Neurenteric Cyst Research Articles

Neurenteric cysts in children: 16 consecutive cases and review of the literature

The authors of this retrospective review and analysis of the literature cover an institutional series of neurenteric cysts of the central nervous system in children treated in the magnetic resonance imaging era during a 14-year period. Sixteen patients 20 days to 14 years of age are described. The most frequent signs and symptoms at presentation were acute spinal cord compression (11 patients), paresis of a cranial nerve (two patients), meningitis or infection (two patients), and intracranial hypertension (one patient). The locations of the cysts were as follows: in the spinal canal in 12 patients (75%), the clivus in two (12.5%), the cavernous sinus in one (6%), and the craniocervical junction in one (6%). The most common location was the ventral aspect of the spinal canal (seven patients). Associated spinal deformities were found in five patients. All patients underwent surgery, with a posterior approach used in all of the spinal cases. Total resection was achieved in 12 of the 16 cases and partial resection in four. Of the four patients who underwent subtotal resection, the cyst recurred in three, requiring further surgery. Neurenteric cysts are uncommon congenital anomalies that can present acutely in the pediatric population. Total removal is usually possible and is associated with a good prognosis.

Neurenteric cyst: Case report and a review of the potential dysembryology

We report a 13-year-old female who presented to an outside emergency room following blunt trauma to the head. As part of her evaluation, an unenhanced CT of the face and radiographs of the cervical spine were performed. She was referred to our clinic with "abnormal" imaging. Radiographs revealed dysmorphic cervical spine and ventral clefting of the vertebral bodies from C7-T2. A subsequent MRI was obtained that demonstrated a large spinal cord mass. MRI demonstrated an unenhanced intramedullary mass of the cervicothoracic spinal cord measuring approximately 1.5 cm in greatest axial diameter. Surgical exploration of her intraspinal mass revealed a neurenteric cyst. Spinal neurenteric cysts are categorized in the spectra of occult spinal dysraphism and most likely arise from incomplete separation of the developing notochord and foregut in the embryo. We discuss potential etiologies for the formation of such cysts. The clinician should consider neurenteric cysts in their differential diagnosis of patients with spinal dysraphism and/or intraspinal masses.

Far-lateral transcondylar approach: surgical technique and its application in neurenteric cysts of the cervicomedullary junction

Neurenteric cysts are rare benign lesions of the central nervous system that are lined by endodermal cell-derived epithelium. Although they occur mostly in the spine, they can occur intracranially, most often in the posterior fossa. Neurenteric cysts that are located in the anterior cervicomedullary junction are even rarer and often require a skull base approach for adequate resection. The authors describe two cases of neurenteric cysts arising from the cervicomedullary junction that were resected via a far-lateral transcondylar approach. They discuss the surgical approach and operative nuances involved in removing these lesions, and review the clinical presentation of neurenteric cysts in this region as well as the neuroimaging characteristics, histopathological findings, and surgical management. Intraoperative videos are presented.

Neurenteric cyst of the posterior mediastinum

We report a case of a large posterior mediastinal neurenteric cyst in a neonate demonstrated by chest radiographs, barium swallow examination, ultrasonography and CT of the thorax. All the investigations revealed a large posterior mediastinal cystic mass with vertebral anomalies in the form of scoliosis and hemivertebra. The cyst was completely excised by a right posterolateral thoracotomy and biopsy showed the features of a neurenteric cyst. The rarity of the lesion prompted us to report this case.

Intramedullary neurenteric cysts of the spine

Neurenteric spinal cysts are infrequently occurring dysraphic lesions that are caused by persistent or abnormal communication among neuroectoderm, notochord, and endoderm. They are generally located at the intradural, extramedullary compartment of the low cervical or upper spinal canal. They occur primarily in infants and in young adults in combination with other congenital abnormalities. The authors report on three cases of lower-thoracic and thoracolumbar intramedullary neurenteric cysts that atypically presented in adult patients in whom there was no concomitant malformation. These lesions appear notable for location, clinical presentation, intraoperative findings, and imaging features.

Spinal neurenteric cyst presenting as recurrent midline sebaceous cysts

A 59-year-old patient had been treated by the general surgery department at her local hospital for a recurrent sebaceous cyst on the back of her neck. Cervical MRI showed a cystic structure, posterior to fused vertebral bodies, connected to the skin by a sinus passing through the middle of a split cord malformation. The lesion was excised via an anterior approach. Histology confirmed a benign neurenteric cyst. This rare developmental anomaly, mimicking a common acquired surgical condition, highlights the need for caution in the diagnosis of midline pathology.

A childhood case of neurenteric cyst that effect of puncture and absorption continued for nine years

A childhood case of neurenteric cyst that effect of puncture and absorption continued for nine years

0344 Spinal neurenteric cyst presenting as burning feet syndrome

0344 Spinal neurenteric cyst presenting as burning feet syndrome

Intracranial laterally based supratentorial neurenteric cyst

Neurenteric cysts are rare developmental cysts lined by endodermal derived epithelium. We present the case of a 68-year-old patient presenting with seizures who was found to have an extra-axial low density lesion with associated wall calcification on CT. Aspiration and biopsy of the cyst revealed a neurenteric cyst. Such lesions of the lateral supratentorial convexity are extremely rare and calcification has not been previously reported in these intracranial cysts.

Surgical Experience in Symptomatic Congenital Intraspinal Cysts

Symptomatic congenital intraspinal cysts are uncommon but not rare. Since these cysts may have various manifestations, a careful differential diagnosis is needed. We retrospectively reviewed findings in 3 women and 2 men (age range, 10–50 years) with 6 symptomatic cysts of the thoracic or thoracolumbar spine. In addition, we statistically analyzed the patients’ Nurick myelopathy grades before and after treatment. Pain and spastic paraparesis were the most frequent manifestations. Radiographs showed the widening of the spinal bony canal in 3 patients with extradural arachnoid cysts. All patients received limited laminectomy and appropriate surgical procedures. Pathology reports indicated neurenteric (n = 1), arachnoid (n = 4), and epidermoid (n = 1) cysts. Their functional status significantly improved by a mean of 2.6 Nurick points (p = 0.0002). Our findings confirm that these cysts have various manifestations. Congenital intraspinal cyst should be included in the differential diagnosis for patients with back pain, radiculopathy, cauda equina syndrome, Brown-Sequard syndrome, or myelopathy. The advent of MR imaging and increased knowledge about the pathogenesis of these cysts have improved the ease and accuracy of their early diagnosis; in addition, postoperative prognoses are excellent if surgery is performed early.

Split cord malformation with neurenteric cyst and pregnancy

Split cord malformation with neurenteric cyst and pregnancy

Split cord malformation with neurenteric cyst and pregnancy

Split cord malformation with neurenteric cyst and pregnancy

Intramedullary Mass Lesion of the Spinal Cord in Children of a Developing Milieu

A retrospective study of 22 children with intramedullary mass lesions (neoplastic and nonneoplastic) was performed to see the frequency of different histotypes of tumors/lesions and the functional outcome in Indian children. The age of the children varied from 1.5 to 16 years with a median of 8 years. The male to female ratio was 2.7:1. The children having a minimum follow-up of 3 months and a complete clinicopathological record were entered into this study. McCormick functional grading was applied retrospectively to assess the preoperative grade and postoperative functional outcome of the children. Histopathologically, these 22 cases were astrocytomas (n = 6, 27.3%), ependymomas (n = 3, 13.6%), dermoids (n = 3, 13.6%), epidermoids (n = 2, 9.1%), ependymal cysts (n = 2, 9.1%), and myxopapillary ependymoma, neurenteric cyst, ganglioglioma, teratoma, tuberculoma and anteriovenous malformation one each (4.55%). Near-total or gross-total resection was achieved in 13 (59%) and subtotal resection in 7 patients (32%), while complete excision of tuberculoma was achieved in 1 patient. Coagulation of perimedullary vessels was done in another single patient with anteriovenous malformations. Fourteen of the 22 cases having had functional grade IV or V preoperatively, improved to grade I or II (n = 7) and to grade III (n = 4, all astrocytomas) following surgery. Two patients died (1 within 2 days and 1 after 3 months due to recurrence of the tumor) and 1 case deteriorated at the follow-up of 4 months. Eight out of the 22 patients who had been admitted with functional grade II or III improved to grade I or II following surgery. The following conclusions were drawn after evaluation of these cases: (1) astrocytomas and ependymomas constituted only 50% of intramedullary tumors; (2) nonneoplastic, malformative tumors like dermoid and epidermoid showed a higher incidence (23%) in comparison with the Western World where all congenital intramedullary tumors (including dermoids and epidermoids) account for 5–8% only; (3) extremely rare cystic lesions like ependymal cyst and inflammatory granuloma may manifest as an intramedullary lesion; (4) even neurenteric cyst, though rare, may manifest as a pure intramedullary lesion without the stigmata of spinal dysraphism, and (5) delayed presentation was the cause of unfavorable outcome in a developing milieu.

Spinal dysraphism: MR imaging rationale

Spinal cord development occurs through the three consecutive periods of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal cord malformations derive from defects in these early embryonic stages, and are collectively called spinal dysraphisms. Spinal dysraphisms may be categorized clinically into open and closed, based on whether the abnormal nervous tissue is exposed to the environment or covered by skin. Open spinal dysraphisms include myelomeningocele and other rare abnormalities such as myelocele, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are further divided into two subsets based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category involves abnormal notochordal development, either in the form of failed midline integration (ranging from complete dorsal enteric fistula to neurenteric cysts and diastematomyelia) or of segmental agenesis (caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging is the imaging modality of choice for evaluation of this complex group of disorders.

A SPINAL NEURENTERIC CYST PRESENTING AS BURNING FEET SYNDROME

SUMMARYSpinal neurenteric cysts are rare congenital cysts of endodermal origin. A 34‐year‐old man presented with burning feet syndrome lasting two years. Magnetic resonance imaging (MRI) revealed an intradural extramedullar cystic mass extending from l_2 to L5 causing severe compression of the terminal portion of the conus medullaris and the filum terminale with displacement to the right side. A tethered cord and filar lipoma were also present. The cystic mass and filar lipoma were resected and the tethered cord released. Histopathology confirmed a neurenteric cyst. This case is reported in view of its peculiar presentation.

Diagnosis and treatment of foregut duplications

In its broadest sense, the term, foregut duplication encompasses the full spectrum of developmental aberrations of the embryonic foregut (bronchopulmonary and alimentary tract). Evidence is emerging that the notochord may have a pivotal role to play in foregut development through the Shh-GLi signalling pathway. The investigation and management of these lesions depends on the clinical presentation and the level of the foregut affected. The presentation of symptomatic foregut duplications also depends on any space-occupying effect they exert and where specific complications related to the malformation occur, such as when the mucosal lining contains acid-secreting cells. In a minority of cases, (eg, where they cause respiratory compromise or spinal cord compression) urgent intervention is required. In the remainder, precise diagnostic imaging according to the level and location of the foregut duplication provides the necessary information to plan surgical excision of the lesions. Magnetic resonance imaging best shows the relationships of complex bronchopulmonary foregut malformations and associated anomalies of the spine. eg, neurenteric canal. Most lesions can be excised with minimal morbidity. Minimal-access surgical techniques can be applied to the simpler cysts, particularly some bronchogenic cysts. Thoraco-abdominal duplications and neurenteric cysts require careful preoperative delineation and more complex surgery. Copyright 2003, Elsevier Science (USA). All rights reserved.

Diagnosis and treatment of foregut duplications.

In its broadest sense, the term, foregut duplication encompasses the full spectrum of developmental aberrations of the embryonic foregut (bronchopulmonary and alimentary tract). Evidence is emerging that the notochord may have a pivotal role to play in foregut development through the Shh-GLi signalling pathway. The investigation and management of these lesions depends on the clinical presentation and the level of the foregut affected. The presentation of symptomatic foregut duplications also depends on any space-occupying effect they exert and where specific complications related to the malformation occur, such as when the mucosal lining contains acid-secreting cells. In a minority of cases, (eg, where they cause respiratory compromise or spinal cord compression) urgent intervention is required. In the remainder, precise diagnostic imaging according to the level and location of the foregut duplication provides the necessary information to plan surgical excision of the lesions. Magnetic resonance imaging best shows the relationships of complex bronchopulmonary foregut malformations and associated anomalies of the spine. eg, neurenteric canal. Most lesions can be excised with minimal morbidity. Minimal-access surgical techniques can be applied to the simpler cysts, particularly some bronchogenic cysts. Thoraco-abdominal duplications and neurenteric cysts require careful preoperative delineation and more complex surgery.

A recurrent intradural cervical neurenteric cyst operated on using an anterior approach: a case report

Background The neurenteric cyst is an uncommon congenital lesion. In most reported cases, it has been operated on via a posterior approach using a laminectomy, despite the fact that the cyst is usually located ventral to the spinal cord. Reports have shown that early postoperative results have been good with the posterior approach, but very few studies of the long-term postoperative recurrence of neurenteric cysts have been conducted. Here, we report on a case of recurrent neurenteric cyst that was operated on using an anterior approach. Case description A 42-year-old woman presented with a cervical neurenteric cyst that had recurred eight years after its partial removal via a posterior approach. The patient complained of pain on the lateral side of her upper arms, and an magnetic resonance imaging showed that the recurrent cyst was located ventral to the spinal cord and compressed the cord dorsally at the C4-6 level. The patient was operated on via an anterior approach using a vertebrotomy at the lower half of C5 and the upper half of C6. The cyst was attached to the spinal cord firmly and was subtotally removed, with the thickest portion adhering to the cord not being removed. The caudal end of the cyst was observed with the assistance of a rigid endoscope. Conclusion A neurenteric cyst may recur after partial removal, and the patient’s condition may deteriorate during postoperative follow-up. The anterior surgical approach provides good visualization and facilitates safe removal of the lesion.

Intramedullary Neurenteric Cyst with a False Mural Nodule: Case Report

OBJECTIVE AND IMPORTANCE Spinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including typical vertebral and cutaneous abnormalities. The interest of the case of a spinal neurenteric cyst described here lies in its rare intramedullary location and in the false mural nodule image on the preoperative magnetic resonance imaging scan. A further distinctive feature is the association with a cleft spinal cord. CLINICAL PRESENTATION A 28-year-old woman presented with a 2-year history of progressive paraparesis and urinary retention. A magnetic resonance imaging study disclosed a T8–T9 intramedullary cystic lesion with a mural nodulelike formation on the posterior face. INTERVENTION A posterior midline myelotomy exposed a cystic lesion that had translucent walls and contained a milky fluid. No mural nodules were found. Once the cyst had been emptied, a collateral finding was a cleft that was clearly observed in the anterior spinal cord. The histological diagnosis was a neurenteric cyst. Retrospectively, the nodular lesion found on the preoperative scan was attributed to mucinous clots deposited at the bottom of the cyst. CONCLUSION This case report demonstrates that neurenteric cysts can vary widely in radiological appearance, depending on the contents of the cyst. These differences become especially important if the associated stigmata are lacking and the preoperative diagnosis rests on magnetic resonance imaging scan appearance alone.

頚椎neurenteric cyst 2例の治療経験

頚椎neurenteric cyst 2例の治療経験