|Year : 2014 | Volume
| Issue : 3 | Page : 373-376
A case report of craniovertebral junction intradural extramedullary neurenteric cyst
Rajeshwari S Vhora1, Sanjay S Vhora2, Anand D Katkar3, Prajakta S Ghate3
1 Department of Emergency Medicine, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Neurosurgery, Byramjee Jeejeebhoy Medical College and Sassoon General Hospital, Pune, Maharashtra, India
3 Department of Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India
|Date of Web Publication||18-Mar-2014|
Rajeshwari S Vhora
D3 Clover Garden, 4 Naylor Road, Off Mangaldas Road, Pune - 411 001, Maharashtra
Source of Support: None, Conflict of Interest: None
A neurenteric cyst of the craniocervical (CV) junction, as a cause of bulbomedullary compression, is very rare. An abnormal communication between the endoderm and neuroectoderm during the third week of embryogenesis may be responsible for its formation. It is a rare spinal condition. The most frequent location is at the lower cervical and higher thoracic spine. Neurenteric cysts of the craniocervical junction are even rarer. We report the case of a CV junction intradural neurenteric cyst.
Magnetic Resonance Imaging (MRI) of our patient demonstrated an intradural extramedullary process of the craniocervical junction. A surgical posterior approach allowed gross total resection of the lesion. The histopathology of the surgical specimen showed that the cyst wall was made up of fibrocollagen walls lined with a partially ciliated columnar epithelium.
Keywords: Craniovertebral junction, extramedullary, intradural, neurenteric cyst enterogenous cyst, endodermic cyst, endoderm, neuroectoderm
|How to cite this article:|
Vhora RS, Vhora SS, Katkar AD, Ghate PS. A case report of craniovertebral junction intradural extramedullary neurenteric cyst. Med J DY Patil Univ 2014;7:373-6
|How to cite this URL:|
Vhora RS, Vhora SS, Katkar AD, Ghate PS. A case report of craniovertebral junction intradural extramedullary neurenteric cyst. Med J DY Patil Univ [serial online] 2014 [cited 2019 Nov 19];7:373-6. Available from: http://www.mjdrdypu.org/text.asp?2014/7/3/373/128989
| Introduction|| |
A neurenteric cyst (NEC), also called an enterogenous cyst or endodermic cyst, is a benign congenital condition. It results from the persistence of an abnormal communication between the endoderm and neuroectoderm in the third week of the life of an embryo. This malformation is encircled by a mucosal secreting epithelioma mimicking the normal gastrointestinal epithelioma tractus. , According to the World Health Organisation Organisation mondiale de la santé [WHO OMS] classification, this tumor belongs to the Central Nervous System (CNS) malformative tumors. Only 114 cases are reported so far in the literature. , The most frequent localization is at the lower cervical and higher thoracic spine. , Neurenteric cysts of the craniocervical junction are even rarer and only 15 cases have been reported. ,,,,,,,,,,,,
Recurrence occurs particularly after subtotal excision or a cyst fenestration, carrying a potential risk of malignant transformation. ,,,,,
Total excision should be the therapy of choice, when possible. ,, Sometimes, adhesion to important vascular or neurological structures may hinder gross total resection. In this situation, the cyst's content should be carefully evacuated and the cystic wall should be partly removed, providing a good outcome, with minimal invasiveness. , The suboccipital approach, sometimes associated with laminectomy, is the classical option to approach this craniocervical junction lesion. 
| Case Report|| |
A 32-year-old male was admitted with a history of occipital headache and neck pain since 15-20 days. His neurological examination was normal.
On magnetic resonance imaging (MRI), a round cystic lesion in the craniocervical junction extending from the lower clivus extremity to the C2 level was visualized. The lesion signal was isointense on T1-weighted images and hyperintense on T2-weighted images, with no enhancement after contrast. This intradural extra axial mass was located ventrally to the medulla oblongata and was visualized to the left lateral side of iso-intense cervicomedullary junction, measuring 42 × 12 mm, with neither bony nor soft tissue abnormalities [Figure 1].
|Figure 1: MRI Scan of the CV Junction (P + C) showing a cystic lesion at the cervicomedullary junction|
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The patient was operated in a prone position through a posterior approach. A suboccipital craniectomy associated with C1 laminectomy was carried out. There was occult spina bifida, as the posterior arch of the Atlas More Details was not fused. An anterior intradural extramedullary yellow cyst with cheesy white content was seen when the dura matter was opened. It was compressing the cervicomedullary junction and had pushed it to the right side [Figure 2]. The lesion was adherent to the surrounding structures, but it could be easily separated. However, it was attached firmly to the cord. Surgical excision was in piecemeal.and finally complete excision was done On pathological examination, the cyst membrane was lined with a single layer of cuboidal to columnar cells with apical mucin [Figure 3] and [Figure 4].
|Figure 2: Intraoperative photograph showing the cyst (yellowish) compressing the bulbo-medullary junction|
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|Figure 3: H and E showing the cyst wall lined with cuboidal epithelium (200X)|
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|Figure 4: Pathology section showing cyst wall lined with cuboidal epithelium (200X)|
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The patient had an uneventful postoperative period.
| Discussion|| |
Neurenteric cysts are congenital abnormalities belonging to notochord dysraphies, dysraphic disorders which are due to an abnormal adherence between the ectoderm and endoderm. It is suggested that failure during the embryogenic phase may be responsible for their formation. ,, The pathogenesis of this lesion is not clarified. However, the most convincing hypothesis is a dysgenesis of the endoderm from the neuroectoderm in the third week of fetal development. , The adherence between the endoderm and ectoderm explains the frequent association between the neurenteric cyst (NEC) and the mesoderm abnormality - like spina bifida and Arnold Chiari malformation More Details. 
These cysts are seen in all ages, with the average age around the third and fourth decade, with a male predominance (sex ratio = 2),  as in our patient.
They are usually intradural extra-axial lesions. There was only one case of intramedullary location described.  The neurenteric cysts of the posterior fossa are extremely rare, with only 10 reported cases. ,,,,, Neurenteric cysts of the craniocervical junction are also infrequent, with no more than 15 reported cases. The most frequent initial symptom is neck pain or occipital headache (10 patients among 15), as it was seen in our patient, sometimes accompanied by neck stiffness. Motor weakness and ataxia are the most frequent neurological signs (eight among 15 patients).
Lower cranial nerve paresis is found in four patients. ,,,,,,,,,,,, Aseptic meningitis resulting from cyst rupture in the subarachnoid space is reported in two cases. 
Computed tomography and T1-weighted MRI usually demonstrate a well-defined, non-calcified, hypodense or hyperintense lesion, with no contrast enhancement. On T2-weighted images, the hyperintense cystic lesion without edema is generally observed. ,,
Immunocytochemistry is the key point of diagnosis, as it demonstrates the endodermic origin of the lesion. The neurenteric cyst shows epithelial membrane antigen, cytokeratine, and a carcinoembryonic antigen. In contrast, gliofibrillar acid protein, neuronal specific enolase, Vimentine, and S100 antibodies are not expressed ,,
Recurrence after a total removal, as was first described by Markwalder and Zimmerman, in 1979, occurs particularly after subtotal excision or cyst fenestration, carrying a potential risk of malignant transformation. ,,,,,
An unusual case of holospinal dissemination of multiple NEC cysts occurring after fenestration of a fourth ventricle's NEC is also described.  Total excision should be the therapy of choice when possible. ,, Sometimes, adhesion to important vascular or neurological structures may hinder gross total resection. In this situation, the cyst's content should be carefully evacuated and the cystic wall should be partly removed, providing a good outcome, with minimal invasion.  The suboccipital approach, sometimes associated with laminectomy, is the classical option to approach this craniocervical junction lesion.  We carried out a similar approach for our patient and also carried out total excision of the cyst.
Another described approach is the lateral trans-condylian approach with the occipital condyle and jugular tubercle translation, providing a direct vision of the clivus and the atlanto-occipital junction.  Some authors also propose the transoral approach when cysts are located in the midline and attached to the anterior surface of the brainstem. Nevertheless, the transoral approach provides a limited operative field and carries the risk of postoperative infection. 
| Conclusion|| |
Craniovertebral Junction (CVJ) intradural neurenteric cyst (NEC) is very rare. It is important to carry out total excision, as recurrence occurs, particularly after subtotal excision or cyst fenestration, carrying a potential risk of malignant transformation.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]