood but may be related to direct mechanical compression of the lumbar and sacral nerves roots arising below the conus medullaris and venous congestion or ischemia.[3][6] The proximal region of the cauda equina is relatively hypovascular leading to neuroischemic symptoms with compression. [6][7]

Causes

Cauda equina syndrome may be caused by[2]

Differentiating cauda equina syndrome from other Diseases

Cauda equina syndrome must be differentiated from spinal disc herniation, epidural hematoma, spinal tumor/metastasis, spinal stenosis, and diabetic amyotrophy.[17][4][18][19]

Epidemiology and Demographics

  • The incidence of cauda equina syndrome is 2 per 100,000 /year.[5][4]
  • Patients of all age groups may develop cauda equina syndrome.
  • Cauda equina syndrome usually affects individuals of all races, although African American individuals are less likely to develop cauda equina syndrome.[20][21][22]
  • Cauda equina syndrome affects men and women equally.

Risk Factors

The most potent risk factor in the development of cauda equina syndrome is spinal disc herniation.[23] Other risk factors include trauma, a spinal tumor, severe infection, spinal stenosis, spinal anesthesia,[24] obesity[25] and female sex.[26]

Screening

There is insufficient evidence to recommend routine screening for cauda equina syndrome.

Natural History, Complications, and Prognosis

If left untreated, 100% progress to permanent nerve damage and neurological deficit.

Common complications of cauda equina syndrome include[4][27]

  • micturition dysfunction 48%
  • defecation dysfunction 42%,
  • sexual dysfunction 53%
  • sciatica 48%
  • altered sensation of the saddle area 57%.

Prognosis of cauda equina syndrome depends on a number of factors, example time from onset of symptoms to decompression, the degree of nerve damage at the time of surgery and the type of cauda equina syndrome; with incomplete being more favourable.[28] Following surgery, the extent of recovery is variable.[4] Long term outcomes postsurgery are bladder, sexual, and motor dysfunction especially in patients with cauda equina syndrome complete with urinary retention.[29]

Diagnosis

Diagnostic Study of Choice

There are no established criteria for the diagnosis of cauda equina syndrome. However, in the presence of Lower back pain, urinary retention, decreased muscle strength, and saddle anesthesia, MRI should be ordered to rule out cauda equina syndrome.

Radiological imaging Sensitivity specificity PPV NPV
CT[30] 98% 86% 72% 99%
MRI[31] 68% 78% 84% 58%

Although CT is shown to be more sensitive and specific for the diagnosis of cauda equina syndrome, MRI is considered the goal standard.[2][3][4]

History and Symptoms

The most common symptoms of cauda equina syndrome include

Physical Examination

Common physical examination findings of cauda equina syndrome include

Laboratory findinds

There are no diagnostic laboratory findings associated with cauda equina syndrome.

Echocardiography

There are no ECG findings associated with cauda equina syndrome.

X-ray

There are no x-ray findings associated with cauda equina syndrome.

Ultrasound

There are no ultrasound findings associated with cauda equina syndrome.

CT Scan

Lumbosacral CT scan may be helpful in the diagnosis of cauda equina syndrome.

CT scan sensitivity for cauda equina syndrome has been estimated to be 98%; specificity, 86%; positive predictive value, 72%; and negative predictive value, 99%.[30]

Findings on CT scan suggestive of/diagnostic of cauda equina syndrome include

Sagittal view CT demonstrates spinal cord compression due to Vertebra fracture after fall from a height (yellow arrow). Case courtesy of Dr Ian Bickle (Picture courtesy: Radiopedia)



MRI

Lumbosacral MRI is the gold standard in the diagnosis of cauda equina syndrome.[2][3][4]

MRI sensitivity for cauda equina syndrome has been estimated to be 68%, specificity 78%, positive predictive value 84% and negative predictive value 58%.[32]

Findings on MRI suggestive of/diagnostic of cauda equina syndrome include

T2-weighted images in non-contrast MRI of the lumbar region at L4/5 level demonstrating a huge isointense lesion (herniated disc) compressing the spinal cord. Sagittal view (Left/yellow arrow) and axial view (Right/yellow arrowhead) (Picture courtesy: Medicine)


Sagittal view MRI of the spine demonstrating a lumbar vertebra destruction due to TB and spinal cord compression (yellow arrowhead). Case courtesy of Dr Rishi Ramaesh (Picture courtesy: Radiopedia)



Other Imaging Findings

There are no other imaging findings associated with cauda equina syndrome.

Other Diagnostic Findings

  • Myelogram
  • Electromyography
  • Pre and post-void bladder scan; if the post-void residual volume is >200ml, the probability of cauda equina syndrome is 43% (P < 0.000003) making bladder scan an adjunct in the diagnosis of cauda equina syndrome.[33]

Treatment

Medical Treatment

Cauda equina syndrome is a medical emergency and requires prompt treatment. Although the mainstay of treatment is surgery, The following medications are used.[9]

  • 5.4 mg/kg·h of methylprednisolone (intravenous) for 2 days
  • 5 mg of dexamethasone (intravenous) every 12 h for 3 days
  • 0.5 mg of mecobalamin tablets (oral) every 8 h
  • Chemotherapy for cases due to tumors
  • Antibiotics for cases due to infection

Surgery

Surgery is the mainstay of treatment for cauda equina syndrome.[3][2] Immediate surgical decompression is the best intervention associated with positive patient outcome.[34] Procedures used include

Primary Prevention

There are no established measures for the primary prevention of cauda equina syndrome.

Secondary Prevention

There are no established measures for the secondary prevention of cauda equina syndrome.

References

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  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 Luo D, Ji C, Xu H, Feng H, Zhang H, Li K (2020). "Intradural disc herniation at L4/5 level causing Cauda equina syndrome: A case report". Medicine (Baltimore). 99 (7): e19025. doi:10.1097/MD.0000000000019025. PMC 7035013 Check |pmc= value (help). PMID 32049799 Check |pmid= value (help).
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 Srikandarajah N, Wilby M, Clark S, Noble A, Williamson P, Marson T (2018). "Outcomes Reported After Surgery for Cauda Equina Syndrome: A Systematic Literature Review". Spine (Phila Pa 1976). 43 (17): E1005–E1013. doi:10.1097/BRS.0000000000002605. PMC 6104724. PMID 29432394.
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  11. 11.0 11.1 11.2 11.3 11.4 11.5 Harrop JS, Hunt GE, Vaccaro AR (2004). "Conus medullaris and cauda equina syndrome as a result of traumatic injuries: management principles". Neurosurg Focus. 16 (6): e4. doi:10.3171/foc.2004.16.6.4. PMID 15202874.
  12. Wu HY, Xu WB, Lu LW, Li HH, Tian JS, Li JM; et al. (2018). "Imaging features of spinal atypical teratoid rhabdoid tumors in children". Medicine (Baltimore). 97 (52): e13808. doi:10.1097/MD.0000000000013808. PMC 6314652. PMID 30593171.
  13. Tello Díaz C, Allegue Allegue N, Gil Sala D, Gonçalves Martins G, Boqué Torremorell M, Bellmunt Montoya S (2019). "Cauda Equina Syndrome Caused by Epidural Venous Plexus Engorgement in a Patient with May-Thurner Syndrome". Ann Vasc Surg. 60: 480.e7–480.e11. doi:10.1016/j.avsg.2019.04.002. PMID 31200048.
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  16. Cohen DB (2004). "Infectious origins of cauda equina syndrome". Neurosurg Focus. 16 (6): e2. doi:10.3171/foc.2004.16.6.2. PMID 15202872.
  17. Panos G, Watson DC, Karydis I, Velissaris D, Andreou M, Karamouzos V; et al. (2016). "Differential diagnosis and treatment of acute cauda equina syndrome in the human immunodeficiency virus positive patient: a case report and review of the literature". J Med Case Rep. 10: 165. doi:10.1186/s13256-016-0902-y. PMC 4895963. PMID 27268102.
  18. Jiménez-Ávila JM, Castañeda-Huerta JE, González-Cisneros AC (2019). "[Bruns Garland syndrome. Report of a case and differential diagnosis with cauda equina syndrome]". Acta Ortop Mex. 33 (1): 42–45. PMID 31480126.
  19. Zhou ZN, Canon C, Matrai C, Chapman-Davis E (2018). "Cauda equina syndrome secondary to leptomeningeal metastases from recurrent primary peritoneal carcinoma". Ecancermedicalscience. 12: 814. doi:10.3332/ecancer.2018.814. PMC 5834310. PMID 29515655.
  20. Schoenfeld AJ, Bader JO (2012). "Cauda equina syndrome: an analysis of incidence rates and risk factors among a closed North American military population". Clin Neurol Neurosurg. 114 (7): 947–50. doi:10.1016/j.clineuro.2012.02.012. PMID 22402198.
  21. Radcliff KE, Kepler CK, Delasotta LA, Rihn JA, Harrop JS, Hilibrand AS; et al. (2011). "Current management review of thoracolumbar cord syndromes". Spine J. 11 (9): 884–92. doi:10.1016/j.spinee.2011.07.022. PMID 21889419.
  22. Small SA, Perron AD, Brady WJ (2005). "Orthopedic pitfalls: cauda equina syndrome". Am J Emerg Med. 23 (2): 159–63. doi:10.1016/j.ajem.2004.03.006. PMID 15765336.
  23. Kapetanakis S, Chaniotakis C, Kazakos C, Papathanasiou JV (2017). "Cauda Equina Syndrome Due to Lumbar Disc Herniation: a Review of Literature". Folia Med (Plovdiv). 59 (4): 377–386. doi:10.1515/folmed-2017-0038. PMID 29341941.
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  25. Cushnie D, Urquhart JC, Gurr KR, Siddiqi F, Bailey CS (2018). "Obesity and spinal epidural lipomatosis in cauda equina syndrome". Spine J. 18 (3): 407–413. doi:10.1016/j.spinee.2017.07.177. PMID 28756300.
  26. 26.0 26.1 26.2 26.3 26.4 26.5 26.6 Long B, Koyfman A, Gottlieb M (2020). "Evaluation and management of cauda equina syndrome in the emergency department". Am J Emerg Med. 38 (1): 143–148. doi:10.1016/j.ajem.2019.158402. PMID 31471075.
  27. Korse NS, Pijpers JA, van Zwet E, Elzevier HW, Vleggeert-Lankamp CLA (2017). "Cauda Equina Syndrome: presentation, outcome, and predictors with focus on micturition, defecation, and sexual dysfunction". Eur Spine J. 26 (3): 894–904. doi:10.1007/s00586-017-4943-8. PMID 28102451.
  28. Gardner A, Gardner E, Morley T (2011). "Cauda equina syndrome: a review of the current clinical and medico-legal position". Eur Spine J. 20 (5): 690–7. doi:10.1007/s00586-010-1668-3. PMC 3082683. PMID 21193933.
  29. Hazelwood JE, Hoeritzauer I, Pronin S, Demetriades AK (2019). "An assessment of patient-reported long-term outcomes following surgery for cauda equina syndrome". Acta Neurochir (Wien). 161 (9): 1887–1894. doi:10.1007/s00701-019-03973-7. PMC 6704093 Check |pmc= value (help). PMID 31263950.
  30. 30.0 30.1 Peacock, J.G.; Timpone, V.M. (2017). "Doing More with Less: Diagnostic Accuracy of CT in Suspected Cauda Equina Syndrome". American Journal of Neuroradiology. 38 (2): 391–397. doi:10.3174/ajnr.A4974. ISSN 0195-6108.
  31. . doi:10.1302/0301-620X. Missing or empty |title= (help)
  32. . doi:10.1302/0301-620X. Missing or empty |title= (help)
  33. Venkatesan M, Nasto L, Tsegaye M, Grevitt M (2019). "Bladder Scans and Postvoid Residual Volume Measurement Improve Diagnostic Accuracy of Cauda Equina Syndrome". Spine (Phila Pa 1976). 44 (18): 1303–1308. doi:10.1097/BRS.0000000000003152. PMID 31479434.
  34. Hogan WB, Kuris EO, Durand WM, Eltorai AEM, Daniels AH (2019). "Timing of Surgical Decompression for Cauda Equina Syndrome". World Neurosurg. 132: e732–e738. doi:10.1016/j.wneu.2019.08.030. PMID 31415897.

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