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OBJECTIVE: To compare cases of dysraphism with ventral tethering of cord with those with dorsal tethering and to find out any differences in the outcome of surgery in them. METHODS: We collected the data of 188 consecutively operated tethered cord patients at our institute in the past 7 years and divided them into ventral tethering and dorsal tethering groups. Those that we felt had both dorsal and ventral tethering were excluded. Their preoperative clinical, radiological, and baseline neurophysiological parameters as well as postoperative clinical and radiological parameters were analyzed in a retrospective study. RESULTS: Among the 188 tethered cord patients, 52 (28%) had ventral tethering and 136 (72%) had posterior tethering. Preoperative neurodeficit and cord signal changes as well as absent baseline MEP (of any one muscle) were significantly more associated with ventral tethered cord than the dorsal tethered cord. The neurological deterioration after surgery occurred significantly in the ventral tethered cord group than in the dorsal tethered cord group. Also, the postoperative MRI had more incomplete detethering cases in the ventral group than in the dorsal tethered cord group. CONCLUSION: Ventral tethered cord is more likely to present with preoperatively neurological deficits. It should be carefully identified in the preoperative MRI, so that the intraoperative difficulties in complete detethering and postoperative deterioration can be anticipated.

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Background Neuroenteric cysts (NECs) are benign lesions mostly found as intradural extramedullary lesions in the cervicothoracic spinal cord. NECs in the cavernous sinus are very rare. To the best of our knowledge, this is only the second reported case and the first in an adult. Presentation We present a left cavernous sinus NEC in a 75-year-old female with gradually worsening headache and facial pain unresponsive to medical treatment. Imaging revealed a cystic mass lesion in the left cavernous sinus encasing the distal petrosal and cavernous segment of the internal carotid artery. Initial differential diagnoses included more common pathologies located near the cavernous sinus, including cystic schwannoma, craniopharyngioma, and dermoid and epidermoid tumors. The patient underwent a left pterional craniotomy with an extradural transcavernous approach for surgical exploration and possible resection of this mass lesion. Histopathology revealed an NEC lined with benign respiratory-type epithelium. Postoperative imaging revealed gross total tumor resection. The patient remained neurologically intact with complete resolution of facial pain. Conclusion We present a rare pathology that can easily be misinterpreted as other types of lesions. NECs should be kept in mind for differential diagnosis of cavernous sinus cystic lesions. The surgical aim should be maximal safe excision.

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Neurenteric cysts (NCs) are rare benign endodermal lesions of the central nervous system (CNS), most commonly found in the spinal cord. Intracranial lesions are rare, among which the posterior fossa appears to be the predominant location. We present a case of a 60-year-old man who presented with a suddenly decreased level of consciousness. After a series of radiological studies were done, a multilobulated cystic lesion in the right posterior fossa was observed. Surgical resection was performed and based on its histopathological characteristics, NC diagnosis was confirmed. Because of the wide list of differential diagnoses and low specificity of radiological features, surgical gross total resection remains the most effective treatment, followed by diagnosis confirmation through histopathological techniques.

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Mediastinal Neuroenteric Cyst is a rare congenital presentation in infants and is associated with a high mortality rate. It is a very uncommon benign lesion and usually develops from abnormal embryological development of the foregut. Till now, only 106 cases have been reported worldwide. In Pakistan only three cases have been published, with varying presentations. The clinical presentation and age at presentation vary from asymptomatic and coincidental finding on chest x-ray, to limb numbness or early presentation with severe symptoms like those in our case. In fact, it poses an important challenge for paediatricians. We present a rare case with emphasis on clinical presentation and diagnostic criteria.

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INTRODUCTION: Split cord malformations (SCMs) are developmental anomalies that are associated with a number of congenital defects. However, a combination of SCM I with a neuroenteric cyst (NEC) is extremely rare, and only 11 cases have been described in the literature. To the best of authors' knowledge, the combination of the above two with dermoid cyst and thickened filum terminale has never been reported in the literature. CASE PRESENTATION: We present a case of the above combination in a 2-year-old child who underwent microsurgical excision of all 4 pathologies and complete recovery. CONCLUSION: NEC and dermoid should be considered in the differential diagnosis when imaging reveals cystic pathology along with SCM. Expeditious surgical repair resulted in an outstanding functional outcome at 1-year follow-up.

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We present the case of a 42-year-old woman with a 5-week history of headache, progressive dysphagia, dysphonia, and hoarseness. A brain magnetic resonance imaging revealed an extra-axial cyst of the left lateral cerebellomedullary cistern. The subsequent histopathologic examination diagnosed a neuroenteric cyst. This case sheds light on the pivotal role of histologic identification of neuroenteric cyst, which could be crucial for further diagnostic investigations, especially in pediatric patients.

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CONTEXT: Endodermal cysts are rare benign developmental cysts lined by mucin-secreting and/or ciliated, cuboidal, or columnar epithelium of probably endodermal origin. AIMS: Endodermal cysts are rarely intracranial, frequently located in the posterior fossa. Supratentorial location is the most infrequent and only few cases are reported in the literature, included our case. SETTINGS AND DESIGN: The authors report a case of intracranial supratentorial endodermal cyst with a review of the literature. SUBJECTS AND METHODS: A 40-year-old woman was admitted to our department because of progressive gait disorder for 3 months due to right brachial and crural motor deficit associated to right crural sensory disorder (tactile hypesthesia) and right Babinski response at neurological examination due to an endodermal cyst located in the left frontoparietal convexity. DISCUSSION: Total resection of endodermal cysts is recommended, despite their location and adhesion to the surrounding structures, due to its high risk of recurrence. Fenestration of the cystic content into the subarachnoid cistern may cause obstructive hydrocephalus or chemical meningism. RESULTS: Although rare, surgeons should be aware that these lesions must be differentiated clinically, radiologically, and histologically from other supratentorial cystic lesions.

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This operative video highlights a rare case of a neuroenteric cyst at the ventral craniocervical junction. The case involved a 30-year-old man who initially presented 13 years earlier with acute onset of headache and visual changes. At that time, he was found to have a small, enhancing ventral intradural extramedullary mass at the rostral aspect of C1 thought to be a meningioma. The lesion was managed conservatively, and surveillance imaging tracked its slow progressive enlargement to a size of 1.4 cm ( Fig. 1A, B ). Although he remained asymptomatic, nonurgent elective resection was recommended because of his age and mass progression. The patient underwent a left far lateral approach to the craniocervical junction for resection of the mass. This involved dissection of the suboccipital musculature to expose the C1 transverse process in the suboccipital triangle and ultimately the vertebral artery. After a small craniectomy and C1 hemilaminectomy, the dura was opened and a cystic lesion encountered ( Fig. 2 ). The cystic contents were debulked and the capsule resected. Histopathologic examination revealed abundant goblet cells consistent with a neuroenteric cyst. Dural closure was bolstered with fascia lata and autologous fat graft. Postoperative magnetic resonance imaging (MRI) was consistent with gross total resection ( Fig. 1C, D ). The patient tolerated the procedure well with no new postoperative neurological deficits and was discharged home on postoperative day 2. On completing a 3-day decadron taper, he developed steroid-responsive symptoms consistent with aseptic meningitis, possibly related to cerebrospinal fluid contamination with the cyst contents during resection. The link to the video can be found at: https://youtu.be/SskETPe5PXQ .

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Neurenteric cysts are rare developmental lesions typically found outside the central nervous system but when they do, we most likely find them in a spinal (cervical or dorsal) intradural extramedular location, often associated with dysraphism. The more unusual intracranial cases have been published because of its rarity, occurring mostly as a posterior fossa extra-axial cyst, in adults. Supratentorial cases are distinctly infrequent, especially in children, resulting in few case reports and even fewer case reviews. We describe a case of a child with a supratentorial neurenteric cyst and present a brief review of the literature about these cysts in children, a noticeable gap in the literature.

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BACKGROUND: Neuroenteric cysts are rare developmental anomalies of the central nervous system that account for approximately 0.7%-1.3% of all spinal cord lesions. The majority of spinal neuroenteric cysts are located ventral to the spinal cord. Dorsally located neuroenteric cysts are rare and are almost always associated with other spinal anomalies. Isolated dorsal spinal neuroenteric cysts, without other associated spinal anomalies, are extremely rare. These lesions can be mistaken for the more commonly encountered spinal cysts (e.g., arachnoid cysts, ependymal cysts, cystic teratomas). CASE DESCRIPTION: We present the first pediatric case of a 9-month-old boy with an isolated dorsal thoracic neuroenteric cyst and cord compression that underwent surgical resection. The patient tolerated the procedure well and remained neurologically stable after surgery. Pathology was consistent with a neuroenteric cyst. Complete resection was not achieved at the time of surgery. In retrospect, knowing the lesion is a neuroenteric cyst, a more aggressive approach aiming for a complete surgical resection should have been undertaken. To our knowledge, only 2 adult cases of isolated dorsal spinal neuroenteric cysts have been reported in the literature. CONCLUSIONS: Neuroenteric cysts can occur dorsal to the spinal cord without any associated spinal anomalies. The gold standard treatment for neuroenteric cysts is surgical resection, and the goal of surgery is complete resection.

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INTRODUCTION: Neuroenteric cysts are rare benign endodermal lesions of the central nervous system that result from incomplete resorption of neuroenteric canal and mostly found in cervical and upper thoracic spinal canal. Intracranial neuroenteric cysts are extra axial and commonly located anteriorly in the posterior cranial fossa. MRI demonstrates variable intensity within the lesion on T1, T2W, and DWI sequences. METHODS: Three cases of posterior fossa non-enhancing cystic lesions of variable signal intensity underwent MRI with MR spectroscopy, where MR spectroscopy demonstrated dominant peak at 2 ppm, mimicking normal Nacetyl aspartate (NAA). CONCLUSION: MR spectroscopy in addition to conventional MRI may help in differentiating intracranial neuroenteric cyst from its close differentials.

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The authors report the case of a mobile spinal enterogenous cyst in a 2-year-old boy, who was admitted to the hospital several times for intermittent paraplegia. Magnetic resonance imaging and CT revealed an isolated cyst in the lumbar spinal canal. The symptoms were caused by transient myelopathy of the conus medullaris and radiculopathy of the cauda equina due to the changing size and location of the cyst. The cyst was surgically extirpated, after which the symptoms resolved. The histopathological diagnosis was enterogenous cyst. The clinical history of intraspinal enterogenous cyst is usually progressive. Mobility and changes in size are rare pathophysiological findings. The authors speculate that the cyst wall did not adhere to the surrounding structures and had ruptured and quickly reformed. Enterogenous cyst should be considered in the differential diagnosis of spinal intradural cysts in children with radiculomyelopathy.

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Objective Modern surgical experience with intracranial neuroenteric cysts is limited in the literature. We review our 15-year institutional experience with these rare lesions. Design Single-institution retrospective study. Setting Large North American tertiary care center. Participants Histologically confirmed cases of intracranial neuroenteric cyst from January 2000 to September 2014. Main Outcome Measures Pre- and postoperative modified Rankin Scale (mRS) scores, extent of resection, and postoperative complications are reported. Clinical presentation, imaging features, pathology, and operative approach are discussed. Results Five spinal and six intracranial neuroenteric cysts were surgically treated over a 15-year period. Median age at presentation for the intracranial cysts was 38.5 years. Mean cyst diameter was 3.8 cm. Five cysts were located in the pre-pontomedullary cistern, and one was located in the third ventricle. Gross total resection was achieved in four of the five posterior fossa cysts through a far lateral transcondylar approach. Postoperative complications included aseptic meningitis (one), transient abducens palsy (one), and pseudomeningocele requiring reoperation (three). Postoperative mRS scores improved to ≤1 by 6.5 months median follow-up. Conclusions Intracranial neuroenteric cysts are rare lesions with a variable imaging appearance. Complete surgical resection through a far lateral transcondylar approach is possible and usually results in symptom improvement or resolution.

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A 50-year-old male presented with acutely progressed paraplegia. His magnetic resonance imaging demonstrated two well-demarcated components with opposite signals in one cystic lesion between the T1- and T2-weighted images at the T1 spine level. The patient showed immediately improved neurological symptoms after surgical intervention and the histopathological exam was compatible with a neurenteric cyst. On operation, two different viscous drainages from the cyst were confirmed. A unique similarity of image findings was found from a review of the pertinent literature. The common findings of spinal neurenteric cyst include an isointense or mildly hyperintense signal relative to cerebrospinal fluid for both T1- and T2-weighted images. However, albeit rarer, the signals of some part of the cyst could change into brightly hyperintensity on T1-weighted images and hypointensity on T2-weighted images due to the differing sedimentation of the more viscous contents in the cyst.

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Neuroenteric cysts (NC) are benign, congenital malformation which are of endodermal origin commonly located in the central nervous system. We report a case of intracranial NC with squamous metaplasia and xanthogranulomatous response masquerading as a white epidermoid on conventional MRI sequences. Lesion showed two components on T2W-images. We observed differential diffusion characteristics including fractional anisotropy, radial diffusivity and axial diffusivity within the two components of the lesion.

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A 50-year-old male presented with acutely progressed paraplegia. His magnetic resonance imaging demonstrated two well-demarcated components with opposite signals in one cystic lesion between the T1- and T2-weighted images at the T1 spine level. The patient showed immediately improved neurological symptoms after surgical intervention and the histopathological exam was compatible with a neurenteric cyst. On operation, two different viscous drainages from the cyst were confirmed. A unique similarity of image findings was found from a review of the pertinent literature. The common findings of spinal neurenteric cyst include an isointense or mildly hyperintense signal relative to cerebrospinal fluid for both T1- and T2-weighted images. However, albeit rarer, the signals of some part of the cyst could change into brightly hyperintensity on T1-weighted images and hypointensity on T2-weighted images due to the differing sedimentation of the more viscous contents in the cyst.

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Background A retroclival lesion can represent a notochordal remnant-derived mass. The differential diagnoses includes benign lesions such as ecchordosis physaliphora (EP) and neuroenteric cyst or malignant ones such as chordomas. In the case of EP and chordoma, although both types arise from remnants of fetal notochord tissues, they represent two separate entities with different radiographic and biologic behaviors. Case Description We present a case of an incidental finding of a retroclival lesion. The magnetic resonance imaging (MRI) characteristics of the lesion match the neuroimaging profile of a benign lesion and are suggestive of an EP. There was no enhancement noted with the addition of gadolinium. Nonetheless, pathology determined the lesion to be a malignant chordoma. Conclusion The differential diagnosis of a retroclival lesion includes benign and malignant notochordal lesions. Here we present a case of a patient with an incidental finding of a retroclival lesion. Radiographic findings were suggestive of a benign lesion, possibly EP, yet the pathology revealed a chordoma. This report suggests that despite benign imaging, chordoma cannot be excluded and the implications for treatment can be significant. It is important to achieve the correct diagnosis because the prognostic and therapeutic implications are different.

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Neuroenteric cysts are benign intradural endoderm cysts lined by gastrointestinal (GI) or tracheobronchial epithelial cells. Their malignant transformation is extremely rare and only six cases have been reported. In these cases, tissue lineage of the cystic endoderm cells giving rise to carcinoma was not clearly identified either as respiratory or as GI type. Herein, we report a case of mucinous adenocarcinoma arising from the neuroenteric cyst with broncho-pulmonary differentiation in the right cerebral hemisphere of a Japanese woman in her late 50s. The cyst wall was entirely lined by the following respiratory epithelial components: stratified bronchial ciliated columnar epithelium with basal cells positive for CK5 and p63, terminal bronchiolar Clara cells positive for thyroid transcription factor (TTF)-1, surfactant B and negative for surfactant C, type I pneumocytes positive for TTF-1, negative for surfactant B and C, and type II pneumocytes positive for TTF-1 and surfactant B and C. In addition, nests of hyperplastic single layered mucinous epithelial cells with bronchial goblet cell phenotype were also demonstrated, and histologic features were almost the same as the pulmonary type I congenital adenomatoid malformation (CCAM) with mucinous epithelial proliferation. The mucinous epithelial nests of type I CCAM are liable to develop mucinous adenocarcinoma and frequently accompany K-ras mutation and expression of p16. However, K-ras mutation and p-16 expression were not detected in this case.

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Neuroenteric cysts are developmental cysts that should be differentiated from other, more common non-neoplastic cysts as well as cystic neoplasms. While these lesions may have varied imaging findings, T1 hyperintense prepontine lesion due to mucinous/proteinaceous content is characteristic. Location and imaging characteristics aid in formulating the correct diagnosis of neuroepithelial/neuroenteric cysts. Magnetic resonance spectroscopy (MRS) has the specific finding of N-Acetyl Aspartate (NAA)-like peak at 2.02 ppm which is not seen in other cystic lesions. In this study, we aim to discuss the imaging findings of these lesions on conventional and advanced MRI findings and provide radiologic-pathologic correlation. We also briefly describe the pathogenesis, embryology and radiological differential diagnoses of these cysts.

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Neuroenteric cyst derives from endodermal tissue displaced dorsally into the spinal canal ventral to the neural plaque through the interposed mesodermal layer that forms the vertebral bodies, embryologically. It is located along the alimentary canal, or ventral to the spinal cord in the cervical, thoracic area, or at the cervicothoracic junction within the boundaries of C3 above and T7 below and lies in an intramedullary or intradural extramedullary location in most patients. To date, retroperitoneal neuroenteric cyst has been described in only few cases. We report a case of asymptomatic retroperitoneal neuroenteric cyst in a 28-year-old man with a brief review of literatures, which found incidentally on health care center.

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