Kasuistiken Z Rheumatol 2015 · 74:75–77 DOI 10.1007/s00393-014-1507-8 Published online: 16. Oktober 2014 © Springer-Verlag Berlin Heidelberg 2014

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E. Reinhold-Keller, Hamburg F. Moosig, Lübeck/Bad Bramstedt

Sacral perineural cysts (Tarlov cysts) are multiple cystic lesions of the nerve root sheet, containing cerebrospinal fluid, most commonly arising in the lower spine. The first report of a sacral perineural cyst was made by Tarlov in 1938, as an incidental finding at autopsy [1]. The prevalence in the adult population is 1–5% [2, 3]. Tarlov cysts are typically located at the junction of the dorsal ganglion and posterior nerve root and usually develop between the endoneurium and perineurium of the sensory nerve root under high hydrostatic pressure [4]. In most cases perineural cysts are small, asymptomatic, and are found incidentally on magnetic resonance imaging (MRI) studies done for other reasons. Large and symptomatic cysts are rare [2]. Depending on their localization, size, and relationship to the nerve roots, sacral perineural cysts may cause sensory disturbances, motor deficits, or bowel/bladder dysfunction [5]. Symptoms may include sharp, burning pain in the hip and down the back of the thigh, possibly with weakness and reduced sensation along the affected leg. Tarlov cysts sometimes enlarge enough to cause erosions of the surrounding bone, which is another way they may cause back pain. We report the case of a symptomatic sacral perineural cyst, to increase the awareness of this rare entity in the rheumatology community.

Case report In August 2013, a 46-year-old female patient was referred to the author’s hospi-

P. Ostojic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade

Sacral perineural cyst mimicking inflammatory low back pain tal because of low back pain radiating into both groins, which had persisted for 2 years. The pain worsened during the night and morning, and was alleviated during daily activities. Morning pain was associated with low back stiffness lasting longer than 2 h. Sometimes the patient felt pain and numbness along the left S1 dermatome. No symptoms or signs of bowel or bladder incontinence were present. Abdominal ultrasound examination revealed a small left-sided kidney stone with several parapyelic cysts. She was treated with different nonsteroidal anti-inflammatory drugs, paracetamol, and myorelaxants, but only with partial pain relief. According to her medical history, in November 1991 she was surgically treated because of benign intracranial meningioma. Since

2000 she had bronchial asthma. Physical examination revealed mild weakness of the plantar flexion of the left foot. Movement of the lumbar spine was painful and somewhat limited. Lasegue’s sign was negative. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated (35 mm/h and 9.4, respectively) and Mennel’s sign was present on both sides, indicating possible inflammation of the sacroiliac joints. However, radiographs of the lumbosacral spine and sacroiliac joints were normal. Magnetic resonance imaging (MRI) was performed and revealed a large spinal meningeal cyst in the sacrum (60×37×22 mm) consisting of multiple perineural cysts (. Fig. 1). The cyst eroded the surrounding sacral bone structures, narrowed several sacral foram-

Fig. 1 8 Sagittal (a) and axial (b) T2-weighted image of a large spinal meningeal cyst in the sacrum (60×37×22 mm) consisting of multiple perineural cysts. The cyst erodes surrounding sacral bone structures, narrows several sacral foramina, and compresses neighboring nerve fibers (radix S1, S2, and S3 right side, and S1, S2, S3, and S4 left side) Zeitschrift für Rheumatologie 1 · 2015 

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Abstract · Zusammenfassung ina, and compressed neighboring nerve fibers (radix S1, S2 and S3 right side, and S1, S2, S3 and S4 left side). No signs of fractures were present, and the sacroiliac and hip joints were normal. She was referred to a neurosurgeon, who indicated surgical treatment. Laminectomy of S1, S2, and S3 was performed using the standard posterior approach. The cyst was excised after ligation of its neck. The nerve root was kept intact, only the covering sheet (containing nerve fibers) was excised. Postoperatively, the patient had dramatic improvement of pain.

Discussion According to the new criteria [6] published in 2009, relevant signs of inflammatory low back pain include: age at onset of back pain younger than 40 years, improvement with exercise, night pain, insidious onset, and no improvement with rest. If at least four of these five parameters are fulfilled, the criteria have a sensitivity of 79.6% and specificity of 72.4%. A limited specificity of around 75–80% was seen also in earlier criteria [7, 8]. Therefore, the diagnosis of inflammatory back pain has proven to be a challenge, as symptoms may be similar to other causes of low back pain. This case describes a woman with a multilevel symptomatic perineural cyst in the sacrum mimicking inflammatory low back pain. Although the etiology of perineural cysts is still unclear, it is believed that communication between the subarachnoid space at the dural sleeve and the nerve root may function as a valve. Cerebrospinal fluid influx is presumably forced during transient pressure increases and blocked from returning by this oneway valve or other obstructions [5]. Cysts with free subarachnoid communication are usually asymptomatic [9]. Tarlov cysts are visible by conventional MRI, appearing as low-intensity intraspinal masses on T1-weighted and high-intensity on T2weighted images, while plain X-ray may detect only associated sacral erosions or fractures [10]. Most sacral perineural cysts are asymptomatic, but sometimes, like in this case, patients present with pain and significant neurological deficits. Generally, three sep-

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Zeitschrift für Rheumatologie 1 · 2015

Z Rheumatol 2015 · 74:75–77  DOI 10.1007/s00393-014-1507-8 © Springer-Verlag Berlin Heidelberg 2014 P. Ostojic

Sacral perineural cyst mimicking inflammatory low back pain Abstract This case describes a 46-year-old woman with local pelvic and perineal pain, persisting for 2 years at presentation. The pain worsened during the night and morning and was alleviated during daily activities. Low back pain was associated with morning stiffness lasting longer than 2 h. Sometimes, she felt pain and numbness along her left S1 dermatome, without overt bladder or bowel incontinence. Lasegue’s sign was negative. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated (35 mm/h and 9.4, respectively) and Mennel’s sign was present on both sides, indicating possible inflammation of the sacroiliac

joints. However, radiographs of the lumbosacral spine and sacroiliac joints were normal. Magnetic resonance imaging (MRI) revealed a large spinal meningeal cyst in the sacrum (60×37×22 mm) consisting of multiple perineural cysts. The cyst eroded the surrounding sacral bone structures, narrowed several sacral foramina, and compressed neighboring nerve fibers. MRI findings on sacroiliac and hip joints were normal. Keywords Tarlov cyst · Low back pain · Magnetic resonance imagining · Perineural cyst · Case report

Sakrale perineurale Zyste mit Lumbalgie wie bei Entzündung Zusammenfassung In der vorliegenden Kasuistik geht es um eine 46 Jahre alte Frau mit lokalisierten Schmerzen im Becken- und Dammbereich, die bei Vorstellung seit 2 Jahren bestanden. Nachts und morgens nahmen die Schmerzen zu und verringerten sich am Tag. Die Lumbalgie ging mit Morgensteifigkeit für mehr als 2 h einher. Manchmal empfand die Patientin Schmerzen und ein Taubheitsgefühl im linken Dermatom S1, aber ohne Harn- oder Stuhlinkontinenz. Das Lasègue-Zeichen war negativ. Die Blutkörperchensenkungsgeschwindigkeit (BSG) und C-reaktives Protein (CRP) waren erhöht (35 mm/h bzw. 9,4) und das Mennel-Zeichen beidseits positiv, was auf eine mögliche Entzündung der Sakroiliakalgelenke hinweist. Die Röntgenaufnahmen der Len-

arate clinical syndromes caused by Tarlov cysts have been identified. The most common is characterized by pelvic or perineal pain with sphincter disturbances, perineal and genital sensory loss, and sciatica due to compression of S1 and/or S2 roots. A second group of patients have local, perineal, and pelvic symptoms, but without sciatica. The third, very rare syndrome includes only serious progressive bowel and bladder dysfunction. Our patient had local pelvic and perineal pain, with numbness along the left S1 dermatome and somewhat weakened plantar flexion of the left foot, probably due to S1 radiculopathy, but without overt bladder or bowel in-

denwirbelsäule und der Sakroiliakalgelenke waren jedoch unauffällig. Allerdings zeigte sich in der Magnetresonanztomographie (MRT) eine große spinale meningeale Zyste im Kreuzbein (60×37×22 mm), die aus mehreren perineuralen Zysten bestand. Durch die Zyste kam es zur Erosion der umgebenden Strukturen des Kreuzbeins, mehreren verengten Foramina sacralia und komprimierter benachbarter Nervenfasern. Die MRT-Befunde des Sakroiliakal- und Hüftgelenks waren normal. Schlüsselwörter Tarlov-Zyste · Lumbalgie · Magnetresonanztomographie · Perineurale Zyste · Kasuistik

continence. Increased CRP and ESR levels in our patient could be explained by compression and inflammation of the nerve roots and surrounding sacral bone structures (even swelling over the sacral area of the spine may be found in such patients), or it may be due to other causes unrelated to the cyst. She was treated with several analgesics, mostly with nonsteroidal anti-inflammatory drugs and paracetamol, but only with partial pain relief. Obviously, her pain is neuropathic in nature, and no significant pain relief using classic pain killers is expected. Gabapentin and pregabalin are often used to treat neuropathic pain of different origin, but there

Fachnachrichten is only one case report about gabapentin in the treatment of pain caused by a sacral perineural cyst [11]. Spinal cord stimulation is a well-established treatment for neuropathic pain of spinal origin, but it is not a treatment for neurological deficits, such as weakness [10, 12]. A more invasive treatment option is computed tomography (CT)-guided percutaneous aspiration of the cyst and obliteration by injecting fibrin. The success rate of this intervention is 75% [10]. Surgical treatment is indicated for cysts that cause serious motor dysfunction and pain. Surgical procedures include posterior spinal laminectomy and decompression, cyst fenestration and imbrication, or ligation and resection of the cyst neck [13]. Most published series on surgical treatment of sacral perineural cysts are small, but all reported excellent outcomes [14, 15].

Conclusion Although sacroiliitis and spondylitis are the most common reasons for inflammatory low back pain, the possibility of a sacral perineural cyst should be always considered in patient with persistent symptoms, especially when associated with symptoms or signs of lumbosacral radiculopathy.

Corresponding address P. Ostojic Institute of Rheumatology, School of Medicine, University of Belgrade Resavska 69, 11000 Belgrade Serbia [email protected]

Compliance with ethical guidelines Conflict of interest.  P. Ostojic states that there are no conflicts of interest. The accompanying manuscript does not include studies on humans or animals.

References   1. Tarlov IM (1938) Perineural cyst of the spinal nerve root. Arch Neurol Psychiatry 40:1067–1074   2. Park HJ, Jeon YH, Rho MH et al (2011) Incidental findings of the lumbar spine at MRI during herniated intervertebral disk disease evaluation. Am J Roentgenol 196:1151–1155   3. Paulsen RD, Call GA, Murtagh FR (1994) Prevalence and percutaneous drainage of cysts of the sacral nerve root sheet (Tarlov cysts). Am J Neuroradiol 15:293–299   4. Guo D, Shu K, Chen R et al (2007) Microsurgical treatment of symptomatic sacral perineural cysts. Neurosurgery 60:1059–1066   5. Lucantoni C, Than K, Wang A et al (2011) Tarlov cysts: a controversial lesion of the sacral spine. Neurosurg Focus 31(6):1–6   6. Sieper J, Heijde D van der, Landewe R et al (2009) New criteria for inflammatory back pain in patients with chronic back pain: a real patient exercise by experts from the Assessment of SpondyloArthritis international Society (ASAS). Ann Rheum Dis 68(6):784–788   7. Rudwaleit M, Metter A, Listing J et al (2006) Inflammatory back pain in ankylosing spondylitis: a reassessment of the clinical history for application as classification and diagnostic criteria. Arthritis Rheum 54(2):569–578   8. Calin A, Porta J, Fries JF et al (1977) Clinical history as screening test for ankylosing spondylitis. JAMA 237(24):2613–2614   9. Davis SW, Levy LM, LeBihan DJ et al (1993) Sacral meningeal cysts: evaluation with MR imaging. Radiology 187(2):445–448 10. Hiers RH, Long D, North RB et al (2010) Hiding in plain sight: a case of Tarlov perineural cyst. J Pain 11(9):833–837 11. Megalhaes E, Mecarenhas AM, Kraychete DC et al (2004) Gabapentin to treat sacral perineal cyst— induced pain. Case report. Rev Bras Anestesiol 54(1):73–77 12. North RB, Kidd DH, Farrokhi F et al (2005) Spinal cord stimulation versus repeated lumbosacral spine surgery for chronic pain: a randomized controlled trial. Neurosurgery 56:98–106 13. Sen RK, Goyal T, Tripathy SK et al (2012) Tarlov cysts: a report of two cases. J Orthop Surg 20(1):87–89 14. Caspar W, Papavero L, Nabhan A et al (2003) Microsurgical excision of symptomatic perineurial cysts: a study of 15 cases. Surg Neurol 59:101–106 15. Mummaneni PV, Pitts LH, McCormack BM et al (2000) Microsurgical treatment of symptomatic sacral Tarlov cysts. J Neurosurg 47:74–79

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Zeitschrift für Rheumatologie 1 · 2015 

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Sacral perineural cyst mimicking inflammatory low back pain.

This case describes a 46-year-old woman with local pelvic and perineal pain, persisting for 2 years at presentation. The pain worsened during the nigh...
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