Published online: 2019-09-26

Case Report

Syringomyelia secondary to cervical spondylosis: Case report and review of literature Savitr Sastri Bhagavathula Venkata1, Arivazhagan Arimappamagan2, Spiros Lafazanos3, Nupur Pruthi2 1Department of , Vydehi Institute of Medical Sciences and Research Centre, 2Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India, 3Department of Neurosurgery, General Military Hospital, Athens, Greece

ABSTRACT secondary to cervical spondylosis is a rare entity to encounter in clinical practice. We discuss the case of a 53-year-old lady who presented with a syringomyelic syndrome and was found to have cervical spondylosis on imaging. Cine-MRI revealed an obstruction of (CSF) flow in the cervical spinal subarachnoid space. Decompression of the same led to clinical and radiological improvement. There is a potential causal association between cervical spondylosis and syringomyelia. MRI CSF flow studies may help in deciding the course of treatment in such cases. A subset of patients with cervical spondylosis and concurrent signal intensity changes may show reversal of the same following intervention. Key words: Cervical spondylosis, cine‑magnetic resonance imaging, laminectomy, syringomyelia

Introduction disabling parasthesias in all four limbs involving both hands and feet since 6 months. In addition, she Syringomyelia has commonly been associated with noted impaired pain and temperature sensation in the Chiari 1 malformation and is believed to be due the right upper limb. Spinal examination was normal.

to disequilibrium between the cranial and spinal Neurologically, she had weakness and wasting of the subarachnoid spaces secondary to tonsilar impaction small muscles of the right hand, with normal tone. at the foramen magnum.[1] This condition may also Deep tendon reflexes were diminished in both upper occur secondary to cervical spinal cord trauma, limbs and normal in the lower limbs. Plantar reflexes tubercular arachnoiditis and as an idiopathic entity. were bilaterally flexor. She had sensory loss to pain and Cervical spondylosis is classically seen as a disease which temperature in bilateral C5 and C6 dermatomes. causes extradural . We present a case in which a patient with cervical spondylosis presented Imaging with a near holocord syringomyelia. The patient was evaluated with an MRI of the craniovertebral junction and the whole spine, which revealed spondylotic changes in the cervical spine Case Report predominantly from C4‑C6. A holocord syrinx, hypointense on T1 and hyperintense on T2 was seen. A 53‑year‑old housewife presented with history of There was no evidence of tonsilar descent and the cistern weakness and wasting of the right forearm and hand magna was well seen [Figure 1a‑d]. since 1 year. She also complained of significant almost

Access this article online Cine MRI was performed to document CSF flow which showed obstruction of CSF flow in the spinal Quick Response Code: Website: subarachnoid space opposite C4‑C6 and free flow of www.ruralneuropractice.com CSF both anterior and posterior to the spinal cord at the foramen magnum [Figure 2a and b].

DOI: 10.4103/0976-3147.145215 Dynamic flexion‑extension x‑rays of the cervical spine did not show any instability. Normal lordosis was maintained.

Address for correspondence: Dr. Nupur Pruthi, Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India. E‑mail: [email protected]

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b

a c d Figure 1: MRI of the cervical spine and CVJ showing a syrinx on T1 (a) and T2 (b) images. Normal position of the tonsils (c) and a holocord syrinx (d)

a b a b Figure 2: Cine-MRI showing a block in CSF flow opposite C4-C6 Figure 3: MRI at 3 months following showing resolution of (a and b) syrinx. T1 (a) and T2 (b) images

Management is an extremely rare entity with very few previous The patient underwent a decompressive laminectomy reports in literature. Lucci, et al. in a description of from C3‑C6 and recovered uneventfully following three cases of neurogenic osteopathy secondary to surgery. She was followed up 6 weeks, 3 months and a syringomyelia found that one of their cases had 6 months following surgery and reported significant an associated cervical compressive element at C4.[2] reduction in the parasthesias. An MRI was performed Kimura et al., in 2004, reported a patient with radicular 3 months following surgery which showed almost symptoms and a syringomyelic syndrome who was complete resolution of the syrnix with small residual found to have cervical spondylosis with focal C4‑5 signal changes opposite C4 [Figure 3a and b]. instability who underwent decompression and stabilization. The patient improved following surgery Discussion and a post operative MRI 2 months following surgery showed marked resolution of the syrinx.[3] Rebai The association of cervical spondylosis with syrinx et al. reported a 70‑year‑old patient with features of

Journal of Neurosciences in Rural Practice | 2014 | Vol 5 | Supplement 1 S79 Venkata, et al.: Cervical spondylosis and syringomyelia syringomyelic syndrome who had evidence of cervical fusion. The patient improved symptomatically but post cord compression secondary to multiple cervical disc operative MRI did not show any significant alteration bulges and an associated syringomyelobulbia. The in cord changes.[6] patients symptoms and the syrinx resolved following extensive decompressive lamimectomy, similar to the In 2006, Butteriss, et al. reported another patient of case reported herein.[4] Kameyama and colleagues cervical spondylosis who had presented with spastic described cervical cord changed in nine patients of quadriparesis and was found to have severe cervical ossified posterior longitudinal ligament and had spondylosis with compression at C5/6 and C6/7 with an one case with a cervical syrinx with significant additional syrinx from C7 to D6. Their patient, however, compression, who improved with immobilization of declined surgical intervention.[7] the neck.[5] In a paper on seven cases of syringomyelia without Kaar et al. reported a patient with cervical spondylotic Chiari malformation, Lee et al. noted arachnoiditis, radiculomyelopathy with significant cord compression post laminectomy kyphosis, and focal at C4‑6, who underwent anterior decompression and compressive lesions at D7 and L1 to be associated

Table 1: Reported cases of syrinx secondary to spinal cord compression Author/year Age/sex Presentation Imaging Treatment Follow-up symptoms Follow-up imaging Lucci, 1981[2] 56/M Syringomyelic, C4 osteophyte with syrinx - - - arthropathy Kaneyama, 1995[5] 59/M C3-4, C6-7 compression. Immobilization Upper limb symptoms - Syrinx at C1-3/4 resolved Kaar, 1996[6] 71/F Syringomyelic C3-4 instability Posterior Not specified, No change in syrinx syndrome, Syrinx - C1-D1 decompression, improved L>R fusion C3/4 Rebai, 2002[4] 70/M Syringomyelic Multiple cervical discs Decompressive 6 months improved Resolution syndrome syringomyelobulbia laminectomy Lee, 2002[8] 18/M Ataxia, L Hydrocephalus, VP/SP shunt, No improvement No change weakness holocord syrinx FMD 28/M Paraparesis Post laminectomy kyphosis - Anterior fusion Neurologically Intact Reduced to C2-C6 C4/5, syrinx C2-T2 C4-6, posterior fusion C3-5 55/M Left lower Post traumatic Removal of cyst, Improved No change limb pain and pseudomeningeal cyst D6-8 augmentation weakness syrinx - thoracic duraplasty 38/M Paraparesis, Syrinx D6-D10, D12-L2, post Laminectomy+SS Mild Resolution voiding traumatic arachnoiditis Shunt, with dysfunction revision to SP 47/M Left lower Syrinx D4-D9 TB D7-10 Mild No change limb pain and arachnoiditis Laminectomy, weakness adhesiolysis 44/M Right Hydrocephalus, holocord VP Shunt Improved Resolution hemiparesis syrinx 48/F Right L1 compression fracture, D12-L2 Improved No change parasthesias, holocord syrinx decompression right lower and fusion, SS limb paresis shunt Kimura, 2004[3] 64/F Cervical C4/5 C6/7 compression C4/5 C6/7 2 months improved Resolution radiculopathy syrinx - C2-D3 discectomy+cage with syringomyelic syndrome Butteriss, 2006[7] 70/M Cervical C5/6 syrinx - C7-D6 Refused - - myelopathy treatment Landi, 2013[9] 66/F Cervical C3-T1 syrinx, disc 4 level Mild improvement Worsening of syrinx myelopathy bulges on extension laminectomy and C34,45,56,67,C7-D1 posterior fusion

Present 53/F Syringomyelic C4-6 compression, holocord C3-4 6 months improved Resolution syndrome syrinx laminectomy VP: Ventriculoperitoneal, SP: Syringoperitoneal, SS: Syringosubarachnoid, FMD: Foramen magnum decompression

S80 Journal of Neurosciences in Rural Practice | 2014 | Vol 5 | Supplement 1 Venkata, et al.: Cervical spondylosis and syringomyelia with syrinxes. Patients were evaluated with pre and represent a “pre‑syrinx” stage, as theorized by Fishbein post intervention cine MRI and in the cases of post et al., and may therefore have a better prognosis following laminectomy kyphosis and compression at D7, surgical treatment.[18] decompression at the appropriate level showed [8] improvement in CSF flow post operatively. Conclusion Most recently Landi et al. have described a patient who The association of cervical spondylosis with had a cervical syrinx and compression secondary to syringomyelia is possibly an etiological one and multiple prolapsed discs demonstrated on a dynamic Cine‑MRI with CSF flow studies in addition of MRI. The patient underwent decompression and evaluation of the CVJ and whole spine to rule out stabilization but on a follow‑up MRI the syrinx showed coincidental causes of a syrinx is a valuable diagnostic progression.[9] tool to plan treatment. Decompression of the local The clinical characteristics, imaging findings, treatment pathology often leads to resolution of the syrinx and outcome of patients with these conditions are shown and clinical improvement. The existing concept of in Table 1. cord signal changes in cervical spondylosis being a poor prognostic factor may not be strictly true and a The theories of genesis of syrnix secondary to cervical small subset of patients with such changes do show spondylosis include ischemia causing degeneration resolution of both clinical symptoms and radiological of tracts,[2] microtrauma at the site of compression findings. causing myelomalacia and caviation,[6,10] a sloshing effect secondary to a local block in CSF flow[3,11] and References dissociation of pressure above and below the block leading to transmural movement of fluid.[12] An 1. Heiss JD, Patronas N, DeVroom HL, Shawker T, Ennis R, Kammerer W, extensive review by Klekamp outlines these theories.[13] et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg 1999;91:553‑62. The most recent theory is the one where the syrinx 2. Lucci B, Reverberi S, Greco G. Syringomyelia and syringomielic syndrome

itself is formed by extracellular fluid movement into by cervical spondylosis. Report on three cases presenting with neurogenic the spinal cord parenchyma due to a dissociation of osteoarthropathies. J Neurosurg Sci 1981;25:169‑72. [14] 3. Kimura R, Park YS, Nakase H, Sakaki T. Syringomyelia caused by cervical intraparenchymal and subarachnoid pulse pressure. spondylosis. Acta Neurochir (Wien) 2004;146:175‑8. The formation of a syrnix in Chiari I malformations 4. Rebai R, Boudawara MZ, Ben Yahia M, Mhiri C, Ben Mansour H. has been attributed to the Bernoulli theorem and an Syringomyelobulbia associated with cervical spondylosis. Pathophysiology and therapeutic implications. Neurochirurgie 2002;48:120‑3. increased velocity of CSF flow across the foramen 5. Kameyama T, Hashizume Y, Ando T, Takahashi A, Yanagi T, Mizuno J. magnum leading to reduced spinal subarachnoid Spinal cord morphology and pathology in ossification of the posterior space pressure and subsequent distension of the longitudinal ligament. Brain 1995;118:263‑78. cord itself.[14] The various theories of generation of a 6. Kaar GF, N’Dow JM, Bashir SH. Cervical spondylotic myelopathy with syringomyelia. Br J Neurosurg 1996;10:413‑5. syrinx in spinal cord compression only point to the 7. Butteriss DJ, Birchall D. A case of syringomyelia associated with cervical fact that our understanding of this pathology is still spondylosis. Br J Radiol 2006;79:e123‑5. incomplete. This fact is further endorsed by reports of 8. Lee JH, Chung CK, Kim HJ. Decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation. Spinal spontaneous regression of syringomyelia even with Cord 2002;40:501‑6. Chiari malformations.[15] There is therefore a definite 9. Landi A, Nigro L, Marotta N, Mancarella C, Donnarumma P, Delfini R. association of syringomyelia with cervical spondylosis Syringomyelia associated with cervical spondylosis: A rare condition. World J Clin Cases 2013;1:111‑5. and reports of clinical and radiological improvement 10. Al‑Mefty O, Harkey LH, Middleton TH, Smith RR, Fox JL. Myelopathic following decompression with or without stabilization cervical spondylotic lesions demonstrated by magnetic resonance points toward an etiological relationship between the imaging. J Neurosurg 1988;68:217‑22. 11. Milhorat TH. Classification of syringomyelia. Neurosurg Focus two conditions. 2000;8:E1. 12. Levine DN. The pathogenesis of syringomyelia associated with lesions at Another valid inference from this case is that historically the foramen magnum: A critical review of existing theories and proposal the presence of myelomalacic changes in the cervical of a new hypothesis. J Neurol Sci 2004;220:3‑21. 13. Klekamp J. The pathophysiology of syringomyelia‑historical overview cord, namely hyperintensity on T2w images has been and current concept. Acta Neurochir (Wien) 2002;144:649‑64. associated with poor prognosis following surgery.[16,17] The 14. Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev prognosis is considered worse if there is a corresponding 2006;29:251‑4. 15. Tortora F, Napoli M, Caranci F, Cirillo M, Pepe D, Cirillo S, et al. hypointensity on T1w sequences. We propose that Spontaneous regression of syringomyelia in a young patient with Chiari some of these patients with limited local changes may type I malformation. Neuroradiol J 2012;25:593‑7.

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16. Avadhani A, Rajasekaran S, Shetty AP. Comparison of prognostic value state: Is there a reversible myelopathic condition that may precede of different MRI classifications of signal intensity change in cervical syringomyelia? Neurosurg Focus 2000;8:E4. spondylotic myelopathy. Spine J 2010;10:475‑85. How to cite this article: Bhagavathula Venkata SS, Arimappamagan 17. Suri A, Chabbra RP, Mehta VS, Gaikwad S, Pandey RM. Effect of A, Lafazanos S, Pruthi N. Syringomyelia secondary to cervical intramedullary signal changes on the surgical outcome of patients with spondylosis: Case report and review of literature. J Neurosci Rural cervical spondylotic myelopathy. Spine J 2003;3:33‑45. Pract 2014;5:78-82. 18. Fischbein NJ, Dillon WP, Cobbs C, Weinstein PR. The “presyrinx” Source of Support: Nil. Conflict of Interest: None declared.

Commentary

In this article, the authors introduced a rare case of management of this disorder. I hope various questions sylingomyelia secondary to cervical spondylosis.[1] with regard to this pathology will be solved in the future. Further, they performed literature review and discovered only 15 cases of other sylingomyelia secondary to spinal Hiroshi Kuroki cord compression. Although sylingomyelia associated Department of Orthopaedic Surgery, University of Miyazaki, with Chiari malformation is occasionally encountered, Miyazaki, Japan these cases are extremely uncommon. Therefore, I guess Address for correspondence: this paper should be published because of rarity. Dr. Hiroshi Kuroki, Department of Orthopaedic Surgery, Faculty of Medicine, University However, a couple of questions are still remaining of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki - 889 1692, Japan. unresolved. E-mail: [email protected]

It is broadly well known that sylingomyelia associated with References Chiari malformation sometimes causes scoliosis.[2,3] The 1. Savitr Sastri BV, Arivazhagan A, Lafazanos S, Pruthi N. Syringomyelia

prevalence of scoliosis accompanied with sylingomyelia secondary to cervical spondylosis – A case report and review of literature. was previously reported 2.8‑25.8%.[4‑6] Whereas, the J Neurosci Rural Pract 2014;5:S78-82. prevalence of sylingomyelia secondary to spinal cord 2. Arai S, Ohtsuka Y, Moriya H, Kitahara H, Minami S. Scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 1993;18:1591‑2. compression is unknown. 3. Kuroki H, Inomata N, Hamanaka H, Chosa E, Tajima N. Single‑group study to explore the optimal management of neuropathic scoliosis caused The authors speculated the pathophysiology of by neural axis abnormalities based on its clinical features. The Open Spine Journal 2012;4:28‑33. sylingomyelia secondary to spinal cord compression 4. Evans SC, Edgar MA, Hall‑Craggs MA, Powell MP, Taylor BA, by bibliographic consideration. But the discussion of Noordeen HH. MRI of “idiopathic” juvenile scoliosis. A prospective pathophysiology of sylingomyelia is also incomplete study. J Bone Joint Surg Br 1996;78:314‑7. 5. Shen WJ, McDowell GS, Burke SW, Levine DB, Chutorian AM. Routine because most diseases by spinal cord compression do preoperative MRI and SEP studies in adolescent idiopathic scoliosis. not always have sylingomyelia. In the sylingomyelia J Pediatr Orthop 1996;16:350‑3. associated with Chiari malformation, overcrowding in the 6. Gupta P, Lenke LG, Bridwell KH. Incidence of neural axis abnormalities in posterior cranial fossa due to a normal‑sized hindbrain infantile and juvenile patients with spinal deformity. Is a magnetic resonance image screening necessary? Spine (Phila Pa 1976) 1998;23:206‑10. induces a downward herniation of the brain as well as 7. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. occlusion of cerebrospinal fluid (CSF) flow across the Pathogenesis of Chiari malformation: A morphometric study of the foramen magnum.[7] This obstructs natural flow of CSF posterior cranial fossa. J Neurosurg 1997;86:40‑7. 8. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology and may be responsible for the origin and maintenance of of syringomyelia associated with Chiari I malformation of the cerebellar syringomyelia by the pulsatile pressure waves forcing CSF tonsils. J Neurosurg 1994;80:3‑15. into the spinal cord through the perivascular and interstitial 9. Tokunaga M, Minami S, Isobe K, Moriya H, Kitahara H, Nakata Y. Natural history of scoliosis in children with sylingomyelia. J Bone Joint [8] spaces. Moreover, some sylingomyelia associated with Surg Br 2001;83:371‑6. Chiari malformation are spontaneously diminishing. [9] In scoliosis cases, Tokunaga et al. reported the size Access this article online of sylingomyelia associated with Chiari malformation Quick Response Code: decreased 50% or more in 14 of 27 cases in accordance with growth that promotes elevation of the tonsils. Website: www.ruralneuropractice.com The next step that we should do is accumulation of similar cases. Amassed data must contribute to properly elucidate prevalence, mechanism, and established

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