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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 1  |  Page : 206-208

Large Neuroenteric Cyst at Cranio-Vertebral Junction with Cardiorespiratory Arrest

Department of Neurosurgery, Medica Superspecialty Hospital, Kolkata, West Bengal, India

Date of Submission20-Apr-2019
Date of Decision22-May-2019
Date of Acceptance27-May-2019
Date of Web Publication24-Feb-2021

Correspondence Address:
Indrajit Rana
Medica Superspecialty Hospital, 127 Eastern Metropolitan Bypass Mukundapur, Kolkata - 700 099, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.310082

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How to cite this article:
Tripathy LN, Rana I, Jain H. Large Neuroenteric Cyst at Cranio-Vertebral Junction with Cardiorespiratory Arrest. Neurol India 2021;69:206-8

How to cite this URL:
Tripathy LN, Rana I, Jain H. Large Neuroenteric Cyst at Cranio-Vertebral Junction with Cardiorespiratory Arrest. Neurol India [serial online] 2021 [cited 2021 Apr 11];69:206-8. Available from:


Neuroenteric cyst (NEC) is a benign congenital condition which results from failure of separation of the neuroectodermal and neuroendodermal elements during third week of embryogenesis. NEC of the cranio-vertebral junction (CVJ) is extremely rare.[1] We report one such case of a large cyst with an acute presentation.

We report a case of 24-year-old lady who was admitted with a history of acute severe neck pain experienced in a flight that aborted a last minute take off by screeching to a halt. On admission, her vitals were stable, she had mild spastic quadriparesis. Contrast MRI showed an anteriorly placed large (50 × 29 × 15 mm3) cystic lesion causing severe compression at the CVJ [Figure 1]. Within 12 h of admission, she developed sudden cardio-respiratory arrest (CRA) which was revived with single cycle of cardio-pulmonary resuscitation (CPR). Following the event, she had significant quadriparesis with bladder involvement. She was taken for emergency surgery. A suboccipital craniotomy, C1 laminectomy and microsurgical excision of cystic intradural lesion were done. During surgery, the cyst could be removed entirely from the anterior cervico-medullary junction after dividing the first ligamentum denticulatum on the right side [Figure 2]. The lower cranial nerves and the right vertebral artery were found to be draped over the cyst wall, which were dissected off the cyst carefully. The contents were milky white and viscid. The cyst content was sterile on culture. Histopathology report confirmed it as NEC [Figure 3]. Her post-operative recovery was satisfactory. There was no residual cyst component in follow-up scan [Figure 4].
Figure 1: MRI scan showing T1 isointensed, T2 hyperintensed anteriorly placed large (50 × 29 × 15 mm3) cystic lesion causing severe compression at the CVJ

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Figure 2: Intraoperative image showing the cyst is being removed

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Figure 3: Histopathological examination (H and E stain) showing pseudostratified cuboidal and columnar epithelium lining the cyst wall

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Figure 4: Follow.up MRI scan showing no residual cyst component at CVJ

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NEC is rarely found in CVJ. About half of the cases are associated with different congenital anomalies, although in our case no other congenital anomaly was found. NEC usually expands slowly, but may increase rapidly due to increased secretion, haemorrhage and inflammation.[2] In the present case, the trauma might have caused sudden increase in the compression and due to whiplash of the upper cervical cord it had acute deterioration. A large CVJ NEC can cause rapidly progressive neurological deficit including respiratory failure.[3]

MRI is the investigation of choice. They are usually intradural extra-axial lesion. Total excision is advisable. Subtotal excision carries potential risk of recurrence, holo-spinal dissemination, and malignant transformation.[4] Spillage of cyst contents into the subarachnoid space should be avoided during surgery to prevent meningism. Long term follow-up with serial MRI is recommended. Histologically, NECs are benign tumors. Typically they are lined by a simple epithelium of cuboidal to columnar cells with an underlying thin basement membrane. Goblet cells are often present. Immuno-histochemical studies are helpful when there is a diagnostic dilemma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient and her parents have given consent for her images and other clinical information to be reported in the journal. Patient understands that her name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Abe K, Oyama K, Mori K, Ishimaru S, Eguchi M, Maeda M. Neurenteric cyst of the craniocervical junction-case report. Neurol Med Chir 1999;39:875–80.  Back to cited text no. 1
Priamo FA, 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. 2
Sharma R, Chandramouli TC, 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.  Back to cited text no. 3
[PUBMED]  [Full text]  
Sahara Y, Nagasaka T, Takayasu M, Takagi T, Hata N, Yoshida J. Recurrence of a neurenteric cyst with malignant transformation in the foramen magnum after total resection. Case report. J Neurosurg 2001;95:341-5.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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