Intra-axial Neurenteric Cyst of Medulla: Case Report and Literature Review

Abstract

Neurenteric cysts (NCs) are rare congenital lesions that are thought to result from the persistence of the neurenteric canal connecting primitive gut and neural tube. Despite the congenital nature, NCs can be diagnosed at any age and at a similar frequency in women and men. To our knowledge, 140 intracranial NCs, confirmed by histology, including the patient presented in this review, have been reported since 1952. Parenchymal NCs are extremely rare, and there are no publications describing the intra-axial NCs of the brainstem at the moment.A 19-year-old female presented to the clinic with the following complaints: moderate dysphagia (two to three times per day) for and liquids and solids, dysphonia, vertigo, spontaneous nystagmus, imbalance, and numbness in the left side of the body, including the face. The magnetic resonance imaging (MRI) of the brain showed a well-defined lesion centered in the medulla. The patient underwent a small right-sided keyhole retro-sigmoid craniotomy. Just under the sulcus, a cyst containing pathological amorphous gray-yellow liquid was evacuated. Accessible parts of the capsule were resected without brain injury. Residual particles of the capsule were coagulated.Two months after the operation, the patient presented with similar symptoms. We used the previous craniotomy during the second surgery. After the evacuation of the cyst, a silicone stent was set for connecting with the cerebellopontine cistern and preventing new synechiae formation. As surely as after the first surgery, all neurological symptoms gradually regressed. In two months after surgery, deglutition and sensations recovered, and hemiparesis and imbalance decreased. Postoperative MRI examination two months after surgery showed no evidence of cyst recurrence.Intra-axial NCs are a rare group of congenital pathological lesions with a favorable prognosis. There are no publications of brainstem NCs with intra-axial localization to date. The treatment of choice in this group of patients is complete microsurgical excision followed by long-term observation.

Understanding the Pathogenesis of Lateral Supratentorial Neurenteric Cysts in Close Proximity to Other Vascular Pathologies: A Case Report and Review of Embryology

“…The extreme rarity of SNC renders them unlikely contenders on the list of differentials for frontal cysts [3]. NCs are more commonly found in the intradural extramedullary ventral aspect of the spinal cord [3], and intracranial NCs are typically located in the posterior fossa, fourth ventricle, or close to the brainstem [3][4][5]. Moreover, SNCs have variable-reported radiographic properties as their MRI signal intensity varies based on the cyst protein content [6].…”

Anterior Craniocervical Junctional Neurenteric Cyst

“…The major determinant for complete neurenteric cyst resection is whether the arachnoid plane between the tumor and the surrounding tissue is preserved. Subtotal cyst resection is an alternative technique used in patients with severe adhesion to prevent damage to adjacent neurovascular structures [ 10 ]. However, revision surgery may be required if neurenteric cyst recurrence occurs from the remnant of cyst wall [ 11 12 ].…”

Commentary: Endoscope-Assisted Resection of Extra-Axial Premedullary Neurenteric Cyst via Far Lateral-Supracondylar Approach: 2-Dimensional Operative Video

“…1 They are believed to arise from the endoderm layer because of an incomplete resorption of the neurenteric canal, a temporary connection between the yolk sac and amnion during the first stage of embryogenesis (third/fourth week of gestation), but other hypotheses include abnormal development or duplication of the notochord. 1,2 Until today, a total of 141 intracranial, histologically confirmed, NECs have been reported in the literature, including the case presented by the authors in this operative video article. 3 Given their benign, slow growing, or even no growing attitude, the actual incidence might be higher, but obviously, only symptomatic NECs are diagnosed and, usually, operated: The presented case, for example, remained stable for 14 yr, but then, given the location and, especially, the evidence of lesion enlargement on radiological imaging, the recommendation to undergo surgery was posed.…”