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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 1 | Page : 206-208
Large Neuroenteric Cyst at Cranio-Vertebral Junction with Cardiorespiratory Arrest
Laxmi Narayan Tripathy, Indrajit Rana, Harsh Jain
Department of Neurosurgery, Medica Superspecialty Hospital, Kolkata, West Bengal, India
|Date of Submission||20-Apr-2019|
|Date of Decision||22-May-2019|
|Date of Acceptance||27-May-2019|
|Date of Web Publication||24-Feb-2021|
Medica Superspecialty Hospital, 127 Eastern Metropolitan Bypass Mukundapur, Kolkata - 700 099, West Bengal
Source of Support: None, Conflict of Interest: None
|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
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. 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 3: Histopathological examination (H and E stain) showing pseudostratified cuboidal and columnar epithelium lining the cyst wall|
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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. 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.
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. 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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]