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Case 3. a Axial and b sagittal T1-weighted MRI showing a lesion located ventral to the medulla appearing homogeneously hyperintense. c Sagittal T2-weighted MRI showing the lesion to be hypointense
Source publication
Neurenteric cysts (NEC) are uncommon, benign, congenital lesions. Ventral foramen magnum (FM) location is very rare. The difficulties in diagnosis and management aspects are detailed with a review of the pertinent literature. We report four new cases of ventral FM NEC, all managed surgically and present a literature review of ventral FM NEC. A retr...
Citations
... Even though complete resection is preferable, adhesion to vital neurovascular structures can make it difficult in some cases. In these cases, a near total or subtotal excision may be sufficient [17]. In our case, NE was adherent to the wall of foramen magnum and but complete resection was possible. ...
... Far lateral approach is more frequently preferred than an anterior trans-oral approach. Cysts should be treated carefully to prevent cyst contents from spilling [17]. We followed the technique utilized by Wang et al. [18]. ...
Purpose: Primary brain tumors are the most frequent solid pediatric tumors, accounting for 40-50% of all cancers in children. Eighty to ninety percent of the 250,000 new cases of pediatric cancer each year are discovered in low and middle-income nations, where nearly 88 percent of the world's children reside. This article aims to emphasize the unusual presentation, management, and surgical outcome of complex pediatric brain tumors. Methods: We performed a retrospective study of patients who were admitted to the neurosurgery department with unusual pediatric brain tumors between March 1, 2019, and March 1, 2022. The study included pediatric patients up to age 18 years. We included those pediatric brain tumors whose (i) location was uncommon, or (ii) presented with unusual clinical presentation , or (iii) histopathology suggested to be a rare tumor, or (iv) radiological features were atypical.
Results: We included 9 cases of rare unusual pediatric brain tumors. Three out of 9 cases required preoperative emboliza-tion due to its hypervascular nature on digital subtraction angiography (DSA). All patients underwent surgical excision within 24-48 h of tumor devascularization. One out of 9 cases died in follow-up period due to pleural effusion and distant metastasis to lungs.
Conclusion: Treatment considerations for unusual pediatric brain tumors include a comprehensive multidisciplinary approach, including community-based screening and proper referral system for early treatment, a variety of treatment modalities, and sophisticated follow-up strategy. Government shall work in coherence with tertiary centers to spread social awareness and provide various financial scheme to prevent treatment dropouts.
... Although the prognosis is mostly favorable, one-third of patients experience a symptomatic recurrence in a period of 2 months to 32 years [36]. Minimum follow-up is recommended for 10 years, every 6 months at the first 2 years [35] and can be complemented with CA 19-9 measurement on cerebrospinal fluid to determine recurrence [37]. ...
This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.
... A CT scan is necessary to exclude congenital bony anomalies or bone malformation which is often observed with NCs. [19] Diffusion tensor imaging (DTI) tractography can be used for preoperative planning of cortical and subcortical brain tumor resection. It can also be used in some cases to help in the assessment of the origin of the cyst whether it is intra-or extra-axial. ...
Background
Incomplete resection of neurenteric cysts (NCs) has been associated with increased recurrence rates in patients compared to complete resection (CR) and information on intracranial NCs appearance on diagnostic imaging is scarce. We sought to identify factors associated with CR and provide the largest up-to-date review of NCs appearances on various diagnostic images.
Methods
Data from Medline, EMBASE, and Web of Science were extracted. Univariate and multivariate logistic regression models were used to analyze factors associated with CR.
Results
A total of 120 publications reporting 162 original cases on posterior fossa NCs met the inclusion criteria for analysis. Eighty-nine (55.6%) of the patients were female, the mean (SD) age of the patients’ during operation was 34.3 (16.9) years, and CR was achieved in 98 (60%) of patients. Univariate analysis identified male sex as a statistically significant predictor for complete reaction (OR 2.13, 95% Cl 1.10–4.11, P = 0.02). The retrosigmoid approach (OR 1.89, 95% Cl 0.98–3.63, P = 0.06), far lateral approach (OR 0.46, 95% Cl 0.21–1.02, P = 0.06), and pediatric patient (OR 2.45, 95% Cl 0.94–6.56, P = 0.07) may be possible predictors for CR, however, they were not statistically significant. NCs are mainly hypodense on CT (32 [61.5%]), varied greatly in intensity on T1WI, hyperintense on T2WI magnetic resonance imaging (98 [67.1%]), and hyperintense on fluid-attenuated inversion recovery (17 [63.0%]).
Conclusion
We recommend utilizing various diagnostic imaging tests to help reduce misdiagnoses when identifying intracranial NCs. For patient safety, CR should be achieved when possible, to reduce risk of additional operations due to recurrence.
... To date, only 80 cases of intracranial neurenteric cysts have been reported in the literature, and these cysts were located at the midline of the posterior fossa, cerebellopontine angle, fourth ventricle and brain stem [3]. Since only a few cases of neurenteric cyst at the anterior craniocervical junction have been reported, its treatment and prognosis remain unclear [4,5]. We present a case of neurenteric cyst at the anterior craniocervical junction that was surgically treated using the far-lateral transcondylar approach. ...
Intracranial neurenteric cyst at the anterior craniocervical junction is very rare, and its treatment and prognosis have not been established. We report a case of neurenteric cyst at the anterior craniocervical junction and review the relevant literature. A 16-year-old girl presented with a 2-month history of slowly progressive headache. MRI revealed a well-defined intradural extramedullary cyst in the anterior medulla and brain stem with C1 cord compression. We performed gross total resection of the cyst using a far-lateral transcondylar approach. Surgical resection is the treatment of choice for neurenteric cysts at anterior craniocervical junction, the far-lateral transcondylar approach might be the optimal surgical approach.
... It shows well-demarcated lesions iso-or tightly hypointense in T1 and hyperintense in T2-weighted sequences and FLAIR [5,6,8,17,18]. NCs usually demonstrate no contrast enhancement but Prasad, et al. reported mild posterior rim enhancement that can be explained by chronic inflammatory changes due to repeated cyst rupture in their view [19]. In our case MRI characteristics were not typical. ...
... It shows well-demarcated lesions, which are iso-or tightly hypointense on T1 and hyperintense on T2-weighted sequences and fluid attenuation inversion recovery (FLAIR) sequence [3,4,6,17]. NCs usually demonstrate no contrast enhancement, but Prasad et al. reported a mild posterior rim enhancement that, in his opinion, can be explained by chronic inflammatory changes due to repeated cyst rupture [18]. In our case, MRI characteristics were not typical. ...
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.
... Many types of intradural cysts have been described at the foramen magnum and atlantoaxial joint. Although rareonly 0.01% of central nervous system tumors-neurenteric cysts are among the best studied [3]. They are commonly located at the cervicothoracic junction and are associated with bony malformations, including scoliosis and spina bifida; when intracranial, they often appear in the posterior fossa [3]. ...
... Although rareonly 0.01% of central nervous system tumors-neurenteric cysts are among the best studied [3]. They are commonly located at the cervicothoracic junction and are associated with bony malformations, including scoliosis and spina bifida; when intracranial, they often appear in the posterior fossa [3]. Neurenteric cysts are postulated to derive from a persistent accessory canal that forms secondary to aberrant duplication of the neurenteric canal during caudal regression of the primitive streak [4]. ...
... Neurenteric cysts are ciliated columnar epithelium, recapitulating respiratory or gastrointestinal epithelium. They therefore reliably stain positive for epithelial membrane antigen (EMA), cytokeratin, carcinoembryonic antigen (CEA) and CA 19-9 but negative for glial fibrillary acidic protein (GFAP) and S-100 [3]. ...
Extradural atlantoaxial cysts are typically related to C1-2 degeneration. Intradural cysts may cause secondary syringobulbia depending on the size and cerebrospinal fluid flow obstruction. However, medullary syrinxes have not been previously described with extradural cysts. Treatment of symptomatic lesions involves surgical resection, often via a far-lateral approach, with consideration of fusion if C1-2 instability is present. We present a case of an extradural C1-2 cyst with intradural extension causing syringobulbia. Effective surgical resection was accomplished via a far-lateral, partial transcondylar approach without fusion. It is important to recognize that cysts of extradural origin may exhibit intradural extension and compress critical neurovascular structures.
... The treatment of choice for neurenteric cysts is complete surgical resection when feasible [14,23]. In certain cases complete removal of the cyst may be hindered by adherence to brain parenchyma or traversing cisternal vasculature and cranial nerves, in which case subtotal resection is considered to be a reasonable alternative to avoid injury to these structures [14,[23][24][25][26]. Following treatment, serial imaging has been recommended by several authors to monitor for recurrence/interval growth or rarely malignant transformation [5,7,14,17,20,21]. ...
We present a case of a 57-year-old female with four-months of diplopia and vertigo. MRI revealed a mixed cystic and solid partially enhancing lesion of the 4th ventricle, foramen of Luschka and cerebellopontine angle. Preoperative differential diagnosis favored ependymoma. Biopsy revealed a neurenteric cyst, a benign developmental lesion that rarely occurs intracranially. This case highlights several atypical manifestations of intracranial neurenteric cyst, with regions of histologically benign regions of solid enhancement, multicompartmental extra-axial location mimicking an ependymoma, and rapid recurrence without evidence of underlying malignancy.
... Foramen magnum (FM) is a typical area in the posterior fossa where tumors frequently have an intimate relationship with vertebral artery and sometimes basilar artery as well [8]. Vertebrobasilar artery can be displaced, narrowed or even encased by tumors in the FM [9,10]. Moreover, mechanical manipulation of the vertebrobasilar artery may go through the whole surgical procedure [11,12]. ...
Objective:
Cerebral vasospasm can occur after skull base tumor removal. Few studies concentrated on the posterior circulation vasospasm after tumor resection in the posterior fossa. We aimed to identify the risk factors associated with postoperative vertebrobasilar vasospasm after tumor resection in the foramen magnum.
Methods:
We retrospectively reviewed the data of 62 patients with tumors in the foramen magnum at our institution from January 2010 to January 2015. The demographic data, tumor features, surgical characteristics were collected. Vertebrobasilar vasospasm was evaluated by bedside transcranial Doppler before surgery and on postoperative day 1, 3, 7. Univariate and multivariate analyses were performed to determine the predictors of postoperative vasospasm in the posterior circulation.
Results:
Vertebrobasilar vasospasm was detected in 28 (53.8%) of the 62 patients at a mean time of 3.5 days after surgery. There were 5 (8%) patients with severe vasospasm according to the grading criteria. Age, tumor type, tumor size, vertebral artery encasement, and surgical time were significantly related to vasospasm in the univariate analysis. Further multivariate analysis demonstrated that only age and vertebral artery encasement were independent risk factors predicting the occurrence of postoperative vertebrobasilar vasospasm.
Conclusions:
The incidence of acute vertebrobasilar vasospasm is not uncommon after foramen magnum tumor resection. Age and vertebral artery encasement are significantly correlated with postoperative vasospasm. Close monitoring of vasospasm should be given to patients with younger age and the presence of vertebral artery encasement on the preoperative imaging to facilitate early diagnosis and intervention.
... About 160 cases of intracranial neurenteric cyst have reported in the literature [1][2][3][4][5], over 75 % of which are in the posterior fossa, including inside the fourth ventricle and at the craniocervical junction [3,6]. These cysts are mostly extra-axial though they may compress and distort the brainstem. ...
