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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 1  |  Page : 129-131

Dorsally placed extradural infected neurenteric cyst in a two-year old with paraspinal extension

1 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Date of Submission26-Nov-2011
Date of Decision30-Nov-2011
Date of Acceptance04-Dec-2011
Date of Web Publication7-Mar-2012

Correspondence Address:
Pravin Salunke
Department of Neurosurgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.93622

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How to cite this article:
Savardekar A, Salunke P, Rane S, Chhabra R. Dorsally placed extradural infected neurenteric cyst in a two-year old with paraspinal extension. Neurol India 2012;60:129-31

How to cite this URL:
Savardekar A, Salunke P, Rane S, Chhabra R. Dorsally placed extradural infected neurenteric cyst in a two-year old with paraspinal extension. Neurol India [serial online] 2012 [cited 2023 Dec 11];60:129-31. Available from:


Neurenteric cysts (NECs) are rare developmental lesions, lined with epithelium of endodermal origin and result from partial obliteration of the neurenteric canal and consist of heterotopic rests of epithelium reminiscent of the gastrointestinal and respiratory tract. These lesions commonly present in the second decade and are usually ventral or ventrolateral to the cord in the intradural compartment. [1] An extradural NEC posterior to the cord without spina bifida is extremely rare. [2]

A two-year-old boy, presented with progressive difficulty in walking and frequent falls since three months. He had not attained bowel and bladder continence yet. Examination revealed paraparesis and loss of perianal sensations. Spine examination showed no tuft of hair or sinus. Plain X-ray of the dorso-lumbar spine revealed erosion of right pedicle at T12 level with enlarged right-sided T11-12 intervertebral foramen, but no spina bifida. Magnetic resonance imaging (MRI) of the spine revealed an enhancing cystic lesion at T11-12 level, placed dorsal to the spinal cord with an extension to the right paraspinal region [Figure 1]a-f. Patient underwent T11-12 laminectomy in prone position. The posterior elements appeared normal. A purely extradural cystic lesion with extension into the paraspinal space on the right side was encountered that was excised completely. The cyst contained pus that grew E. coli. Histopathology showed a multi-loculated cyst lined by tall columnar epithelium with villi and goblet cells suggestive of enteric cyst [Figure 2]a-e.
Figure 1: (a) Axial contrast MRI image showing dorsally placed lesion with enhancing wall (arrow); (b) axial image showing extension of the lesion through right neural foramina (arrow); (c) axial MRI image showing extension of the cyst in the paraspinal muscles (arrow). The lamina is seen intact in all the three images. (d) X-ray AP view of the thoracic spine showing erosion of the pedicle on the right side (arrow). (e) Paraspinal MRI image showing lesion in the neural foramina (arrow). (f) Posterior extradural lesion (arrow) seen on the midsagittal MRI image

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Figure 2: (a) Scanner view (× 2 magnification, hematoxylin and eosin stain) showing cystic space with wall of variable thickness, lumen being filled with proteinaceous material. (b-d) Sequential higher power views (× 20 and × 40 magnification) showing the cyst wall to be composed of a nearly completely formed small intestinal wall, with mucosa, muscularis mucosae, submucosa and muscularis propria. Mucosa is lined by tall columnar epithelium with goblet cells. Intestinal crypts are also seen. (e) High magnification (× 40) showing presence of goblet cells and Paneth cells

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NECs may be found at any level from the posterior clinoid to the coccyx, although commonly encountered near the cervicodorsal junction. [3],[4] The spectrum varies depending upon the portion of the neurenteric canal that fails to obliterate. They may be located between the gut and vertebral bodies (commonly in the posterior mediastinum) or intraspinally or both, entering the spinal canal through vertebral body defect. [3] These cysts are generally located ventral or ventrolateral to the spinal cord. The majority of them are intradural extramedullary (95%), the remaining being intramedullary, though a few may extend extradurally. [3],[5] Dorsally placed NECs are very rare. [6] Extradural intraspinal NECs have been reported. [7] However, most of them were associated with spina bifida. [2] An extensive review of the literature revealed only 2 children with purely extradural NECs.One of them (16 years old) had no bony abnormality. [6] NECs, in 50% of cases, are associated with vertebral anomalies like spinal dysraphism, scoliosis, spina bifida, split cord malformation and Kippel-Fiel syndrome, anterior spina bifida being the hallmark. [1] Dorsally placed neurenteric lesions are associated with spina bifida. [2]

NECs in the spinal canal usually present in the second and third decade of life with myelopathy and should be considered an important differential in a ventrally placed intradural extramedullary cystic lesion. They rarely present in early childhood. [2] Infected NECs are rare. [8] Our patient had infected NEC without any focus for hematogenous infection or dermal sinus. The dormant microbes within quiescent nest cells of the gut epithelium may start actively proliferating depending upon the milieu and immune status.

MRI usually shows extra-axial, non-enhancing cystic lesions, isointense to the cerebrospinal fluid (CSF) in both T1 and T2-weighted images. [1] Management of NEC is surgery; total excision is desirable as these cysts are known to recur. However, a safe total excision cannot be achieved in all cases due to tight adhesion of the cystic wall to neural and vascular structures. [1] Laminoplasty is preferred over laminectomy especially in young children to avoid postoperative kyphotic deformity. [6]

The highlights of this report are early age of presentation (<2 years), dorsal extradural location without any associated bony abnormality and presence of infection. Absence of a dermal sinus and bony defects actually favor the possibility that NECs are due to ectopia. The possible theories for the formation of NECs are: a) persistent or accessory neurenteric canal with split notochord, b) primary adhesion of endoderm anterior to notochord, c) incomplete excalation of the notochord, and d) ectopic endoderm. [2]

 » References Top

1.Savage JJ, Casey JN, McNeill, Sherman JH. NECs of the spine. J Craniovertebr Junction Spine 2010;1:58-63.  Back to cited text no. 1
2.Rauzzino MJ, Tubbs RS, Alexander E, Grabb PA, Oakes WJ. Spinal NECs and their relation to more common aspects of occult spinal dysraphism. Neurosurg Focus 2001;10:e2.  Back to cited text no. 2
3.Kumar R, Jain R, Rao KM, Hussain N. Intraspinal NECs-report of three pediatric cases. Childs Nerv Syst 2001;17:584-8.  Back to cited text no. 3
4.Satyarthee GD, Mahapatra AK. Presacral NEC in an infant. Pediatr Neurosurg 2003;39:222-4.  Back to cited text no. 4
5.Agrawal D, Suri A, Mahapatra AK, Sharma MC. Intramedullary NEC presenting as infantile paraplegia: A case and review. Pediatr Neurosurg 2002;37:93-6.  Back to cited text no. 5
[PUBMED]  [FULLTEXT] Oliveira RS, Cinalli G, Roujeau T, Sainte-Rose C, Pierre-Kahn A, Zerah M. NECs in children: 16 consecutive cases and review of the literature. J Neurosurg 2005;103(6 Suppl):512-23.  Back to cited text no. 6
7.Sharma RR, Ravi RR, Gurusinghe NT, Coutinho C, Mahapatra AK, Sousa J, et al. Cranio-spinal enterogenous cysts: Clinico-radiological analysis in a series of ten cases. J Clin Neurosci 2001;8:133-9.  Back to cited text no. 7
8.Abhishek A, Anushree A, Patir R, Sehgal AD. Extreme lateral approach in a case of acute-onset quadriplegia due to high cervical neurenteric cyst. Pediatr Neurosurg 2007;43:134-6.  Back to cited text no. 8


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