- Department of Neurosurgery, Illinois Neurological Institute, University of Illinois College of Medicine, Peoria, Illinois
Jay A. Vachhani
Department of Neurosurgery, Illinois Neurological Institute, University of Illinois College of Medicine, Peoria, Illinois
DOI:10.4103/2152-7806.98525Copyright: © 2012 Vachhani JA. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Vachhani JA, Fassett DR. Intramedullary neurenteric cyst associated with a tethered spinal cord: Case report and literature review. Surg Neurol Int 14-Jul-2012;3:80
How to cite this URL: Vachhani JA, Fassett DR. Intramedullary neurenteric cyst associated with a tethered spinal cord: Case report and literature review. Surg Neurol Int 14-Jul-2012;3:80. Available from: http://sni.wpengine.com/surgicalint_articles/intramedullary-neurenteric-cyst-associated-with-a-tethered-spinal-cord-case-report-and-literature-review/
Background:Neurenteric cysts are benign tumors of the central nervous system (CNS) that represent 0.3% to 0.5% of all spinal cord tumors. They are usually extramedullary and found in the lower cervical and thoracic spine. Only 12.2% of neurenteric cysts are documented to be intramedullary.
Case Description:The authors report a case of a 35-year-old female that presented with progressive weakness and loss of coordination in her legs. Magnetic resonance imaging (MRI) showed an intramedullary cystic lesion in the thoracolumbar region and a low-lying conus medullaris suggesting tethered cord. The patient was taken to the operating room for detethering of her spinal cord and resection of the lesion. Pathologic examination of the tissue confirmed the diagnosis of a neurenteric cyst.
Conclusion:A search of the literature since the advent of MRI showed 29 published cases of intramedullary neurentic cysts. Of the 24 published cases with a follow-up MRI, the average recurrence rate was 25% with a mean follow up of 51 months.
Keywords: Intramedullary, neurenteric cyst, tethered spinal cord
Neurenteric cysts, also known as enterogenous cysts or endodermal cysts, are benign tumors of the central nervous system (CNS) which are thought to represent 0.3% to 1.3% of all spinal cord tumors.[
Since the advent of magnetic resonance imaging (MRI), it has become possible to diagnose these lesions preoperatively. On T2-weighted imaging, the cysts are hyperintense and display minimal or no enhancement on T1 postcontrast imaging. Typically, the cysts are intradural/extramedullary (78.6%) and usually arise from the cervical, cervicothoraric, and thoracic spine (73.6%).[
This case is unique because the patient presented at the age of 35 with an intramedullary neurenteric cyst in the thoracolumbar spine and a tethered spinal cord. In this paper, we will discuss the treatment of this patient as well as a review of all published literature on patients with intramedullary neurenteric cysts since the advent of MRI.
A 35-year-old female presented with weakness in both legs and loss of coordination over the last year. She had some numbness and burning from her feet to her knees bilaterally and intermittent episodes of bladder incontinence.
On examination, she had no cranial nerve deficits and strength and sensation were normal in both upper extremities. She had bilateral lower extremity weakness, grade 3/5 in both dorsiflexors, and 4/5 in all other muscle groups. Her reflexes were 1+ at the knees and ankles with a very wide-based antalgic gait.
MRI of the spine was performed which showed a cystic dilation of her distal cord near T12-L1 that had a similar intensity to cerebrospinal fluid (CSF) and no contrast enhancement. The cord also appeared to be low lying and terminated at the level of L3 [Figures
A laminectomy was performed from T11 to L1 and L5 to S1. Initial attention was focused at the L5-S1 level where an intradural dissection was performed and the filum terminale was indentified, cauterized, and sectioned. After release of the tethered cord, the dura was opened from T11 to L1 and the spinal cord was visualized with an intraoperative microscope. A midline myelotomy was performed and a firm capsule was encountered within the cord. The cyst capsule was incised to internally decompress the cyst and allow for resection while minimizing trauma to the normal surrounding spinal cord. A milky white fluid was expressed upon opening the cyst. Microsurgical resection of the cyst capsule was performed while closely monitoring motor evoked potentials (MEP) and somatosenory evoked potentials (SSEP). A large portion of the cyst wall was resected, but a complete resection was aborted after an 80% decrease in MEP.
The patient awoke with increased weakness, in comparison to her preoperative exam, with 2/5 hip flexors, 3/5 knee extensors, 1/5 knee flexors, and absent dorsiflexors, and plantar flexors. Her neurological function gradually improved over the next four weeks. With rehabilitation, her strength and coordination improved with the exception of her left dorsiflexors, which remained 3/5 strength.
Hematoxylin and eosin staining revealed multiple irregular cystic spaces lined by epithelium [
Intramedullary neurenteric cysts are thought to represent 12.2% of all neurenteric cysts. In the published pediatric literature, the cervical spine was the most common presenting location (36%).[
The treatment options for intramedullary neurenteric cysts remain controversial. Most authors advocate total resection when possible.[
In the current published literature, the overall recurrence rate for neurenteric cysts after surgical resection varies from 11.6% to 37%.[
Tethered cord syndrome has been reported in association with neurenteric cysts.[
Intramedullary neurenteric cysts are extremely rare spinal cord lesions that are benign in nature. We report a case of an intramedullary neurenteric cyst in the thorocolumbar region with an associated tethered cord. There are 29 cases in the published literature since the advent of MRI and of the cases with a follow-up MRI, the overall recurrence rate was found to be 25%.
The authors acknowledge Dr. Meena Gujrati for providing digital copies of the patient's pathology slides and Joanna Fleckenstein for her assistance in proof reading and editing.
1. Agarwal D, Sura A, Mahapatra AK, Sharma MC. Intramedullary neurenteric cyst presenting as infantile paraplegia: A case and review. Pediatr Neurosurg. 2002. 37: 93-6
2. Aydin AL, Sasana M, Ucar B, Afsharian R, Ozer AF. Prenatal diagnosis of a large, cervical, intraspinal, neurenteric cyst and postnatal outcome. J Pediatr Surg. 2009. 44: 1835-8
3. Bakaris S, Senoglu M, Yuksel Z, Gokce M. Lumbosacral neurenteric cyst with associated tethered cord in an adult. Clin Neuropathol. 2005. 24: 51-5
4. Cai C, Shen C, Yang W, Zhang Q, Hu X. Intraspinal neurenteric cysts in children. Can J Neurol Sci. 2008. 35: 609-15
5. De Oliveira RS, Cinalli G, Roujeau T, Sante-Rose C, Pierre-Kahn A, Zerah M. Neurenteric cysts in children: 16 consecutive cases and review of the literature. J Neurosurg. 2005. 103: 512-23
6. De Oliveira RS, Cinalli G, Sante-Rose C, Machado HR, Zerah M, Ozek MM, Cinalli G, Maixne WJ.editors. Neurenteric cysts. Spina Bifida: Management and Outcome. New York, NY: Springer; 2008. p. 465-85
7. Freund F, Thale A, Hutzelmann A. Radiologic and histopathologic findings in a rare case of complex occult spinal dysraphism with association of a lumbar fibrolipoma, neurenteric cyst and tethered cord syndrome. Eur Radiol. 1998. 8: 624-7
8. Gupta SK, Khosla VK, Sharma BS, Mathuriya SN, Pathak A, Tewari MK. Tethered cord syndrome in adults. Surg Neurol. 1999. 52: 362-70
9. Jain RS. A spinal neurenteric cyst presenting as burning feet syndrome. Int J Clin Pract. 2003. 57: 846-7
10. Jhawar SS, Mahore A, Goel A. Intramedullar spinal neurenteric cyst with fluid-fluid level. J Neurosurg Pediatr. 2012. 9: 542-5
11. Klekamp J. Tethered cord syndrome in adults. J Neurosurg Spine. 2011. 15: 258-70
12. Lee GY, Paradiso G, Tator CH, Gentili F, Massicotte EM, Fehlings MG. Surgical management of tethered cord syndrome in adults: Indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine. 2006. 4: 123-31
13. Lew SM, Kothbauer KF. Tethered cord syndrome: An updated review. Pediatr Neurosurg. 2007. 43: 236-48
14. Lipmann CR, Arginteanu M, Purohit D, Naidich TP, Camins MB. Intramedullary neurenteric cysts of the spine: Case report and review of the literature. J Neurosurg. 2001. 94: 305-9
15. Menezes AH, Traynelis VC. Spinal neurenteric cysts in the magnetic resonance imaging era. Neurosurgery. 2006. 58: 97-105
16. Muzumdar D, Bhatt Y, Sheth J. Intramedullary cervical neurenteric cyst mimicking an abscess. Pediatr Neurosurg. 2008. 44: 55-61
17. Nagi S, Ghorbel D, Drissi C, Maatallah Y, Hammami N, Hamouda M. Intramedullary neurenteric cyst without concurrent malformation. Australas Radiol. 2007. p. 51-
18. Paolini S, Ciappetta P, Domenicucci M, Guiducci A. Intramedullary neurenteric cyst with a false mural nodule: Case report. Neurosurgery. 2003. 52: 243-6
19. Rauzzino MJ, Tubbs RS, Alexander E, Grabb PA, Oakes WJ. Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism. Neurosurg Focus. 2001. 10: e2-
20. Reinders JW, Wesseling P, Hilkens PH. Intramedullary enterogenous cyst presenting with spastic paraparesis during two consecutive pregnancies: A case report. J Neurol Neurosurg Psychiatry. 2001. 71: 528-30
21. Riviérez M, Buisson G, Kujas M, Ridarch A, Mignon E, Jouannelle A. Intramedullary neurenteric cyst without any associated malformation: One case evaluated by RMI and electron microscopic study. Acta Neurochir (Wien). 1997. 139: 887-90
22. Rotondo M, D’Avanzo R, Natale M, Pasqualetto L, Bocchetti A, Agozzino L. Intramedullary neurenteric cysts of the spine: Report of three cases. J Neurosurg Spine. 2005. 2: 372-6
23. Savage JJ, Casey JN, McNeill IT, Sherman JH. Neurenteric cysts of the spine. J Craniovertebr Junction Spine. 2010. 1: 58-63
24. Sharma RR, Ravi RR, Gurusinghe NT, Coutinho C, Mahapatra AK, Sousa J. Cranio-spinal enterogenous cysts: Clinico-radiological analysis in a series of ten cases. J Clin Neurosci. 2001. 8: 133-9
25. Singhal BS, Parakh HN, Ursekar M, Deopujari CE, Manghani DK. Intramedullary neurenteric cyst in mid thoracic spine in an adult: A case report. Neurol India. 2001. 49: 302-4
26. Stetler WR, Park P, Sullivan S. Pathophysiology of adult tethered cord syndrome: Review of the literature. Neurosurg Focus. 2010. 29: E2-
27. Takahashi S, Morikawa S, Saruhashi Y, Matsusue Y, Kawakami M. Percutaneous transthoracic fenestration of an intramedullary neurenteric cyst in the thoracic spine with intraoperative magnetic resonance image navigation and thoracoscopy. J Neurosurg Spine. 2008. 9: 488-92
28. Theret E, Litre CF, Lefebvre F, Eap C, Duntze J, Scherpereel B. Huge intramedullar neurenteric cyst with intrathoracic development in a 1 month-old boy: Excision though the anterior approach. A case report and review of the literature. Acta Neurochir (Wien). 2010. 152: 481-3
29. Yamada S, Won DJ, Pezeshkpour G, Yamada BS, Yamada SM, Siddiqi J. Pathophysiology of tethered cord syndrome and similar complex disorders. Neurosurg Focus. 2007. 23: E6-
30. Yilmaz C, Sulsen S, Sonmez E, Ozger O, Unlukaplan M, Caner H. Intramedullary bronchogenic cyst of the conus medullaris. J Neurosurg Spine. 2009. 11: 477-9
31. Ziu M, Vibhute P, Vecil GG, Henry J. Isolated spinal neurenteric cyst presenting as intramedullary calcified cystic mass on imaging studies: Case report and review of literature. Neuroradiology. 2010. 52: 119-23