Intracranial neurenteric cyst: A rare cause of chemical meningitis

Intracranial neurenteric cysts are exceedingly rare congenital intracranial lesions that result from disorder of gastrulation. Still, more rarely, the cyst contents may leak into the CSF and give rise to recurrent episodes of chemical meningitis. We present a case of chemical meningitis due to a leaking posterior fossa neurenteric cyst in a young female, with emphasis on its imaging features. The final diagnosis was achieved by sufficiently characteristic imaging features; histopathologic documentation could not be achieved as the patient denied surgery.     

Cranio-spinal enterogenous cysts: clinico-radiological analysis in a series of ten cases.

Enterogenous cysts are rare congenital lesions resulting from dysgenesis during the third embryonic week at the time of notochord development and the transitory existence of the neurenteric canal. Enterogenous cysts are usually lined by mucus secreting epithelium resembling that of the gastrointestinal tract. The inclusion of the endodermal elements in the spinal canal may produce extradural, intradural extramedullary or intradural intramedullary cystic lesions. The endodermal inclusions producing cysts in the intracranial compartment are extremely rare, especially in the supratentorial region. Plain X-rays may show concomitant cranio-spinal anomalies. However, CT and MRI scans show better definition and characterisation. Total excision is the procedure of choice in these benign cysts with favourable long term prognosis. Approximately 100 histologically proven cases have been described, with a few exceptions, as isolated case reports the authors report a detailed analysis of the clinico-radiological aspects of a series of ten patients with cranio-spinal enterogenous cysts with a review of the literature.     

Intraparenchymal supratentorial neurenteric cyst

BACKGROUND: Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma. METHODS: A patient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature. CASE REPORT: A 35-year-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved. CONCLUSION: Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.     

Intracranial neuroenteric cysts: a concise review including an illustrative patient

Neuroenteric cysts (NC) are rare, benign lesions lined by mucin-secreting cuboidal or columnar epithelium of an intestinal or respiratory type. They are regarded as ectopic endodermal cysts, and tend to be found in the spine rather than an intracranial location. Advances in neuroimaging have led to an increased frequency of diagnosis of NC, especially as an incidental finding, although such cysts may be confused radiologically with other lesions such as epidermoid and arachnoid cysts. We undertook a PubMed search of the literature using the search terms "neuroenteric cyst" and its many pseudonyms, including "endodermal cyst", "enterogenous cyst", "neurenteric cyst", "epithelial cyst", "intestinome", "teratomatous cyst", "gastrocytoma", and also "enterogenic", "foregut", "respiratory", and "bronchogenic cyst". Only reports in English and those containing histopathologically-confirmed NC were considered for this review. In total, 140 patients with intracranial NC were found, including the patient reported in the present review. This review describes the classification, epidemiology, embryology, clinical presentation, radiology, histopathology, and surgical treatment of NC, and includes an illustrative patient.     

Intramedullary neurenteric cyst of the conus medullaris without associated spinal malformation: a case report and review of the literature

Spinal neurenteric (NE) cysts are rare congenital anomalies that may occur either alone or in the context of a complex malformative disorder. They are usually intradural-extramedullary lesions. Intramedullary NE cysts not associated with other congenital anomalies are very rare and only a few cases have been reported in the conus medullaris region. Intramedullary neurenteric cysts not associated with other spinal anomalies are very rare especially in the conus medullaris region. MRI is useful to define the cyst and the osseous anomalies associated with this lesion. The goal of treatment of an intramedullary neurenteric cyst is total excision at the first operation, if possible. Life-long follow-up with annual MRI is recommended due to the risk of cyst recurrence. We report an intramedullary NE cyst of the conus medullaris without associated malformation and the relevant literature is briefly reviewed.     

An endodermal cyst mimicking an intra-axial tumor in the medulla oblongata

Introduction  

Endodermal cysts, also known as enterogenous, neurenteric, foregut, epithelial, bronchogenic, or respiratory cysts, are rare benign lesions lined by columnar epithelium of a presumed endodermal origin. Endodermal cysts are rarely intracranial, but when this occurs, the cysts are frequently located in the posterior fossa with the most common locations being anterior to the brainstem and within the fourth ventricle. However, an endodermal cyst located in the medulla oblongata is extremely rare.     

Solitary Cervical Neurenteric Cyst in an Adolescent Patient

Spinal neurenteric cysts are uncommon congenital lesions, furthermore solitary neurenteric cysts of the upper cervical spine are very rare. A 15-year-old boy having an intraspinal neurenteric cyst located at cervical spine presented with symptoms of neck pain and both shoulders pain for 2 months. Cervical spine magnetic resonance (MR) imaging demonstrated an intradural extramedullary cystic mass at the C1-3 level without enhancement after gadolinium injection. There was no associated malformation on the MR imaging, computed tomography, and radiography. Hemilaminectomy at the C1-3 levels was performed and the lesion was completely removed through a posterior approach. Histological examination showed the cystic wall lined with ciliated pseudostratified columnar epithelium containing mucinous contents. Neurenteric cyst should be considered in the diagnosis of spinal solitary cystic mass.     

Differential diagnosis and origin of epithelial cysts in the central nervous system--report of seven cases and review of literature

Seven cases of epithelial cysts are presented with special reference to histological findings. Differential diagnosis and origin of the cysts are also discussed. Two are autopsy cases and 5 are surgical cases. Median age of the patients is 41 years. Three cysts are in the posterior fossa, 1 in the supratentorial region, 1 in both infra- and supratentorial regions and 2 in the spinal canal. On light microscopy, the type of cell lining the cyst wall and the presence of cilia and PAS-positive cells are studied. All cyst walls were lined by a single layer of cuboidal to columnar epithelium. Cilia was seen in 1 and PAS-positive cells were found in 5 out of 7 cases. On electron microscopy of the 4 cases available for study, continuous basement membrane and microvilli were observed in all cases. Coating material covering microvilli was noted in 2 cases. According to these histological findings, these cysts are classified as follows: 1 multiloculated cyst, 1 (respiratory) epithelial cyst, 3 (enterogenous) epithelial cysts, 1 ependymal lined cyst and 1 neuroectodermal cyst. Various non-neoplastic cystic lesions are found in the central nervous system, such as arachnoid cyst, ependymal cyst, colloid cyst, choroid epithelial cyst, neurenteric cyst, and Rathke's cleft cyst. Although histological difference between arachnoid cyst and other epithelium-lined cysts is relatively clear, the precise discrimination between other cystic lesions is difficult and controversial. Some authors have considered these cysts as a neuroectodermal origin because of their histological similarity with choroid plexus or ependyma.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cestode parasitic infestation: intracranial and spinal hydatid disease--a clinicopathological study of 29 cases from South India

In developing countries hydatidosis is both a medical and economic problem related to environmental hygiene and healthy veterinary practice. This cestode parasitic infestation, uncommonly involving the nervous system, presents with varied clinical manifestation, at times causing diagnostic dilemmas. Multiple intracranial and spinal hydatidosis is rare. A series of 29 histologically confirmed cases of hydatidosis of neuraxis (21 intracranial and 8 spinal) from South India are presented. Among the 21 cases of intracranial hydatidosis, 12 cases were in pediatric age, while only 1 spinal lesion was noted in a 5-year-old child. The clinical presentation of intracranial lesions was predominantly that of raised intracranial pressure and visual symptoms, while spinal hydatidosis manifested with severe back pain, weakness and sphincter disturbances. The cranial cysts were usually single and uniloculated (12 cases), multiple in 7 and single but multiloculated in 2. In spinal hydatidosis, the cysts are usually multiple and extradural, rare ones being intramedullary and intradural. Based on clinical features and imaging, the differential diagnosis for intracranial lesions were cystic tumors and arachnoid cyst while metastasis and tuberculosis were considered in cases of spinal hydatidosis because of vertebral bony involvement. The majority of the cysts could be surgically resected totally and some were aspirated under control suction and resected. None of the cases had anaphylactic reaction, with no significant post-operative morbidity and no mortality. One intracranial and 2 spinal lesions caused by fertile cysts recurred to undergo repeated surgery.     

Intraspinal neurenteric cysts – report of three paediatric cases

BACKGROUND: Neurenteric cysts are rare congenital lesions of the spine and are lined with entodermal epithelium. They result from anomalous endodermal-neuroectodermal adhesion in the 3rd week of embryonic life with persistence of canal of Kovalevsky. The nature of the eventual abnormality depends on the extent to which this adhesion subsequently disappears. Persistence of the entire tract results in the extreme form of combined anterior and posterior spina bifida with dorsal enteric fistula and persistence of only a part of the tract producing the isolated intraspinal cyst. The most common location is the cervico-dorsal region, and usually it lies ventral to the spinal cord. The lumbosacral location is uncommon. Associated vertebral anomalies, gut cysts, bowel duplication, the presence of keratin markers and mucin-secreting cuboidal or columnar intestinal epithelium in their walls confirm their entodermal origin. PATIENTS: We describe here three unusual cases of neurenteric cysts in patients aged 5-18 years who had already had symptoms for some time. One of these had a cyst sited predominantly in the sacral canal, another presented with a lumbar neurenteric cyst, and the third patient had an intradural extramedullary thoracic lesion. Two of these children had associated anomalies, the one with lumbar cyst also having a lipomeningomyelocele and spina bifida while the other also had deformed vertebrae. All three patients underwent laminectomy and gross excision of the cysts through a posterior approach. RESULTS AND CONCLUSION: The diagnosis of neurenteric cysts was confirmed by demonstrating mucin-producing cuboidal or columnar epithelium lining the cystic cavity.     

Intradural extramedullary neuro-enteric cysts without associated malformation. 3 cases with magnetic resonance imaging

We report 3 cases of extramedullary neurenteric cyst without associated dysraphic lesions. One of the cases had an ultrastructural study. Magnetic resonance imaging provided a preoperative diagnosis. The embryogenesis of neurenteric cysts, their main clinical aspects and their surgical treatment are reviewed.     

Cerebellopontine angle neurenteric cyst with focal malignant features

Neurenteric cysts are uncommon cystic lesions lined by endodermal‐derived epithelium, which are rarely found in the CNS, especially in the intracranial region. Although recurrences and disseminations of these cysts have been reported, only one case of intracranial malignant transformation has previously been described. Here we report a cerebellopontine angle neurenteric cyst in a 26‐year‐old woman. The cyst wall was lined by columnar epithelium with atypical nuclei and high MIB‐1 index. In addition, stromal invasion was found in the subepithelial areas, which shows malignant features. Dissemination was speculated on MRI 6 months after total excision of the original cyst.     

Extradural thoracic meningeal cyst without spinal dysraphism causing adulthood myelopathy: Case illustration and review of the literature

Extradural spinal meningeal cysts are rare lesions in the adult spine and are an uncommon cause of neurologic deficits. We present the case of an adult who presented with myelopathic symptoms related to a dorsally based extradural thoracic meningeal cyst in the absence of any defect in the posterior spinal elements and no history of spinal dysraphism or trauma. We also performed a review of the literature to evaluate the surgical techniques for extradural meningeal cysts. Most thoracic cysts are intradural arachnoid cysts, yet this lesion is an extradural meningeal cyst, not an intradural arachnoid cyst. Because of the rarity of this lesion, its anatomic characterization can be difficult to conceptualize. An artist’s illustration helps illustrate the anatomic characteristics of this cyst and our surgical management.     

Primary extradural hydatid cyst associated with nephrotic syndrome in a pediatric patient

Background  

Primary intracranial extradural hydatid cyst is a very rare entity. Overlying bony erosion and extension of an extradural hydatid cyst into the scalp has never been described in literature. Association of nephrotic syndrome is described in pulmonary and hepatic hydatid cysts, but never reported in intracranial hydatidosis.     

Giant intradiploic infratentorial epidermoid cyst

Epidermoid cysts are benign, uncommon lesions (1% of all intracranial tumors). Their localization is intradiploic in 25% of cases, and exceptionally subtentorial. We report here a rare case of giant intradiploic infratentorial epidermoid cyst. A 74-year old patient presented with recent diplopia and sindrome cerebellar. CT scan and MR imaging revealed a giant osteolytic extradural lesion of the posterior fossa (5.2 cm x 3.8 cm) with a small area of peripheral enhancement after contrast injection. Retrosigmoid suboccipital craniectomy allowed a satisfactory removal of the tumor, followed by an acrylic cranioplasty. The outcome was good. Neuropathological examination confirmed an epidermoid cyst. We review the literature and discuss our case.     

Lumbosacral neurenteric cyst with associated tethered cord in an adult

Spinal neurenteric cysts are rare congenital lesions of endodermal origin that lead to spinal cord compression or tethering. These cysts are usually lined by mucus-secreting epithelium resembling that of the gastrointestinal tract. A 31-year-old man presented with low back and bilateral limb pain of three-year duration. Magnetic resonance imaging (MRI) revealed an intradural extramedullary heterogeneous mass lesion at L4 - L5 disc level causing severe compression and displacement of terminal portion of conus medullaris to the left side. Additionally, tethered cord, syringohydromyelia and spina bifida were present. He had also polysyndactyly on the left foot. The patient underwent laminoplasty and partial excision of the lesion through a posterior approach. Neurosurgical exploration revealed a large intradural, extramedullary solid lesion with cystic component, compressing the spinal cord. Histopathology confirmed a neurenteric cyst with mucin-producing cuboidal or columnar epithelium lining the cystic cavity. Clinical suspicion is important for an early diagnosis, and their early preplanned surgical removal should be the goal of treatment, which leads to a better outcome. This case is reported in view of rare congenital abnormality in an adult with peculiar presentation.     

Discal Cyst of the Lumbar Spine

Discal cysts are rare lesions that can cause radiating leg pain. Because they are very rare, their natural history and the details of the therapeutic guidelines for the treatment of these cysts are still unknown. A 30-year-old male patient presented to our institute with radiating pain in his left leg and mild back pain. Magnetic resonance imaging (MRI) revealed an intraspinal extradural cystic mass with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images at the L5-S1 level. The partial hemilaminectomy and cyst resection were performed. We report a patient with low back pain and radiating leg pain caused by a lumbar discal cyst and discuss the treatment of this cyst.     

Spontaneous hemorrhage into a neurenteric cyst of the cerebellar vermis

Neurenteric cysts are rare benign developmental lesions of the central nervous system which are lined by endodermal cell-derived epithelium. Here we present a 17-year-old male who presented with acute onset headache and cerebellar ataxia. He was found to have a hyperdense lesion of the cerebellar vermis on CT scan. The lesion was hyperintense on T?-weighted and hypointense on T?-weighted MR images. Surgical resection of the cyst revealed hemorrhage into the neurenteric cyst.     

Intramedullary neurenteric cyst in mid thoracic spine in an adult: a case report

Neurenteric cysts are very rare, particularly in adults. These are congenital intraspinal cysts of endodermal origin. A 67 years old man, presenting with backache and paraesthesiae of one and half years' duration, followed by subacute flaccid paraplegia, developing in a week is described. MRI revealed intramedullary cyst at T7. He underwent emergency thoracic laminectomy and complete excision of the cyst. Histopathology confirmed a neurenteric cyst. In view of their rarity, peculiarity in terms of age, location and presentation, we report this case.     

Cerebellar intra-axial dermoid cyst: a case of unusual location

Objects Intra-axial dermoid cysts are rare intracranial space occupying lesions, more so in the pediatric age group. Dermoid cysts account for about 0.2 to 1.8% of all intracranial tumors and are commonly located in the cisternal spaces, mainly in the cerebellopontine angle and parasellar cisterns. A purely intra-axial position as reported in this paper is quite exceptional. Materials and methods We report a case of intra-axial dermoid cyst located in the cerebellum that was surgically treated. The origin, clinical course, radiological features, pathological findings, and surgical treatment are discussed based on a review of the literature. Conclusion An intra-axial dermoid cyst is a rare entity and a preoperative diagnostic dilemma, as the radiologic findings overlap with many other intra-axial cerebellar masses. The case is reported in this paper to highlight the fact that, although rare, the possibility of an intra-axial mass being a dermoid cyst does exist.