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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 187-189

Ventral foramen magnum neurenteric cyst presenting as acute rapidly progressive quadriparesis and respiratory compromise: A case report and review of literature

Department of Neurosurgery, Vikram Hospital, Bengaluru, Karnataka, India

Date of Submission26-Nov-2012
Date of Decision11-Feb-2013
Date of Acceptance17-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Ravi Mohan Rao
Department of Neurosurgery, Vikram Hospital, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.111151

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How to cite this article:
Sharma R, Chandramouli T C, Rao RM. Ventral foramen magnum neurenteric cyst presenting as acute rapidly progressive quadriparesis and respiratory compromise: A case report and review of literature. Neurol India 2013;61:187-9

How to cite this URL:
Sharma R, Chandramouli T C, Rao RM. Ventral foramen magnum neurenteric cyst presenting as acute rapidly progressive quadriparesis and respiratory compromise: A case report and review of literature. Neurol India [serial online] 2013 [cited 2021 Jul 28];61:187-9. Available from:


Neurenteric cysts of the central nervous system are rare and result from failure of separation of the neuroectodermal and neuroendodermal elements during the third week of embryogenesis. [1],[2] These cysts are extremely rare at the craniovertebral junction. [1],[2],[3] An unexcised craniovertebral junction cyst maybe fatal. [1] We report a case of a ventral foramen magnum neurenteric cyst presenting with rapidly progressive neurologic deficits.

An 18-year-old girl noticed left hemiparesis after getting up from sleep. Over the next 12 hours, she developed similar weakness in her rightsided limbs. On admission, vital parameters were normal and single breath count was 26. Neurologic examination revealed quadriparesis with motor power of 3/5 in all four limbs; all deep tendon reflexes were brisk, jaw jerk was absent, and bilateral Babinski sign was present. There was no cranial nerve or sensory deficits. There were no bony anomalies in the radiograph of the cervical spine. Magnetic resonance imaging (MRI) of the cervical spine [Figure 1] showed a ventral foramen magnum cystic lesion measuring 1.2 × 1.3 × 1.2 cm compressing the cervicomedullary junction ventrally and displacing it posteriorly. Next morning, she developed breathing difficulty and further neurological deterioration. Respiratory rate was 36 breaths per minute with the activation of accessory muscles during inspiration and oxygen saturation was 99%. Motor power deteriorated to 2/5 in all four limbs. The patient was intubated and started on mechanical ventilation. She was taken up for emergency surgery and underwent a right far lateral approach, cyst decompression, and near-total excision of cyst wall as we failed to dissect the cyst wall free from the anterior surface of the cervicomedullary junction (due to dense adhesions). The cyst contained thick milky fluid. There was no evidence of hemorrhage or rupture of the cyst. Postoperatively, her respiration was normal and limbs power improved to 4/5. Microscopic examination [Figure 2] showed that the cyst was lined with pseudostratified cuboidal and columnar epithelium, thus confirming an enterogenous cyst. At a follow-up after a month, she was independent and had started going to school. Postoperative MRI [Figure 3] at one month showed a small signal change within the lower medulla and muscular plane pseudomeningocele. She has been advised regular clinical and MRI follow-up.

Neurenteric cysts can present at any age and are more prevalent in males. [4],[5] As neurenteric cysts are space-occupying lesions compressing the nervous tissue, the clinical presentation depends upon the location. Cysts at the craniovertebral junction are characterized by waxing and waning neck pain and occipital headache. Quadriparesis due to anterior medullary compression by the cyst is sufficiently described in the literature. [6] Dysfunction of the lower cranial nerves occurs rarely due to slow expansion of the cyst and availability of ample space at the craniovertebral junction. Meningitis secondary to bacterial infection or chemical irritation following leakage of cyst fluid is also common. [1] Though these cysts usually expand slowly, they may expand rapidly by mechanisms like increased secretion, hemorrhage, inflammation, or accumulation of cerebrospinal fluid (CSF). [1],[7],[8] MRI is the imaging modality of choice. These cysts are usually single, well defined, smooth, rounded, unilocular, and nonenhancing. [2] However, variability of signal changes on T1 and T2 is well known. [7] Bone abnormalities, which are commonly associated with spinal neurenteric cysts, are rarely noted with intracranial neurenteric cysts. [1],[3] Radiologic differential diagnosis is arachnoid, dermoid, or epidermoid cyst. [7],[9] Aspiration followed by complete microsurgical resection of the cyst wall is the treatment of choice, as aspiration alone generally results in recurrence. However, if the cyst wall is densely adherent to neural structures, evacuation of the cyst with partial removal of the cyst wall is acceptable. [1],[3] Coagulation of these adhered remnants of the cyst wall may be done to reduce chances of recurrence. [8] Also, definitive diagnosis can be made only from the tissue obtained at surgery. [1],[8] Though such lesions can be removed through anterior, anterolateral, lateral, or posterior approaches, anterior approaches (like transoral) are preferred as cysts are usually located ventral to neural structures. [3],[10] During surgery; spillage of cyst contents into the subarachnoid space should be avoided to prevent postoperative meningism. [8] Long-term follow-up and serial imaging is recommended for incompletely excised neurenteric cysts because of the possibility of recurrence, dissemination, and malignant transformation. [7],[11],[12],[13] Radiotherapy or chemotherapy is not indicated for the residual lesion. [8]
Figure 1: Preoperative magnetic resonance imaging of cervical spine (a) sagittal section showing ventral foramen magnum rounded lesion isointense on T1 compressing cervicomedullary junction causing cervicomedullary junction kink, (b) coronal section showing lesion uniformly hyperintense on T2, (c and d) T2 axial sections showing the absence of the plane of cleavage between posterior surface of the lesion and anterior surface of cervicomedullary junction; bilateral vertebral arteries are draping over the lesion

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Figure 2: Histopathological examination (H and E stain) showing pseudostratified cuboidal and columnar epithelium lining the cyst wall

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Figure 3: Postoperative magnetic resonance imaging of cervical spine T2 sequence (a, b, c, d) showing a small signal change (black arrow) in lower medulla and muscular plane pseudomeningocele

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This is probably the first case report in which a ventral foramen magnum neurenteric cyst presented with rapidly progressive neurologic deficits including respiratory failure, needing emergent life-saving surgical treatment. Acute onset and rapid deterioration within 36 hours of onset in our patient might be related to rapid expansion of the cyst secondary to increased secretion.

  Acknowledgment Top

The authors thank Dr. Susmita H Rakshit (Pathologist) for providing histopathological examination images.

  References Top

1.Fuse T, Yamada K, Kamiya K, Inagaki H. Neurenteric cyst at the craniovertebral junction: report of two cases. Surg Neurol 1998;50:431-6.  Back to cited text no. 1
2.Lee CW, SH Lee, Yu ST. Intracranial neurenteric cyst of the anterior brain stem in a girl. J Korea Child Neurol Soc 2012;20:18-22.  Back to cited text no. 2
3.Abe K, Oyama K, Mori K, Ishimaru S, Eguchi M, Maeda M. Neurenteric Cyst of the Craniocervical Junction. Neurol Med Chir (Tokyo) 1999;39:875-80.  Back to cited text no. 3
4.Faraji-Rad M. Neurenteric cyst of the craniovertebral junction. Arch Iranian Med 2003;6:59-62.  Back to cited text no. 4
5.Ashrafzadeh F, Faraji M. Torticollis caused by neuroenteric cyst of upper cervical region. IJCN 2006;1:43-6.  Back to cited text no. 5
6.Kak VK, Gupta RK, Sharma BS, Banerjee AK. Craniospinal enterogenous cyst: MR findings. J Comput Assist Tomogr 1990;14:470-2.  Back to cited text no. 6
7.Priamo FAI, Jimenez ED, Benardete EA. Posterior fossa neurenteric cysts can expand rapidly: case report. Skull Base Rep 2011;1:115-24.  Back to cited text no. 7
8.Gauden AJ, Khurana VG, Tsui AE, Kaye AH. Intracranial neuroenteric cysts: A concise review including an illustrative patient. J Clin Neurosci 2012;19:352-9.  Back to cited text no. 8
9.Preece MT, Osborn AG, Chin SS, Smirniotopoulos JG. Intracranial neurenteric cysts: imaging and pathology spectrum. AJNR 2006;27:1211-6.  Back to cited text no. 9
10.Gupta SK, Sharma BS, Khosla VK, Mathuria SN, Pathak A, Tewari MK. Far lateral approach for foramen magnum lesions. Neurol Med Chir (Tokyo) 2000;40:48-54.  Back to cited text no. 10
11.Ghannane H, Laghmari M, Aniba K, Lmejjati M, Benali SA. Craniocervical intradural neurenteric cyst. Pan Arab J Neurosurg 2011;15:64-7.  Back to cited text no. 11
12.De Oliveira RS, Cinalli G, Roujeau T, Sainte-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.  Back to cited text no. 12
13.Menezes AH, Traynelis VC. Spinal neurenteric cysts in the magnetic resonance imaging era. Neurosurgery 2006;58:97-105.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]

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