A Rare Case of Neurenteric Cyst of Spinal Cord with Thoracic Vertebra Fusion Successfully Managed with Spinal Cyst Excision and Posterior Instrumentation Surgery

@#The incidence of neurenteric cyst (NC) is rare amongst spine tumors. It is most often asymptomatic but may present with sensory and motor symptoms. When associated with thoracic vertebra fusion it is not reported before, this complicates the placement of pedicle screw during posterior instrumentation. Herein, we report a case of thoracic spinal neurenteric cyst in a 40-year-old man that presents with chronic back pain, left lower limb weakness and numbness. Elective excision of NC over T6-T7 with laminectomy and multilevel posterior instrumentation was successfully performed with significant improvement of the symptoms. Neurenteric cyst is a rare spinal cord lesion which may cause permanent neurological sequalae. Complete surgical excision with spine fixation in this case provides good long-term outcome.

Cervical neurenteric cyst: a case report / Cisto neurentérico cervical: relato de caso / Quiste neurentérico cervical: reporte de un caso

ABSTRACT Objective Neurenteric cysts account for 0.7-1.3% of all spinal cord tumors. These rare lesions are composed of heterotopic endodermal tissue. Methods A 26-year-old woman with a 13-month history of severe cervicalgia and brachial paresthesia. Clinically she had mildbilateral brachial paresis (4/5), generalized hyperreflexia and a left Babinski Sign. Past medical history was significant for a cervical fistula closure when she was 1yearold. The superior somatosensory evoked potentials revealed medullary axonal damage with a left predominance. A cervical magnetic resonance imaging of the neck was performed showing a dorsal homogeneous cystic intradural extramedullary lesion with high signal intensity on T2. Computed tomography revealed a Klippel-Feil syndrome. Results A posterior laminectomy and surgical excision were performed without complications. Post-operative follow-up showed a complete recovery of arm strength. The histopathological report confirmed the preoperative diagnosis of neurenteric cyst. Most neurenteric cysts are located in the spine, mainly in a ventral position. A total of 95% of neurenteric cysts are found in the intradural/intramedullary compartment. Symptomatic neurenteric cysts typically appear in the second and third decades of life and are 1.5 to 3 times more common in men. In 50% of the cases, other vertebral malformations have also been reported. In this case, a congenital dorsal enteric cyst and a Klippel-Feil syndrome were observed. Conclusions The intraspinal neurenteric cysts are rare lesions that must be included in the differential diagnosis of a dorsal, intradural cystic structure. The diagnosis may be overlooked, especially in cases of chronic neck pain without neurological deficit. Level of evidence V; Expert Opinion.

RESUMO Objetivo Os cistos neurentéricos são responsáveis por 0.7 a 1.3% de todos os tumores da medula espinhal. Essas lesões raras são compostas por tecido endodérmico heterotópico. Métodos Uma mulher de 26 anos, com história de cervicalgia ao longo da vida, relatou história de 13 meses de piora da cervicalgia e parestesia braquial. Clinicamente, apresentava paresia braquial bilateral leve (4/5), hiperreflexia generalizada e um sinal de Babinski à esquerda. Na história médica pregressa, história de fechamento de fístula cervical com 1 ano de idade. Durante avaliação neurofisiológica, os potenciais evocados somatos-sensitivos superiores revelaram lesão axonal medular com predomínio à esquerda. Na ressonância magnética cervical, foi evidenciada uma lesão extra medular dorsal cística, intradural homogênea hiperintensa em T2. A tomografia computadorizada evidenciou síndrome de Klippel-Feil. Resultados A laminectomia posterior e a excisão cirúrgica foram realizadas sem complicações. O acompanhamento pós-operatório mostrou recuperação completa da força dos braços. O laudo histopatológico confirmou o diagnóstico pré-operatório de cisto neurentérico. A maioria dos cistos neurentéricos est ão localizados na coluna vertebral, principalmente em posição ventral, sendo 95% dos cistos neurentéricos encontrados no compartimento intradural e intramedular. Cistos neurentéricos sintomáticos aparecem tipicamente na segunda e na terceira décadas de vida e são 1,5 a 3 vezes mais comuns no sexo masculino. Em 50% dos casos, eles também foram relatados com outras malformações vertebrais. Neste caso, um cisto entérico dorsal congênito e uma síndrome de Klippel-Feil foram observados. Conclusão O cisto neurent é rico intramedular é uma lesão rara que deve ser conhecida pelo neurocirurgião e incluída no diagnóstico diferencial de lesões císticas intramurais dorsales, pois seu diagnóstico é muitas vezes difícil, especialmente em casos de cervicalgia crônica sem déficits neurológicos. Nível de evidência V; Opinião de Especialista.

RESUMEN Objetivo Los quistes neuroentéricos representan del 0,7 al 1,3% de todos los tumores de la médula espinal. Estas lesiones raras están compuestas de tejido endodérmico heterotópico. Métodos Una mujer de 26 años con cervicalgia severa de 13 meses de evolución acompañada de parestesia braquial. Clínicamente con paresia braquial bilateral leve (4/5), hiperreflexia generalizada y signo de Babinski izquierdo. En los antecedentes de importancia, le realizaron un cierre de una fístula cervical cuando tenía 1 año. Los potenciales evocados somatosensoriales superiores revelaron daño axonal medular con un predominio izquierdo. Se realizó una resonancia magnética cervical evidenciando una lesión ventral extramedular intradural quística homogénea hiperintensa en T2. La tomografía computarizada reveló un síndrome de Klippel-Feil. Resultados Se realizó una laminectomía posterior y resección quirúrgica sin complicaciones. El seguimiento postoperatorio mostró una recuperación completa de la fuerza de los brazos. El informe histopatológico confirmó el diagnóstico preoperatorio de quiste neuroentérico. La mayoría de los quistes neuroentéricos se localizan en la columna vertebral, principalmente en una posición ventral. El 95% de los quistes neuroentéricos se encuentran en el compartimento intradural/intramedular. Los quistes neuroentéricos sintomáticos aparecen típicamente en la segunda y tercera décadas de la vida y son 1.5 a 3 veces más comunes en los hombres. En el 50% de los casos, también se han notificado otras malformaciones vertebrales. En este caso, se observaron un quiste entérico dorsal congénito y un síndrome de Klippel-Feil. Conclusiones Los quistes neuroentéricos intraspinales son lesiones poco frecuentes que deben incluirse en el diagnóstico diferencial de una estructura quística intradural en posición dorsal. El diagnóstico puede pasarse por alto, especialmente en caso de dolor cervical crónico sin déficit neurológico. Nivel de evidencia V; Opinión de Especialista.

Quiste neuroentérico intradural: Reporte de un caso / Intradural neurenteric cysts. Case report

El quiste neuroentérico espinal es una lesión de rara presentación, sólo existen reportes de casos aislados. Representa del 0,3% al 1,3% de los tumores espinales según las distintas citas bibliográficas. Suele afectar el canal espinal, especialmente a nivel cervical bajo y torácico superior. Aproximadamente el 90% de estos quistes se localiza en el espacio intradural/ extramedular, mientras que el 10% restante se divide entre la localización intradural/intramedular o extradural. El tratamiento de los quistes neuroentéricos intraespinales es quirúrgico, mediante la resección lo más radical posible. Los resultados son favorables y las tasas de recurrencia después de la resección parcial son bajas. Nivel de Evidencia: IV

Spinal neurenteric cyst is a rare lesion, only anecdotal case reports have been published. The neurenteric cyst accounts for 0.3 to 1.3% of spinal axis tumors. It affects the inferior cervical and superior thoracic spine level. Approximately 90% of neurenteric cysts are located in the intradural/extramedullary compartment while the remaining10% are divided between an intradural/intramedullary or extradural location. Surgical intervention with radical resection is the best treatment. The outcome is favorable and the recurrence rates after partial resection are low. Level of Evidence: IV

Fourth Ventricle Neurenteric Cyst Mimicking Hemangioblastoma

This report presents a case of fourth ventricle neurenteric cyst (NE cyst) mimicking hemangioblastoma, which developed in a 50-year-old woman. A tiny enhancing mural portion of the fourth ventricle in MRI suggested that the cyst was hemangioblastoma, but pathological evidence showed that the cyst was in fact NE cyst in the fourth ventricle. In order to make proper decision on to what extent of surgical resection should be done, considering every possibility in differential diagnosis might be helpful. This case reports an unusual pathology in 4th ventricle, considering the patient's age, and demonstrates that a rarer disease may share radiological features of a common disease.

Cisto neuroentérico espinhal: relato de um caso e revisão da literatura / Spinal Neuroenteric Cyst: Report of a Case and Review of the Literature

Cistos neuroentéricos espinhais são anomalias do desenvolvimento, mais comumente encontrados no mediastino posterior e raramente presentes no sistema nervoso central. Geralmente localizados na coluna cervicotorácica. Principalmente encontrados em pacientes jovens e com predomínio no sexo masculino (2:1). Várias hipóteses têmsido sugeridas para explicar a embriogênese dos cistos neuroentéricos, entre elas a mais aceita seria uma incompleta separação entre o ectoderma e o endoderma. Manifesta-se principalmente como dorsalgia e déficit neurológico progressivo. Alguns pacientes apresentam surtos de dor e déficits transitórios. A ressecção completa da lesão, com esvaziamento do cisto e remoção do envoltório, é o tratamento de eleição. As principais complicações pósoperatórias são a recidiva da lesão, aracnoidite e piora do déficit neurológico.

Developmental abnormalities aremore commonly found in the posterior mediastinum and rarely present in the central nervous system. Usually located in the cervical and thoracic spine. Mainly found in young patients with predominance in males (2:1). Several hypotheses have been suggested to explain the embryogenesis of neuroentéricos cysts, including the most accepted would be an incomplete separation between the ectoderm and endoderm. It manifests primarily as back pain and progressive neurological deficits. Some patients experience bouts of pain and transient deficits. Complete resection of the lesion with dissection of the cyst and removing the wrap, is the treatment of choice. The main postoperative complications are recurrence of injury, arachnoiditis and worsening of neurological deficit.

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.

Lesiones quísticas de línea media de base de cráneo anterior / Cystic lesions in midline of anterior skull base

Las lesiones quísticas en línea media del segmento posterior de la base de cráneo anterior corresponden mayoritariamente a malformaciones congénitas, cuya ubicación puede ser selar, supraselar o intraesfenoidal. Generalmente asintomáticas, pueden presentar síntomas determinados por su crecimiento y/o el compromiso mecánico sobre las estructuras adyacentes. Su diagnóstico suele realizarse producto de un hallazgo imagenológico o endoscópico, constituyendo la resonancia magnética la mejor alternativa ante una aproximación diagnóstica inicial. Estas lesiones quísticas presentan un comportamiento benigno y no todas requieren un manejo quirúrgico, siendo suficiente en la gran mayoría de ellas, un seguimiento clínico. La necesidad de cirugía deberá decidirse en cada caso y depende principalmente de la sintomatología producida por la compresión de estructuras vecinas e hipertensión endocraneana. El abordaje quirúrgico endoscópico extendido es el método resectivo de elección. Presentamos una serie de casos clínicos, correspondiente a cinco pacientes con lesiones quísticas en línea media de base de cráneo anterior. Tanto la sintomatología como el diagnóstico etiológico tuvieron una presentación diversa en la serie expuesta; independiente de lo cual, todos fueron manejados exitosamente por cirugía endoscópica. Presentamos finalmente, una propuesta de diagnóstico inicial, basado en el estudio imagenológico con resonancia magnética de estas lesiones.

Cystic lesions in the midline of the anterior skull base are rare and mostly congenital malformations. Usually asymptomatic, symptoms can be determined by their growth and/or mechanical compromise on adjacent structures. The diagnosis is usually the result of imaging or endoscopic findings, MRI is the best alternative for the initial diagnostic approach. These cystic lesions are benign xx and not all require a surgical management. Some of them require a watchfull follow up. If they need surgery the expanded endoscopic approach is the method of choice in our hands. We present a case series, correspondent to five patients with cystic lesions in the midline of the skull base. All these were successfully managed by an extended endoscopic approach. Finally, we presented a proposal for initial diagnosis approach, based on the MRI findings of these lesions.

Intracranial Neurenteric Cyst of the Anterior Brain Stem in a Girl

Neurenteric cyst is a rare, congenital, and benign cystic lesion of the central nervous system, which is generally thought to result from failure of separation of the neuro-ectodermal and neuro-endodermal elements during week 3 of embryogenesis. Neurenteric cysts in the intracranial area are very rare lesions that typically occur in the spinal canal or even more seldom in the posterior cranial fossa. A girl presented to the outpatient clinic with complaints of moderate to severe episodes of headache with associated vomiting for 2 weeks. There was a positive sign of severe neck stiffness. Her brain MRI showed a neurenteric cyst located within the anterior intradural space of the foramen magnum. We report our experience with an intracranial neurenteric cyst located in the anterior brain stem area.

Craniospinal Neurenteric Cysts: Various MR Imaging Features

PURPOSE: Craniospinal neurenteric (NE) cysts are rare developmental non-neoplastic cysts of the central nervous system with diverse MR imaging findings. The purpose of this study was to evaluate various MR imaging findings of intracranial and intraspinal NE cysts. MATERIALS AND METHODS: We retrospectively reviewed the MR imaging findings of six NE cysts that were confirmed by pathology. We evaluated anatomic location, signal intensity, size and enhancement pattern of NE cysts. RESULTS: Two intracranial lesions were located extra-axially in the cerebellopontine angle and quadrigeminal cisterns. Three spinal lesions were intraduralextramedullary cysts, located ventral to the spinal cord, but one thoracic lesion was an intramedullary cyst. The signal intensity of the cysts was hyperintense on T1-weighted images as compared with the cerebrospinal fluid (CSF) for two intracranial lesions and one cervical lesion. In addition, all intracranial lesions showed diffusion restriction. For the remaining three spinal lesions, the signal intensity was nearly the same as the signal intensity of the CSF as seen on both T1- and T2-weighted images. On contrast-enhanced studies, two intracranial cysts showed a small nodular enhancement and one thoracic spinal lesion showed rim enhancement. CONCLUSION: NE cysts have various locations, signal intensities, and possible focal nodular or rim enhancement. Therefore, NE cysts can be included in the differential diagnosis of various craniospinal cystic or tumorous cystic lesions.

A Case of Neurenteric Cyst with Hemiplegia

Spinal neurenteric cyst results from the persistence of an abnormal communication between endodermal and neuroectodermal layer. It is a rare malformation that lead to spinal cord compression. The patient is a 22-month-old boy with sudden, progressive left side motor weakness. Preoperative magnetic resonance image revealed intradural extramedullary cyst in C4-C5 level that compressed the spinal cord on the left side. After the resection of the cyst, motor weakness of the left side was partially improved, and his motor power was completely recovered after rehabilitation. Histologic examination revealed a ciliated columnar epithelial neurenteric cyst. The pre- and postoperative clinical features of the case of neurenteric cyst is described with the review of literature.

An Intramedullary Neurenteric Cyst in the Conus Medullaris with Recurrent Meningitis

Neurenteric cysts are rare congenital lesions of the spine that are lined with endodermal epithelium. Their most common location is the cervico-dorsal region, and the mass usually lies ventral to the spinal cord. However the conus medullaris area location is an uncommon location. Neurenteric cysts are best treated by decompression and as near total excision of cyst membrane as possible. We report a case of a 7 year-old-girl with a neurenteric cyst in the conus medullaris. The patient had a history of meningitis and a gait disturbance. Magnetic resonance imaging (MRI) showed an intramedullary mass lesion in the conus medullaris with syringomyelia. There was no associated bone or soft-tissue anomaly. The mass was subtotally removed through a posterior approach. However 4 months later, meningeal irritation signs developed and MRI showed recurrence of the cyst. At the second operation, the cystic membrane was totally removed and the patient's neurological symptoms improved postoperatively. We reports a case of recurred neurenteric cyst occurred in unusual location with the review of literature.

Cervical Intramedullary Neurenteric Cyst in an Elderly Patient

Intraspinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including vertebral, visceral and cutaneous abnormalities. Most of these lesions have an intradural extramedullary location. Rarely, intramedullary neurenteric cysts not associated with other malformations have been reported. A 72-year-old woman presented with a 5-year-history of progressive paraparesis(I/II), urinary retention. A magnetic resonance(MR) image revealed a C6-C7 intramedullary cystic lesion which was located ventrally to the spinal cord and no other spinal abnormalities. The cystic lesion were subtotally removed through posterior approach. On histopathological examination, fibrous connective tissue surrounded by cuboidal cells which contained mucin vacuoles were consistent with neurenteric cyst. Postoperative MR image showed the decompression of the spinal cord. Postoperatively, neurological deficits were improved. We report a case of neurenteric cyst occured in highly old age and unusual location.

Recurrent Intramedullary Neurenteric Cyst of the Spine

Neurenteric cysts are developmental cysts derived from embryonic endodermal layers. Fewer than 100 have been reported in which there were no associated bone or soft-tissue malformations and only six among those cases showed intramedullary location in the literatures. The authors report a 16-year-old young man with a thoracic intramedullary neurenteric cyst which presented with symptoms of axillary pain and paraparesis. The magnetic resonance imaging showed intramedullary mass extended from level of T3 to T7. There was no associated bone or soft-tissue anomaly. This cyst was partially excised and marupialized into subarachnoid space. The pathological findings were compatible with neurenteric cyst. Nine months later, the cyst recurred and at second operation, cyst wall was removed completely.

Neurenteric Cyst of the Posterior Cranial Fossa: Case Report

Neurenteric cyst(enterogenous cyst) is a rare congenital lesion, usually affecting the lower cervical or upper thoracic areas, and its occurrence in cranial vault is even rarer. We present a patient with intracranial neurenteric cyst anterior to the pons and medulla oblongata. A 40-year old man presented with two-month history of occipital headache and facial spasm. The cyst was decompressed via far lateral suboccipital approach, and the patient recovered without permanent neurological deficits. Features on imaging studies and histological findings are discussed with the review of the literatures.

Neurenteric Cyst Presented by Asymptomatic Mediastinal Mass

Neurenteric cyst is a rare bronchopulmonary foregut malformation, originating from notochord defect. Characteristics of this anomaly are the intrathoracic cyst associated with vertebral abnormalities, such as hemivertebrae, anterior and posterior spina bifida, absence of vertebrae and diastematomyelia. The most common symptom is respiratory distress and treatment is surgical excision of mass. We report a case of neurenteric cyst in a neonate presented with asymptomatic huge mediastinal cystic mass.

A Neurenteric Cyst in Cervical Spinal Canal: A Case Report

We present the clinical, radiological and pathological features of a case of neurenteric cyst occurring at the low cervical region in a 30-year-old female. The patient complained paresthesia and weakness in her low extremities. Magnetic resonance imaging revealed intradural extramedullary cyst at C6 level. It was located ventral to the cord and was not associated with vertebral anormaly or mediastinal mass lesion. We operated through anterior approach and excised the cyst. This congenital cyst is important because it is readily amenable to surgery.

Spinal Neurenteric Cyst of Foregut Origin

The neurenteric cyst with associated anomalies is the result of an ectoentodermal communication that exists during embryogenesis. The variety of lesions include intraspinal cysts, congenital vertebral deformities, thoracic cyst, malformations of the digestive tract, and occasionally, dysrhaphias of the sinodermal or myelomeningocele type. A case of intraspinal neurenteric cyst in a 3-year-old boy is presented. He presented with cyclic abdominal pain, fever and constipation of 30 days' duration. These symptoms progressed rapidly into gait disturbance and left hemiplegia. A single epithelial cyst, located ventral to the spinal cord in the lower thoracic region, traversed the cleft of spina bifida of thoracic vertebrae and connected to retromediastinal cyst. The inner cyst wall was lined with pseudostratified ciliated epithelia and a few squamous cells. The cyst wall contained well-developed muscle coat, myenteric plexuses, and scattered seromucinous glands.

A Neurenteric Cyst in Cervical Spinal Canal: Case Report

A case of neurenteric cyst within the cervical canal is reported. He has been suffered from gradual aggravated quardriparesis since 4 months. On spine C-T scan and cervical myelographic examination, intradural extramedullary mass was detected. And so total laminectomy of C4, C5 and C6 was performed. We could diagnose by the pathological findings.