TARLOV CYSTS - Canada the misinformed!!!

The rare diagnosis of Tarlov Cyst Disease poses significant challenges in the realm of medical care. Most Canadian Medical Practitioners lack the expertise to accurately diagnose, treat, or manage this condition. As a result, patients often find themselves navigating a healthcare system ill-prepared to address their needs, leaving them feeling lost, scared, and isolated. This lack of medical understanding perpetuates feelings of misinformation and uncertainty among Canadian patients. Through this blog, I aim to share my journey of navigating the Canadian Healthcare system while living with multiple rare disease diagnoses. I have dedicated myself to learning as much as possible about my rare diseases, hoping that my experiences and hard-earned self-education can support and guide others on their own rare disease journey. Together, let's shed light on the challenges those living with rare diseases face and work towards a more compassionate and informed healthcare system.

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Monday, April 11, 2011

IJO India Journal Of Orthopeadics (click here for link to site)

Excellent information about treatment of symptomatic Tarlov Cysts.

I love it whenever I see countries taking a true, determined interst in their residents health care. This is a perfect example of India taking a lead on rare diagnosis treatment and care.



CASE REPORT Table of Contents
Year : 2007 | Volume : 41 | Issue : 4 | Page : 401-403
Tarlov cyst: Case report and review of literature


Bhagwat Prashad, Anil K Jain, Ish K Dhammi
University College of Medical Sciences and Guru Teg Bahadur Hospital, Shahadara, Delhi, India


Click here for correspondence address and email
Abstract


We describe a case of sacral perineural cyst presenting with complaints of low back pain with neurological claudication. The patient was treated by laminectomy and excision of the cyst. Tarlov cysts (sacral perineural cysts) are nerve root cysts found most commonly in the sacral roots, arising between the covering layer of the perineurium and the endoneurium near the dorsal root ganglion. The incidence of Tarlov cysts is 5% and most of them are asymptomatic, usually detected as incidental findings on MRI. Symptomatic Tarlov cysts are extremely rare, commonly presenting as sacral or lumbar pain syndromes, sciatica or rarely as cauda equina syndrome. Tarlov cysts should be considered in the differential diagnosis of patients presenting with these complaints.


Keywords: Low back pain, sacral perineural cyst, sciatica, tarlov cyst
How to cite this article:
Prashad B, Jain AK, Dhammi IK. Tarlov cyst: Case report and review of literature. Indian J Orthop 2007;41:401-3


How to cite this URL:
Prashad B, Jain AK, Dhammi IK. Tarlov cyst: Case report and review of literature. Indian J Orthop [serial online] 2007 [cited 2011 Apr 12];41:401-3. Available from: http://www.ijoonline.com/text.asp?2007/41/4/401/37007
Tarlov cysts were first described in 1938 as an incidental finding at autopsy. [1] Tarlov described a case of symptomatic perineural cyst and recommended its removal. Since then a few cases have been reported in the literature. [2],[3],[4]


Paulsen reported the incidence of Tarlov cysts as 4.6% in back pain patients (n=500). Only 1% of back pain patients (n=500) were symptomatic. [4] The patient may present as low back pain, sciatica, coccydynia or cauda equina syndrome. The cysts are usually diagnosed on MRI, which reveals the lesion arising from the sacral nerve root near the dorsal root ganglion. [5]


Tarlov advised extensive surgery with sacral laminectomy and excision of the cyst along with the nerve root. [6] Paulsen reported CT-guided percutaneous aspiration of these perineural cysts for relief of sciatica. [4] Recently, microsurgical excision of the cyst has been advocated, combined with duraplasty or plication of the cyst wall. [7]


We report a case of symptomatic Tarlov cyst presenting as back pain, to increase the awareness of this rare entity in the orthopedic community.




Case Report Top




A 29-year-old female presented with right thigh pain off and on for nine months. The pain was not associated with specific time, posture or activity and it used to get relieved by non steroidal antiinflammatory drugs (NSAID). Clinical examination at this stage did not reveal any findings at spine, bilateral hips and left thigh.


For last three months, the intensity and duration of pain had increased, which was now unrelieved by taking NSAID. The pain had progressed to the lower back and bilateral upper thigh up to the ankle. The pain was aggravated by activity and prolonged standing and was more bothersome in the evening. She used to get up in the middle of the night with pain. Later the patient started having rest pain as well. Examination showed no spinal tenderness. Straight leg raising was 50° on the right side and normal on the left side. There was mild blunting of sensations along the S1 and S2 dermatome on the right side, no motor deficit in both lower limbs.


X-ray of the lumbosacral spine did not reveal any abnormality [Figure - 1]. The MRI of the spine revealed fluid-filled cystic lesion, arising from the second sacral nerve root on the right side and measuring 2cm in diameter [Figure - 2].


The patient was taken for sacral laminectomy, excision of the cyst and plication of the cyst wall, while retaining the nerve root [Figure - 3]. Histopathological examination of the cyst wall showed nerve cells, which confirmed the diagnosis of Tarlov cyst.


Patient appreciated relief of pain immediately after the surgery. Postoperative period was uneventful and the patient made prompt recovery. On nine months followup, the patient had no pain in lower limbs and back. The patient is back at her job and is asymptomatic. Postoperative MRI taken at nine months [Figure - 4] did not show any evidence of recurrence of the cyst.




Discussion Top




Tarlov cysts are rare causes of low back pain. They are more common in females. [4],[7] Clinical presentation of Tarlov cysts is variable. The cysts may cause local and/or radicular pain. The dominant syndrome is referable to the caudal nerve roots, either sciatica, sacral or buttocks pain, vaginal or penile paraesthesia or sensory changes over the buttocks, perineal area and lower extremity. Depending on their location, size and relationship to the nerve roots, they may cause sensory disturbances or motor deficits to the point of bladder dysfunction. Tenderness on firm pressure over the sacrum may be present. Commonly, the symptomatology is intermittent at its onset and is most frequently exacerbated by standing, walking and coughing. Bed rest alleviates the discomfort. [8]


Plain X-rays are usually normal. However, they may reveal characteristic bone erosion of the spinal canal or anterior or posterior neural foramina. [9] A CT scan can demonstrate cystic masses isodense with CSF located at the foramina. Bony changes may also be present. [10] An MRI gives a much better soft tissue contrast and is currently the investigation of choice for perineural cysts. The cysts demonstrate low signal on T-1 weighted images and high signal on T-2 weighted images, similar to CSF. [5] Myelography showing the filling of the meningocele sac 1h after injection of contrast medium is highly suggestive of a perineural cyst. [11]


Microscopic features of the cyst were described by Tarlov. The early stage in cyst formation is that of a space between the arachnoid which covers the root or the perineurium and the outer layer of the pial cover of the root or the endoneurium. It usually begins in one portion of the circumference of the perineural space, the larger cysts compressing the nerve root to one side. The cyst occupies the posterior root abutting the proximal portion of the dorsal ganglion. Its main part is bordered by reticulum or by nerve fibers. [1]


The pathogenesis of perineural cysts is uncertain. Tarlov felt that hemorrhage into the subarachnoid space caused accumulations of red cells which impeded the drainage of the veins in the perineurium and epineurium, leading to rupture with subsequent cyst formation. Four out of the seven patients in Tarlov's 1970 article had a history of trauma. [8] Schreiber and Haddad also supported this posttraumatic cause of cyst formation. [12] Because many of the patients with perineural cyst in their series did not have histories of trauma, Fortuna et al . believed that the perineural cysts were congenital, caused by arachnoidal proliferations within the root sleeve. [13]


There is no consensus on a single method of treatment. Various methods have been advocated. Tarlov advised that symptomatic, single perineural cysts should be completely excised together with the posterior root and ganglion from which they arise. [8] Paulsen reported CT-guided percutaneous aspiration of these perineural cysts in two patients for the relief of sciatica caused by compression. [4] According to Caspar microsurgical excision of the cyst combined with duraplasty or plication of the cyst wall is an effective and safe treatment of symptomatic sacral cysts. The parent nerve root is always left intact. [7]


Tarlov cysts are a documented cause of sacral radiculopathy and other radicular pain syndromes. They must be considered in the differential diagnosis of patients presenting with these clinical presentations and appropriately treated by cyst excision.




References Top


1. Tarlov IM. Perineural cysts of the spinal nerve roots. Arch Neural Psychiatry 1938;40:1067-74. Back to cited text no. 1
2. Chaiyabud P, Suwanpratheep K. Symptomatic Tarlov cyst: Report and review. J Med Assoc Thai 2006;89:1047-50. Back to cited text no. 2 [PUBMED]
3. Acosta FL Jr, Quinones-Hinojosa A, Schmidt MH, Weinstein PR. Diagnosis and management of sacral Tarlov cysts. Case report and review of the literature. Neurosurg Focus 2003;15:E15. Back to cited text no. 3
4. Paulsen RD, Call GA, Murtagh FR. Prevalence and percutaneous drainage of cysts of the sacral nerve root sheath (Tarlov cysts). AJNR Am J Neuroradiol 1994;15:293-9. Back to cited text no. 4 [PUBMED]
5. Rodziewicz GS, Kaufman B, Spetzler RF. Diagnosis of sacral perineural cysts by nuclear magnetic resonance. Surg Neurol 1984;22:50-2. Back to cited text no. 5 [PUBMED]
6. Tarlov IM. Cysts (perineurial) of the sacral roots. J Am Med Assoc 1948;138:740-4. Back to cited text no. 6
7. Caspar W, Papavero L, Nabhan A, Loew C, Ahlhelm F. Microsurgical excision of symptomatic sacral perineurial cysts: A study of 15 cases. Surg Neurol 2003;59:101-6. Back to cited text no. 7 [PUBMED] [FULLTEXT]
8. Tarlov IM. Spinal perineurial and meningeal cysts. J Neural Neurosurg Psychiatry 1970;33:833-43. Back to cited text no. 8
9. Taveras JM, Wood EH. Diagnostic neuroradiology. 2 nd ed. Vol 2. Williams and Wilkins: Baltimore; 1976. p.1139-45. Back to cited text no. 9
10. Tabas JH, Deeb ZL. Diagnosis of sacral perineural cysts by computed tomography. J Comput Tomogr 1986;10:255-9. Back to cited text no. 10
11. Nishiura I, Koyama T, Handa J. Intrasacral perineurial cyst. Surg Neurol 1985;23:265-9. Back to cited text no. 11
12. Schreiber F, Haddad B. Lumbar and sacral cysts causing pain. J Neurosurg 1951;8:504-9. Back to cited text no. 12
13. Fortuna A, La Torre E, Ciappetta P. Arachnoid diverticula: A unitary approach to spinal cysts communicating with the subarachnoid space. Acta Neurochir (Wien) 1977;39:259-68. Back to cited text no. 13


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Bhagwat Prashad
K-1/133, First Floor, Chitaranjan Park, New Delhi-110 019
India

Wednesday, April 6, 2011

What is Arachnoiditis?

Arachnoiditis ** Provided by Spine Universe**


Arachnoiditis is a debilitating condition characterized by severe stinging and burning pain and neurologic problems. It is caused by an inflammation of the arachnoid lining—one of the 3 linings that surround the brain and spinal cord. This inflammation causes constant irritation, scarring, and binding of nerve roots and blood vessels. The predominant symptom of arachnoiditis is chronic and persistent pain in the lower back, lower limbs or, in severe cases, throughout the entire body. Other symptoms may include:
  • Tingling, numbness, or weakness in the legs
  • Bizarre sensations such as insects crawling on the skin or water trickling down the leg
  • Severe shooting pain (which some liken to an electric shock sensation)
  • Muscle cramps, spasms, and uncontrollable twitching
  • Bladder, bowel, and/or sexual dysfunction
If the disease progresses, symptoms may become more severe or even permanent. This disorder can be very debilitating, as the pain is constant and intractable. Most people with arachnoiditis are unable to work and have significant disability.


Causes of Arachnoiditis
There are 3 main causes of arachnoiditis:
  • Trauma/surgery-induced
    Arachnoiditis has long been recognized as a rare complication of spinal surgery (particularly after multiple or complex surgeries) or trauma to the spine. Other similar causes include multiple lumbar punctures (especially if there is a "bloody tap" with bleeding into the spinal fluid), advanced spinal stenosis, or chronic degenerative disc disease.
  • Chemically-induced
    In recent years, myelograms have come under scrutiny as being a possible cause of this condition. A myelogram is a diagnostic test in which a radiographic contrast media (dye) is injected into the area surrounding the spinal cord and nerves. This dye is then visible on x-rays, CT, or MRI scans and used by physicians to diagnose spinal conditions. There is now a concern that exposure (especially repeated exposure) to some of the dyes used in myelograms may cause arachnoiditis. Similarly, there is concern that the preservatives found in epidural steroid injections may cause arachnoiditis, especially if the medication accidentally enters the cerebral spinal fluid.
  • Infection-induced
    Arachnoiditis can also be caused by certain infections that affect the spine such as viral and fungal meningitis or tuberculosis.
Treatment
There is no cure for arachnoiditis. Treatment options are geared toward pain relief and are similar to treatments for other chronic pain conditions. Some examples include the following:
  • Pain medications such as NSAIDs, corticosteroids (orally or injected), anti-spasm drugs, anti-convulsants (to help with the burning pain), and in some cases, narcotic pain relievers. Some of these medications may be administered through an intrathecal pump which, when implanted under the skin, can administer medication directly to the spinal cord.
  • Physical therapy such as hydrotherapy, massage, and hot/cold therapy.
  • Transcutaneous Electrical Nerve Stimulation (TENS) is a treatment in which a painless electrical current is sent to specific nerves through electrode patches that are placed on the skin. The mild electrical current generates heat that serves to relieve stiffness, improve mobility, and relieve pain.
  • Spinal cord stimulator is a device that transmits an electrical signal to the spinal cord for pain relief.
Surgery is not recommended for arachnoiditis because it only causes more scar tissue to develop and exposes the already irritated spinal cord to more trauma.


Living with Arachnoiditis
Unfortunately, this condition can cause serious disability. It is never easy to live with chronic pain. Not only does it adversely affect your body, it can also cause mental stress as well. Sufferers of arachnoiditis are encouraged to join support groups or find other therapeutic outlets for stress. Treatment methods should be focused on pain relief and maintaining quality of life. More research is needed about this and other chronic pain conditions so that someday a cure may be found.


Updated on: 12/10/09

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