Neurenteric Cyst Research Articles

Kyste neurentérique de la fosse postérieure. À propos d’un cas, revue de la littérature

Intracranial neurenteric cysts are rare entities. The term is currently used to describe epithelial cysts that are lined with a presumed endodermal-derived epithelium and are mostly located in the posterior fossa. Preoperative diagnosis is often difficult because of their clinical presentation, which may resemble a subarachnoid hemorrhage, and the radiological aspect, which can mimic vascular pathologies. We describe a posterior fossa neurenteric cyst in a 27-year-old woman, who presented with sudden headache as the only symptom and who was addressed to our hospital for subarachnoid hemorrhage. Diagnostic angiography was negative and MRI revealed a prepontine cystic lesion. The patient underwent a posterolateral approach on the right side, with subtotal resection of the lesion. We discuss the embryologic, diagnostic and therapeutic aspects of these cysts and review the literature.

Craniovertebral junction neoplasms in the pediatric population

The incidence of tumors at the craniovertebral junction in the pediatric population is low. Because of the variable pathology and the rarity of these tumors, ideal therapies are only now being defined. Thirty-eight children with tumors affecting the craniocervical junction were encountered between 1991 and 2006. These comprised neoplasms of osseous origin and neural extramedullary tumors. Chordomas of the clivus and foramen magnum were seen in eight, fibrous dysplasia in four, aneurysmal bone cysts in four, eosinophilic granuloma affecting the atlas and axis vertebra in four, Ewing's sarcoma involving the atlas in two, osteoblastoma in two, neurenteric cysts in four, meningioma in five, schwannoma in two, and plexiform neurofibromas in three. The location of these tumors was predominantly ventral, and a very small number had a lateral or dorsal location. The ventral tumors included chordoma, meningioma, fibrous dysplasia, aneurysmal bone cyst, and osteoblastoma. Plexiform neurofibroma affecting the craniocervical junction was ventral to the clivus and upper cervical spine causing severe kyphosis of the craniocervical region. Pain in the head and neck occurred in 70%. Paresthesias and dysesthesias in the hands were seen in 40% and spastic weakness of extremities in 22%. Cranial nerve palsies were seen in 33%. Twenty-eight percent of children showed dysphagia or dysarthria. The cranial nerves affected were the vagus followed by hypoglossal and glossopharyngeal nerves. This led to dysphagia, slurred speech, repeated aspiration pneumonia, and weight loss. The most common findings for chordomas at the craniocervical junction were isolated hypoglossal nerve palsy. All individuals underwent magnetic resonance imaging, computed tomography, and 3D computed tomography and angiography. Vertebral angiography was used to understand the dynamics of collateral circulation and tumor vascularity. Tumor embolization was performed in chordoma and aneurysmal bone cysts. Our experience and results are presented here.

Surgical approaches: postoperative care and complications “posterolateral–far lateral transcondylar approach to the ventral foramen magnum and upper cervical spinal canal”

Lesions that affect the lower clivus, foramen magnum, the craniocervical junction, and the upper cervical spinal canal that are anterolateral and at times intradural require access ventral to the cerebellum and spinal cord. The posterolateral transcondylar approach provides such a route. In addition, posterior craniocervical stabilization can be accomplished at the same time. The author has reviewed the technique as well as the surgical results here. The posterolateral transcondylar approach to the craniocervical junction was utilized in children with schwannoma, meningioma, and chordoma affecting the cervicomedullary junction. Other entities such as neurenteric cysts and hemangioblastoma were also seen. Extradural tumors such as aneurysmal bone cysts of the atlas and the axis vertebrae as well as proatlas segmentation abnormalities and bone tumors were seen. The stability of the craniocervical junction was assessed preoperatively so that a fusion procedure could be accomplished at the same operative setting, if necessary. Preoperative evaluation of the lower cranial nerves was vital. The surgical procedure was accomplished in the prone position. The occipital bone removal was carried out up to the sigmoid sinus and toward the jugular bulb. Relocation of the vertebral artery was made at the atlas vertebra and thus provided posterolateral exposure into the posterior fossa and upper cervical spinal canal. Occipital condyle removal was limited to one-third of the medial occipital condyle. Twenty-five children underwent a posterolateral transcondylar approach. New lower cranial nerve dysfunction occurred in two and only one required a tracheostomy. This was seen in a child with clivus chordoma. A complete removal was accomplished in meningioma and schwannoma as well as in neurenteric cyst and hemangioblastoma. Clivus chordomas required more than one surgical procedure. The tumors of the bone were all treated with simultaneous fusion. The posterolateral transcondylar route is a versatile avenue to approach a variety of lesions ventrolateral to the brain stem and upper cervical cord. Exposure is quite satisfactory with minimal or no retraction of important neurovascular structures in the region. Modifications of this theme can be applied as the lesions require.

High Cervical Neurenteric Cyst; Acute Post-traumatic Rupture and Respiratory Failure: A Case Report

The neuroenteric cyst is a rare developmental lesion originating from incomplete formation of the primitive neuroenteric canal. We report on the difficult management of an acute post-traumatic rupture of a cervical neuroenteric cyst. A 35-year-old male patient came to our observation after a spinal injury incurred during a football match. The patient immediately demonstrated tetraplegia and respiratory failure and was operated on. After two weeks the patient died of cardiopulmonary failure. High cervical neurenteric cysts, even those representing benign lesions, can produce dramatic results after a spinal injury.

Abdominal neurenteric cyst

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a loculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy (CT) scan revealed a 16 cm multiply 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive single-layered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.

Neurenteric Cyst of the Craniocervical Junction in an Infant -Case Report-

A 1-year-old boy presented with an extremely rare neurenteric cyst of the craniocervical junction manifesting as weakness in all extremities, slowly progressing over 2 months with rapid deterioration during the last few days. Neurological examination on admission showed tetraparesis with hyper-reflexia in the extremities. Magnetic resonance imaging demonstrated an intradural extramedullary cystic lesion located ventrally to the cord, extending from the craniocervical junction to the C3 level. The cyst wall was not enhanced with contrast medium, and the cyst content was isointense to the cerebrospinal fluid. He underwent total removal of the cyst through a left suboccipital craniectomy with C1 hemilaminectomy. The lesion had not adhered to the surrounding structures except for attachment to the left C2 nerve root outlet. Histological examination of the surgical specimen revealed typical neurenteric cyst with single layer epithelium. His symptoms improved significantly after the surgery, and he suffered no recurrence during the 2-year follow-up period. Neurenteric cyst should be considered in the differential diagnosis of intradural extramedullary cystic lesion located ventrally to the cord at the craniocervical junction in infants. Early surgical removal is recommended.

Is total excision of spinal neurenteric cysts possible?

Spinal neurenteric cysts are intradural cystic lesions. These represent a part of the spectrum of developmental anomalies. These are rare lesions. Most of the data in literature is review of case reports. This made us evaluate our results of 23 cases and review the literature. This is a retrospective study of 23 patients managed at our institute over 20 years. The slides were retrieved and histopathological features studied. Twenty patients were less than 30 years old and 21 were males. Follow-up was available for 21 patients with mean duration of 71 months (range 2 months to 23 years). The typical presentation was backache with progressive neurological deficits pertaining to the level. Atypical presentations as acute onset, recurrent episodic events and aseptic meningitis were also noted. The cervicothoracic region was the most common site. 16 patients had cyst in intradural extramedullary plane and seven had intramedullary location. Associated vertebral anomalies as hemivertebrae, Klippel – Feil and spina bifida were noted in seven patients. There were two histological types of cysts with no correlation between the type of cysts and associated vertebral anomalies and extent of outcome. Partial excision though had higher risk of recurrence, was not associated with poorer outcome. Dorsal approach is an acceptable route with reasonably good results for this lesion. Spinal neurenteric cysts present at younger age with varied clinical presentations. These are commonly located intradurally ventral to the cord. Histological types have no effect on the outcome. Total excision is the choice of treatment. However, partial excision is a feasible option in intramedullary lesions and when significant adhesions occur. Although associated with higher risk of recurrence, the outcome is still good in these patients on re-excision.

Intramedullary neurenteric cyst without concurrent malformation

Neurenteric cysts (NECs) are dysraphic lesions. They are usually extramedullary, the intramedullary forms are very rare. To our knowledge, only 10 cases of isolated intramedullary NECs have been reported. We report a case of intramedullary NEC without any associated dysraphic lesion. The clinical findings were: cervical pain and left hemicorporal weakness which predominated at the upper limb. Neuroradiological evaluation was performed by means of MRI which revealed a cystic lesion at C3-C4 level. A partial excision was performed and the diagnosis of NEC was established by histological study.

Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

Computed tomography (CT) and magnetic resonance (MR) imaging reliably demonstrate typical features of vestibular schwannomas or meningiomas in the vast majority of mass lesions responsible for cerebellopontine angle (CPA) syndrome. However, a large variety of unusual lesions can also be encountered in the CPA. Covering the entire spectrum of lesions potentially found in the CPA, these articles explain the pertinent neuroimaging features that radiologists need to know to make clinically relevant diagnoses in these cases, including data from diffusion- and perfusion-weighted imaging or MR spectroscopy, when available. A diagnostic algorithm based on the lesion's site of origin, shape and margins, density, signal intensity and contrast material uptake is also proposed. Non-enhancing extra-axial CPA masses are cystic (epidermoid cyst, arachnoid cyst, neurenteric cyst) or contain fat (dermoid cyst, lipoma). Tumours can also extend into the CPA by extension from the skull base (paraganglioma, chondromatous tumours, chordoma, cholesterol granuloma, endolymphatic sac tumour). Finally, brain stem or ventricular tumours can present with a significant exophytic component in the CPA that may be difficult to differentiate from an extra-axial lesion (lymphoma, hemangioblastoma, choroid plexus papilloma, ependymoma, glioma, medulloblastoma, dysembryoplastic neuroepithelial tumour).

Neurenteric cyst—a case report of this rare disorder

Neurenteric cysts are rare congenital lesions that are believed to be the result of the split notochord syndrome. We report the clinical case of a 5-year-old boy presenting with vague gastrointestinal symptoms and fatigue, who had undergone resection of a small intestine duplication cyst as a newborn. Computed tomography revealed a mediastinal neurenteric cyst with partial destruction of several thoracic vertebrae. Resection of the tumor proved effective. Recognition of this disorder is important: because of its benign nature, the prognosis after surgical resection can be good. If the diagnosis is made in an early stage, unnecessary progressive destruction of surrounding structures may be prevented.

Etiology and outcome of prenatally detected paracardial cystic lesions: a case series and review of the literature

Isolated paracardial cysts, defined as cystic structures adjacent to or originating from the heart, are rare and etiologically heterogeneous congenital abnormalities. The purpose of this study was to review our experience with prenatally diagnosed isolated cysts. We reviewed retrospectively the medical charts and ultrasound records of all cases with an antenatal diagnosis of paracardial cyst at our institution between 2001 and 2006. Where applicable, the diagnosis was further substantiated by other imaging modalities and pathology. The cysts in six fetuses were diagnosed at a median gestation of 20 (range, 19-38) weeks. Three of these fetuses presented with a fluid-filled cyst attached to or within the pericardial space (pericardial cysts), which resolved spontaneously by the time of delivery. In contrast, the cysts did not change in size or shape in the remaining three fetuses. Postnatal examination of the persistent cysts revealed three different etiologies: (1) a microcystic lymphangioma, located in the anterior mediastinum; (2) an isolated neurenteric cyst; and (3) a single bronchogenic cyst, both within the posterior mediastinum. The lymphangioma and neurenteric cyst were removed surgically after birth. Fetal echocardiography enables early detection of paracardial cyst. Pericardial cysts disappeared spontaneously during the course of gestation without signs of fetal cardiac compromise, suggesting a benign prognosis. Imaging by magnetic resonance and computerized tomography were particularly useful to clarify the etiology, structure and extent of those cysts that had not resolved by the time of birth. Published by John Wiley & Sons, Ltd.

Excision of an asymptomatic cervical intradural neurenteric cyst through the anterior approach: a study of two cases and a review of the literature

Background Spinal neurenteric cysts are very rare lesions, especially after the second decade of life. They account for 0.3% to 0.5% of all spinal tumors and occur most commonly in ventral locations. The cysts are usually removed via a posterior approach. Purpose To present the clinical and radiologic results of patients with spinal neurenteric cysts who were treated via anterior approach procedures. Study design This report is composed of two cervical neurenteric cyst cases that are compared with published studies. Methods We present two patients, 41- and 39-year-old women, each with a cervical intradural neurenteric cyst. Both of these patients had apparent neck pain without neurological deficit. MRI revealed neurenteric cysts located at C7 and C7–T1 levels. After anterior corpectomy, the intradural cysts were removed, and then fusion was performed. Results The postoperative period went well. The follow-up cervical MRI studies were performed at 3, 6, and 18 months postoperatively, and there were no abnormalities found. Conclusions This study has led to the conclusion that although neurenteric cysts are rare in adults, they can still be present with only persistent neck pain and without neurological deficits. This may lead to misdiagnosis. The importance of MRI is not controversial in the early diagnosis. An anterior approach may be considered the first preference for surgical technique in patients with ventrally located neurenteric cysts.

NEURENTERIC CYST ARISING IN THE HIGH CONVEXITY PARIETAL LESION

Supratentorial neurenteric cysts (NC) are rare; only 16 cases were reported previously, the majority of which were located in the anterior fossa or in the deep midline structure. We report a case of NC arising in the high convexity parietal lesion and briefly discuss the histogenesis of NC in such an unusual location. A 63-year-old man presented with a 1-month history of progressive headache and dizziness. Computed tomographic and magnetic resonance imaging scans demonstrated a 4 x 5 x 5 cm extra-axial cystic lesion in the right parietal convexity adjacent to the sagittal sinus without contrast enhancement. A translucent thin-walled cyst containing a milky-white fluid was partially excised via a right parietal craniotomy. The cyst wall was composed of a thin fibroconnective tissue lined by a single layer of ciliated columnar epithelial cells and pseudostratified columnar epithelium. Mucous-secreting goblet cells of epithelial lining reacted to periodic acid-Shiff staining. The internal epithelial cells were immunoreactive to cytokeratin and epithelial membrane antigen, but not to carcinoembryonic antigen, glial fibrillary acidic protein, or S-100 protein. A follow-up magnetic resonance imaging scan taken 6 months later showed resolution of mass effect without evidence of cyst recurrence. The patient was discharged home in excellent condition. A case of NC arising in the high convexity parietal lesion is described. Supratentorial NCs located far from the location of the primitive endoderm suggest the possibility that NCs could arise from ectopic endodermal tissue through an undetermined process of anomalous differentiation. Total surgical excision of the cyst wall and contents is the best treatment for the symptomatic cases. Subtotal or partial resection without injury to important neurovascular structures are reasonable alternatives, but long-term careful follow-up magnetic resonance imaging or computed tomography is necessary to monitor for recurrence of any residual lesion.

Brainstem Epidermoid Cyst in a Child-Case Report-

A 10-year-old girl presented with a brainstem epidermoid cyst manifesting as dysphagia and post-prandial vomiting. Magnetic resonance imaging disclosed a cystic mass in the brainstem with associated hydrocephalus. She was treated under a presumptive diagnosis of abscess until the definitive histological diagnosis was established at the fourth admission. In spite of four interventions, she remained in good condition with minimal cranial nerve pareses. Subtotal removal of the epidermoid cyst in the brainstem results in recurrence within a relatively short period, especially in children. Total resection of the tumor is the optimum treatment. The differential diagnosis includes abscess, cystic glioma, and neurenteric cyst.

True Intraspinal Neurenteric Cyst in the Lumbosacral Region-Case Report-

A 56-year-old man presented with a very rare true neurenteric cyst in the conus medullaris without evidence of vertebral or visceral anomaly manifesting as a 6-month history of mild low back and bilateral inguinal pain. No motor weakness was found in the bilateral lower extremities. He had also suffered dysesthesia in the bilateral feet for several weeks before admission. Lumbar spine magnetic resonance (MR) imaging demonstrated a cystic intradural extramedullary mass at the L1-2 levels without enhancement after gadolinium injection. MR imaging, computed tomography, and radiography detected no vertebral anomaly. Lumbar laminectomy at the L1-2 levels was performed and the lesion was incompletely removed. Histological examination showed the cystic wall lined with ciliated columnar epithelium. Neurenteric cyst should be considered in the diagnosis of isolated cystic mass lesion at the lumbosacral region even in the absence of vertebral or visceral abnormality.

Unusual split cord with neurenteric cyst and cerebellar heterotopia over spinal cord

To report a case of splitting of a cord, which could neither be classified as type I nor type II according to Pang's classification. The child had associated neurenteric cyst and cerebellar tissue heterotopia. A 3-year-old girl presented with a tuft of hair and dermal sinus at her upper dorsal spine. She had splitting of cord into two halves by two intradural horny osseocartilaginous spurs leaving a gap of 3 mm in between (two spurs and two halves of cords). A neurenteric cyst was seen passing from the anterior to posterior aspect of the cords through this gap. An associated heterotopic cerebellar tissue mass was encountered over the dorsum of cord proximal to splitting. The dermal sinus, neurenteric cyst, and spurs were excised to detether the cord. The heterotopic tissue was biopsied. The child had no neurological deficit at 2.5 years follow-up. An unusual splitting of cord by unusual spurs may leave a gap between two spurs and two halves of cords to pass a neurenteric cyst. Rarely, heterotopic tissue may be associated with split cord syndrome. A dermal sinus may lead into underlying neurenteric cyst (rather than dermoid).

Spectrum of Nonterminal Myelocystoceles

To present the magnetic resonance imaging features, clinical findings, and possible embryologic bases for nonterminal myelocystoceles, a distinct subset of closed spinal dysraphisms. We retrospectively analyzed imaging series and clinical records from five newborns and one older child with skin-covered soft tissue masses along the posterior midline spine. Spinal (6 patients) and brain (5 patients) magnetic resonance imaging was performed before surgical repair and compared with clinical findings, observations at surgery, and final lesion histology. The lesions affected the cervical (n = 3), thoracic (n = 2), and lumbar (n = 1) regions. In each case, the dome of the mass was covered by thickened, dystrophic epithelium with no subcutaneous fat, whereas the base and lateral walls of the mass were covered by normal skin. All patients were neurologically intact at presentation. In three cases, a stalk emanated from the dorsal normal spinal cord, crossed a narrow posterior bony spina bifida, and coursed through a posterior meningocele to attach to the inner aspect of its dome. The other three cases showed dissection of a hydromyelic cavity into the stalk, converting it into a second "cyst" within the meningocele. Concurrent anomalies included focal hydromyelia immediately cranial to the origin of the posterior stalk (n = 2), mild Chiari II malformation (n = 3), triventricular hydrocephalus from aqueductal stenosis (n = 1), filar lipoma (n = 1), and presumed neurenteric cyst (n = 1). At surgery, the sac was resected in all cases, but intradural exploration and untethering was performed in only three children. Embryologic considerations indicate that the spectrum of these lesions likely arises from partial limited closure of the neural tube, failed disjunction of the cutaneous ectoderm, and variable degrees of hydromyelia. The nonterminal myelocystocele is a distinct form of closed spinal dysraphism characterized by a skin-covered meningocele, which is either crossed by a fibroneurovascular stalk that extends from the dorsal aspect of the spinal cord to attach to the dome of the meningocele (abortive form, or myelocystocele manqué) or contains a hydromyelic cavity that is continuous with the ependymal canal of the spinal cord (complete form).

Spinal neurenteric cyst presenting as recurrent midline sebaceous cysts

Spinal neurenteric cyst presenting as recurrent midline sebaceous cysts

Intracranial neurenteric cyst with recurrence and extensive craniospinal dissemination

Neurenteric cyst represents a rare cystic lesion of the central nervous system, and is generally thought to result from failure of separation of neuro-ectodermal and endodermal elements during week 3 of embryogenesis. Although recurrences have been reported, only one case of craniospinal dissemination has previously been reported. We describe a unique intracranial neurenteric cyst with recurrence and dissemination to the spinal cord.

Spinal Neurenteric Cysts in the Magnetic Resonance Imaging Era

Neurenteric cysts are derived from displaced entodermal tissue. They are infrequently found in the ventral spinal canal with varying degrees of success in their removal. Experience with 10 such individuals is critically analyzed to aid in the diagnosis and management. Ten patients, ages 4 through 55 years, with neurenteric cysts were encountered in the last 20 years. This series included three females and seven males; seven children and three adults. The follow-up periods ranged from 3 to 18 years. Three cysts were located at the ventral cervicomedullary junction, five in the midventral cervical spine, and one thoracic and one lumbosacral. The symptoms reflected the location. Six of the 10 patients had associated bony abnormalities such as bifid clivus, hemivertebrae, segmentation failures at the site of the lesion, and blocked vertebra. The patient with the thoracic spinal lesion (age, 55 yr) had symptoms from early childhood. A diplomyelia at the site of the lesion was seen in one individual and tethered spinal cord in the same adult and in a young child. Two patients had undergone laminectomy for aspiration and partial resection before referral to our institution at the time of recurrence. Cervicomedullary junction lesions were approached via the far lateral transcondylar approach; two of the cervical intramedullary lesions were resected via a ventral corpectomy with radical resection and interbody fusion. The goal in each case was complete resection to avoid recurrences. Spinal neurenteric cysts are ventrally located, usually intradural and extramedullary, but may insinuate into the spinal cord. They are isointense on T1 images and hyperintense on T2-weighted images on magnetic resonance imaging without true enhancement. These lesions may be associated with block and hemivertebrae with a predisposition to the craniocervical region. Partial resections have led to recurrence and arachnoiditis.