Ependymal Cyst Research Articles

Neuroendoscopic Approach to Intracranial Ependymal Cysts

Intraparenchymal cysts without communication to the ventricles or the subarachnoid space are named ependymal or epithelial cysts. The estimated ratio of their incidence compared with arachnoid cysts is 1:10. Neurologic deficit can occur when the cyst exerts mass effect on its surroundings. We evaluated the success rate of endoscopic fenestration of intracranial ependymal cysts. Our prospectively maintained endoscopy database was screened for all cases of ependymal cysts. The charts were retrospectively reviewed for symptoms, surgery, postoperative course, and complications. Magnetic resonance imaging scans performed before and after surgery were analyzed. We identified 6 patients harboring an intracranial ependymal cyst. The cyst location was frontoparietal, parietal, occipital, or mesencephalic. Patients presented with several symptoms according to the location of the cyst (i.e., epilepsy, hemiparesis, diplopia, hemianopsia). All patients were treated by navigation-guided endoscopic fenestration of the cyst to the ventricular system. Two complications occurred: a cerebrospinal fluid leak, which was managed surgically by wound revision without the need for cerebrospinal fluid shunting, and a chronic subdural hematoma, which occurred 6 weeks after surgery and required burr hole evacuation. Follow-up period ranged from 6 months to 9 years. Magnetic resonance imaging revealed that all cysts decreased in size. Symptoms improved in all patients. Endoscopic fenestration of ependymal cysts to an adjacent ventricular cavity is a treatment option with excellent long-term results and minimal morbidity. It should be considered as the therapy of choice to avoid craniotomy and shunt dependence.

Ependymal Cyst of Third Ventricle Presenting as Intermittent Ataxia in a 2 Year Old Child

The purpose of this study is to present and document the rare and dynamic behavior of an ependymal cyst. We report 18 months old Caucasian girl who presented with multiple episodes of intermittent ataxia and vomiting with unremarkable neurological examination. Non contrast head CT was consistent with marginally dilated right lateral ventricle. MRI showed 1.7 × 1.5 × 1.1 cm cystic structure at the anterior aspect of the third ventricle at the level of the foramen of Monro. With right frontal endoscopic approach endoscopic ablation of the cyst was achieved. Histologic findings and the immunophenotype was consistent with a diagnosis of ependymal cyst. The patient made an excellent recovery after the procedure.

Coexisting intramedullary schwannoma with an ependymal cyst of the conus medullaris: A case report.

Synchronous spinal intramedullary ependymal cysts and intramedullary schwannomas are rare. To the best of our knowledge, the present study is the first report of a case of intramedullary schwannoma coexisting with an ependymal cyst. A 35-year-old male presented with lower back pain and weakness in the left leg. Magnetic resonance imaging identified an intramedullary cystic-solid lesion at the thoracolumbar junction of T11-L2; based on the clinical presentation and radiological features, a pre-operative diagnosis of ependymoma was formed. Subsequently, the patient underwent a T11-12 laminectomy via a posterior approach, with intraoperative monitoring of somatosensory and motor-evoked potentials, achieving a gross total resection of the tumor with a well-demarcated dissection plane. Post-operative histopathological examination demonstrated a schwannoma coexisting with the ependymal cyst, and the neurological status of the patient markedly improved compared with the pre-operatively observed neurological deficit.

Development of a Short Sleeper Phenotype after Third Ventriculostomy in a Patient with Ependymal Cysts

A naturally short sleeper phenotype with a sleep need of less than 6 hours without negative impact on health or performance is rare. We present a case of an acquired short sleeper phenotype after third ventriculostomy. A 59-year-old patient suffering from chronic hydrocephalus reported an average of 7-8 h of nocturnal sleep. After surgical intervention, the patient noted a strikingly reduced sleep need of 4-5 h without consequent fatigue or excessive daytime sleepiness, but with good daytime performance and well-balanced mood. Short sleep per 24 hours was confirmed by actigraphy. Postoperative imaging revealed decreased pressure around the anterior third ventricle. The temporal link between development of a short sleeper phenotype and third ventriculostomy is striking. This might suggest that individual short sleep need is not only determined by genetics but can be also be induced by external factors.

Unilateral multilevel interlaminar fenestration: A minimally invasive approach for cervical intramedullary lesions

The traditional approach for cervical intramedullary lesions is laminectomy, but the procedure may result in spinal instability and spinal deformity. Unilateral multilevel interlaminar fenestration (UMIF) is an alternative minimally invasive approach that may have great advantages in preserving spinal stability. However its use in cervical intramedullary lesions is rare, and the indications, safety and limitations of this approach for cervical intramedullary lesions are still under investigation. We report five patients (three males, two females, age range 12–46years) who were treated between 2010 and 2011 for cervical intramedullary lesions. The lesions included three ependymomas, one astrocytoma and one ependymal cyst, and the locations of the lesions were at the medulla–T2, C4–T1, C5–C7, C4–C7 and C6–C7. All of these lesions were completely removed through UMIF, and all patients had stable or improved neurological status after surgery. No recurrences or spinal deformities were detected during the follow-up period which ranged from 24 to 35months (mean=27.4months). UMIF is a feasible approach for selected cervical intramedullary lesions. This approach allows complete resection of multilevel lesions without increasing the risk of injury to the spinal cord, and minimizing the risk of postoperative spinal instability. The indications for and limitations of UMIF are discussed.

Intracranial extracerebral glioneuronal heterotopia with adipose tissue and a glioependymal cyst: a case report and review of the literature.

The presence of central nervous system (CNS) tissue outside the cranium is often referred to as although technically this should be termed ectopia, according to the dictionary definition. Glioneuronal heterotopia (GH) is a rare, mass-forming, malformative lesion. Ectopic glioneuronal tissue of the head and neck has been detected in the nasopharynx, oropharynx, tongue, palate, tonsils, soft tissue, eye, and orbit, and intracranial extracerebral glioneuronal heterotopia (IEGH) has also been reported, although less frequently.1,2 Since the first description of neuroglial heterotopia in the dorsal meninges of the cervical spinal cord by Wolbach in 1907,3 fewer than 20 cases of IEGH have been reported. Glioependymal cysts are rare, ependyma-lined, cystic lesions of the subarachnoid space, which have been referred to as epithelial or ependymal cysts. Histopathologically, they are lined with ependymal cells abutted on the glial layer and are commonly detected in the posterior fossa. The origin of glioependymal cysts of the posterior fossa is not clear, but these cysts may represent neuroglial heterotopia, persistent Blake's pouch (diverticulum of the roof of the fourth ventricle), or remnants of a tela chorioidea. We report here a case of IEGH that was predominantly composed of cerebellar tissue with some fat tissue and a large glioependymal cyst, and was initially misdiagnosed as a teratoma with a glioependymal cyst.

Clinical characteristics and surgical outcomes of spinal intramedullary ependymal cysts

Intramedullary ependymal cysts are exceedingly rare lesions, and have been previously reported in the literature as case reports. The aim of this study was to discuss the clinical presentation and the outcomes of microsurgery for these benign lesions. The authors retrospectively reviewed the records of ten patients who underwent microsurgery for intramedullary ependymal cysts. All patients had preoperative and postoperative magnetic resonance imaging. The surgical treatment included gross total resection and biopsy plus a cyst-subarachnoid shunt. The diagnosis of intramedullary ependymal cysts was based on radiological and pathological criteria. All patients were followed up, with a mean duration of 47.6 months. Intramedullary ependymal cysts were hypointense on T1-weighted images and hyperintense on T2-weighted images. Contrast-enhanced T1-weighted images showed no enhancement. Gross total resection was achieved in one case. Biopsy of the cyst wall plus cyst-subarachnoid shunt placement was achieved in nine cases. Long-term neurological function was improved in eight patients. No recurrence was observed on magnetic resonance imaging. Ependymal cysts should be considered in the differential diagnosis of intramedullary cysts. For symptomatic patients, early surgery should be performed before neurological deficits deteriorate. Complete decompression and cyst-subarachnoid shunt placement is the optimal treatment and the outcome may be favorable.

Glioependymal cyst in children: A case report

Ependymal cysts remain a very rare cause of neurological symptoms among intracranial cystic lesions. It differs from an arachnoid cyst, as it does not communicate with subarachnoid space.A 33-year-old woman was admitted in our neurosurgery department with history of headache, vomiting and right unilateral blindness. Neuroimaging studies showed an intraparenchymal cystic lesion in the right frontal lobe with radiologic characteristic as seen in the ependymal cyst or hydatic cyst. She underwent the neurosurgical procedure of marsupialization and was discharged on day 6 post op. At one month follow up, the patient presented with a decrease vision acuity on the left eye, and head CT scan showed a recurrence of the cyst hence, we performed a second surgery with shunt procedure. Histological examination of the resected tissue demonstrated that, it was an ependymal cyst.Since there is often times a clinico-radiological discordance they are generally incidental findings in majority of cases. While complete surgical excision is the treatment of choice for the symptomatic cysts, cystic fluid diversion presents a better compromise nevertheless in cases where this may not be achieved.

Ependymal cyst of the midbrain

We present the case of a 30-year-old man who developed an acute hydrocephalus secondary to an obstruction of the cerebral aqueduct by a midbrain cystic lesion. After a ventriculo-peritoneal shunt was placed to relief symptoms of intracranial hypertension, the patient underwent a neuronavigation-assisted endoscopic fenestration of the cyst. A careful immunohistochemical staining confirmed the diagnosis of an ependymal cyst. An extensive review of the literature has revealed that this is the first report of a periaqueductal ependymal cyst with definite histological diagnosis. This is a rare cause of acute non-communicating hydrocephalus but an important entity in the differential diagnosis.

Intra-Extramedullary Drainage as an Effective Option for Treatment of Intramedullary Ependymal Cyst of Thoracic Spine: Technical Note

Intramedullary neuroepithelial cysts are extremely rare and only 15 cases have been reported in the literature. Clinico-radiological features are not indicative of a specific diagnosis; for this reason, diagnosis is based mainly on the histological features. In the literature, total surgical removal is considered the treatment of choice. The risk of recurrence is higher after partial removal and in cases of occlusion of intra-extramedullary shunt. For this reason, a surgical strategy that ensures the shunt patency in case of incomplete removal of the cyst becomes a very safe option for treatment of this pathology. We report the case of a 51-year-old woman who was found to have a dorsal (D9) intramedullary neuroepithelial cyst. She underwent surgical treatment with partial removal and placement of a Nelaton drainage device (8 French) inside the intra-extramedullary shunt. The patient experienced a complete regression of preoperative symptoms and magnetic resonance imaging (MRI) follow-up showed no radiological evidence of recurrence 24 months after surgical treatment. Spinal ependymal cysts show a high frequency of recurrence, especially in cases of partial removal of the cyst wall. Unfortunately, the cyst walls are often closely adherent to the spinal cord, making total removal impossible. Intra-extramedullary shunting is a viable option, although there is a high frequency of recurrence in cases of obstruction of the shunt. Placing an 8 Ch Nelaton drain between the dorsal columns is a reliable technique, especially in cases of partial removal. In fact, it allows continuous drainage of cyst fluid and subsequent resolution of symptoms, and it decreases the incidence of recurrences due to obstruction of the shunt.

An ependymal cyst in cerebello-pontine angle presenting with syncope.

Intracranial ependymal cysts are rare, congenital, benign lesions. These commonly occur in the supratentorial regions and usually generate no symptoms. The cerebellopontine angle (CPA) is an extremely rare site for ependymal cysts. Furthermore, there are no previous reports of CPA ependymal cysts related to syncope. We report a case of ependymal cyst in the left CPA with syncope. The patient underwent a cardiologic evaluation for syncope after admission, but there were no definite cardiologic abnormal findings. He underwent fenestration into the subarachnoid space, and the pathologic diagnosis revealed an ependymal cyst. We analyzed this case with review of other literatures.

Ependymal Brain Cyst with Posterior Cerebral Artery Infarct

Primary intracranial ependymal cysts are congenital, benign, ependyma-lined cysts rarely seen in the pediatric age group. The authors report such a case of intracranial fronto-parietal ependymal cyst in a 3 y-old girl. Computed tomography showed a large cystic lesion which was not in communication with the ventricular system and was associated with ipsilateral posterior cerebral artery infarct and raised intracranial pressure causing midline shift. A differential diagnosis of the commoner arachnoid, hydatid or porencephalic cyst was considered. Surgical removal to decompress the brain was done; histopathology revealed an ependymal cyst. Identification of this rare pathology in the pediatric age group is emphasized with a review of the literature.

Spinal Intramedullary Ependymal Cysts : A Case Report and Review of the Literature

We report a rare case of a spinal intramedullary ependymal cyst in a 46-year-old female and review the 17 pathologically proven cases in the literature. The patient presented with a two-week history of gradually increasing tingling in her left posterior thigh and calf. A preoperative magnetic resonance image revealed a well-defined intramedullary cystic lesion on the ventral side of the spinal cord at the T11 to T12 levels. The lesion was hyper intense in T2-weighted images and hypointense in T1-weighted. The patient underwent a right-side hemilaminectomy at the T11 to T12 levels and fenestration of the cyst wall. After having the cyst wall partially removed and communication established between the cyst and the subarachnoid space, the patient improved neurologically. A histological study of the surgical specimens revealed that the cyst wall consisted of glial cells lined by a simple cuboidal to columnar epithelium. An immunohistochemical examination of the cells lining the cyst wall was positive for S-100 protein, glial fibrillary acidic protein, epithelial membrane antigen, and cytokeratin. We suggest that the optimal treatment of intramedullary ependymal cysts creates adequate communication between the cyst and the subarachnoid space.

Kyste neuroépithélial thalamo-mésencéphalique

Thalamo-mesencephalic neuroepithelial cysts are rare lesions of the central nervous system. They are thought to arise from neuroectoderm and are also referred to as ependymal cysts due to their origin. It can remain asymptomatic throughout life or rarely can cause symptoms. We describe a 42-year-old woman who presented with thalamic syndrome due to a neuroepithelial cyst of the thalamo-midbrain. Differential diagnosis is made with other cystic lesions in the brain. However a good analysis of imaging feature led to diagnosis. When the lesion is symptomatic, mini-invasive procedure is indicated.

The histopathological spectrum of cutaneous meningeal heterotopias: clues and pitfalls

To describe the histopathological features of heterotopic cutaneous meningeal tissue. Nineteen cases were collected between 1993 and 2010. Immunohistochemistry for epithelial membrane antigen (EMA), neuron specific enolase (NSE), S100, glial fibrillary acid protein (GFAP), progesterone receptor (PR), CD31, glucose transporter-1 (Glut-1), podoplanin and NKI-C3 was performed. Lesions were congenital (100%) and presented as aplasia cutis with alopecia (63%) or lumps (37%), on the scalp (18 of 19) and sacral region. Resonance magnetic imaging revealed four underlying anomalies of the neuraxis. Histopathological analysis showed meningeal tissue arranged in four variably associated architectural patterns: fibrous (100%), pseudovascular (100%), cellular (68%) and pseudomyxoid (32%). Other features included collagen bodies (58%), calcifications (26%) and dermal melanocytes (32%). Heterotopic brain tissue or heterotopic ependymal cyst was associated in two cases. Arachnoidal cells expressed EMA and NSE, but not S100 protein, CD31 or GFAP. They expressed podoplanin (93%), especially in pseudovascular areas, NKI-C3 (79%), and less frequently Glut-1 (46%) and PR (30%). Histopathological features of cutaneous meningeal heterotopias are various and sometimes misleading. Fibrous lesions should not be misdiagnosed as aplasia cutis. Podoplanin-positive pseudovascular spaces represent the main pitfall and should not be diagnosed as lymphangioma. Correct diagnosis is confirmed by EMA and NSE coexpression within the lesion.

Ependymal Cyst in the Cerebrum of an African Green Monkey ( Chlorocebus aethiops)

A focal lesion was detected by magnetic resonance imaging in the right caudal occipital lobe of the cerebrum in an African green monkey (Chlorocebus aethiops). Neurological signs were not observed in this animal. At necropsy examination, an 8mm wedge-shaped intracranial cavity was found, which apparently did not communicate with the ventricles. Microscopically, the inner surface of the cavity was lined by ciliated cuboidal epithelium with positive immunoreactivity for S100 protein, glial fibrillary acidic protein and cytokeratin. Based on the gross, microscopical and immunohistochemical findings the lesion was classified as an ependymal cyst. To the best of our knowledge, this is the first report of an ependymal cyst in an African green monkey.

The endocrine spectrum of intracranial cysts in childhood and review of the literature

Intracranial cysts (ICC) may cause a wide spectrum of endocrinological disorders. We evaluated 27 patients who were diagnosed with ICC during investigation for neuroendocrine dysfunctions and reviewed the relevant literature. The types of ICC in the patients were arachnoid cysts (n = 13); Rathke cleft cysts (n = 7); pineal cysts (n = 5); an ependymal cyst (n = 1) and a cavum septum pellucidum cyst (n = 1). The neuroendocrine dysfunctions of the patients were obesity (n = 7), isolated growth hormone deficiency (n = 6), central precocious puberty (n = 6), multiple pituitary hormone deficiency (n = 3), central diabetes insipidus (n = 1), growth hormone deficiency and central precocious puberty (n = 1), obesity and galactorrhea (n = 1), obesity and hypogonadotropic hypogonadism (n = 1) and growth hormone neurosecretory dysfunction (n = 1). Only three patients, who had arachnoid cysts, showed neurologic symptomatology. Although three patients underwent surgery, no improvements in endocrinological dysfunctions were observed. ICC should be considered when evaluating patients with endocrinological problems and patients with coincidental ICC should be recommended for follow-up.

Ependymal cyst in the conus medullaris

The immunohistological features and surgical treatment of an intramedullary ependymal cyst in the conus medullaris is presented. An intramedullary ependymal cyst is a rare lesion of dysembryoplastic origin. There have been only seven patients reported with pathologically proven ependymal cysts in the conus medullaris. A 64-year-old woman reported pain and numbness in both thighs and feet. Neither sensory nor motor impairment was present in the lower extremities. MRI revealed a cyst on the right side of the conus medullaris, compressing the spinal cord upward. Clinical signs and symptoms disappeared following surgical resection of the cyst. Histological examination showed that this cyst was lined with a single layer of tall columnar or low cuboidal cells on fibrous connective tissue. The basement membrane was absent in the cyst wall. Reactivity to CAM5.2 and AE1/AE3 anti-keratin antibodies suggested that the cyst was of neuroepithelial origin. No recurrence has been noted 3 years after surgery.

Cervical intradural extramedullary ependymal cyst associated with congenital dermal sinus: a case report

Spinal intradural extramedullary ependymal cyst is a very rare entity with only few cases reported in the literature. Its association with congenital dermal sinus has not been described so far. We present a unique report of a 3-year-old male child who presented with spastic quadriparesis with a trophic ulcer in the right great toe of 1-year duration. He harbored a congenital dermal sinus in the cervical spine since birth. Intraoperatively, the sinus was associated with an intradural cyst which proved to be an ependymal cyst on histopathological examination. The clinical profile along with review of literature of this rare entity is presented.

A patient with Huntington’s disease and long-surviving fetal neural transplants that developed mass lesions

Transplantation of human fetal neural tissue into adult neostriatum is an experimental therapy for Huntington's disease (HD). Here we describe a patient with HD who received ten intrastriatal human fetal neural transplants and, at one site, an autologous sural nerve co-graft. Although initially clinically stable, she developed worsening asymmetric upper motor neuron symptoms in addition to progression of HD, and ultimately died 121 months post transplantation. Eight neural transplants, up to 2.9 cm, and three ependymal cysts, up to 2.0 cm, were identified. The autologous sural nerve co-graft was found adjacent to the largest mass lesion, which, along with the ependymal cyst, exhibited pronounced mass effect on the internal capsules bilaterally. Grafts were composed of neurons and glia embedded in disorganized neuropil; robust Y chromosome labeling was present in a subset of grafts and cysts. The graft-host border was discrete, and there was no evidence of graft rejection or HD pathologic changes within donor neurons. This report, for the first time, highlights the potential for graft overgrowth in a patient receiving fetal neural transplantation.