Glial Heterotopia Research Articles

Congenital midline nasofrontal masses

Congenital midline nasal masses are uncommon anomalies including nasal dermoids/epidermoids, nasal glial heterotopias and encephaloceles. These lesions can occur at the nasal bridge, extend intranasally and have intracranial extension with communication to the subarachnoid space. Therefore, accurate diagnosis of these lesions is critically important for presurgical planning and prevention of potentially fatal complications. Neuroimaging is essential in the evaluation of congenital midline nasal masses to identify the specific type of lesion, evaluate for the presence of intracranial extension and allow for appropriate presurgical planning.

Glial Heterotopia of the orbit: A rare presentation

BackgroundGlial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness.Case presentationA 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit.ConclusionThough the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness.

Pulmonary Glial Heterotopia in a Child Diagnosed With Fanconi Anemia and Epilepsy

Pulmonary glial heterotopia is rare and its pathogenesis is still obscure as for Fanconi anemia (FA). This study describes a very rare case of an incidental finding of pulmonary glial heterotopia in a girl diagnosed with FA, epilepsy, and mental retardation. Before this report, the association of pulmonary glial heterotopia and FA had not been described. The unique finding in this patient could be a link between FA and abnormal cell migration, but it certainly teaches us that there is still much to be learnt of the molecular mechanisms underlying the clinical manifestations in FA.

Glial Heterotopia in ENT—Two Case Reports and Review of Literature

The purpose of presenting these case reports is to highlight the occurrence of heterotopic glial tissue of the tongue and nose in children. So far, literature review has revealed few case reports of such lesions in neonates, but our patients presented with this unique lesion at the age of two and a half years and 3years. This is a rare congenital anomaly in the tongue, which can mimic a lingual thyroid, teratoma, dermoid cyst etc. Surgical excision is mandatory when the lesion causes obstructive symptoms. The authors discuss the problems in diagnosis, pathology and management and review the literature.

Glial heterotopia of maxilla: A clinical surprise

Glial heterotopia is a rare congenital mass lesion which often presents as a clinical surprise. We report a case of extranasal glial heterotopia in a neonate with unusual features. The presentation, management strategy, etiopathogenesis and histopathology of the mass lesion has been reviewed.

Endoscopic Management of a Rare Case of Nasal Glioma in Meckel's Cave in an Adult: Case Report

Nasal glioma or glial heterotopia is a rare embryologic anomaly that heralds its presence shortly after birth or in childhood. Nasal glioma in an adult is very rare, often asymptomatic and the occurrence of nasal glioma in Meckel's cave in an adult has not been previously reported. The authors encountered a case of an incidentally diagnosed Meckel's cave nasal glioma in a 40-year-old male which was successfully excised by an endonasal endoscopic transmaxillary transpterygoid approach. The occurrence of a nasal glioma in Meckel's cave an adult is very rare. Considering the deep skull base location, endonasal endoscopic surgery provides a minimal access technique to reach this location with excellent results.

Glial Heterotopia of the Uterine Cervix: DNA Genotyping Confirmation of its Fetal Origin

Uterine glial heterotopia is a rare, yet biologically intriguing lesion, mostly involving the cervix. Although an implantation of the fetal brain tissue is widely accepted as the etiology, there has been no confirming evidence to support this hypothesis. We investigated a case of polypoid glial heterotopia of the uterine cervix in a 42-year-old woman who underwent an elective termination of pregnancy of a Down syndrome fetus. One year earlier, the patient had a pregnancy termination of a fetus with Klinefelter's syndrome. Gross and microscopic examination revealed a 2.5 cm polypoid cervical lesion consisting of lobulated mature glial tissue covered by endocervical glandular epithelium. The neural nature of the lesion was confirmed by glial fibrillary acidic protein and S100 immunohistochemistry. DNA genotyping of the cervical polyp, maternal, first and second fetal tissue samples showed an identical genetic profile between the cervical glial tissue and the first aborted fetus. Genotyping also attested the presence of Klinefelter's syndrome in the first gestation and Down syndrome in the second gestation. Therefore, this molecular case investigation confirms the fetal origin of uterine glial heterotopia.

Simultaneous Occurrence of Glial Heterotopia and Meningocele in the Orbit With Clinical Anophthalmia and Neurological Anomalies

The authors describe a 5-year-old boy who had three congenital anomalies (clinical anophthalmos, meningocele, and glial heterotopia) in the orbit. These were associated with multiple neurological anomalies.

Bilateral cystic pulmonary glial heterotopia and palatinal teratoma causing respiratory distress in an infant

We report on a male infant with extensive, bilateral cystic and solid lung lesions who presented postnatally with respiratory distress caused by bilateral cystic lung lesions. Parenchyma-sparing resections were performed. Histology revealed the presence of neuroglial cell-lined cysts and glial nodules. In addition, a neural element containing palatinal teratoma was detected and excised. Based on previously published cases, the pathogenesis and clinical features of pulmonary neuroglial heterotopia are discussed.

17- NASAL GLIOMA, A CASE REPORT AND LITERATURE REVIEW

Nasal glioma (also known as nasal glial heterotopia) has been used to describe a congenital benign tumor of the nasal region containing neural tissue. It arises from failure of closure of foramen caecum at about the third week of gestation. The patient described in this report is an 18 months old girl who presented with a nasal bridge swelling for three months. The differential diagnosis included nasal encephalocele, nasal dermoid and epidermoid cysts. All are due to failure of ectoderm and neuroectoderm embryologic separation. CT scan and MRI imaging can be used to look for probable concomitant intracranial tumors and the existence of a connection between nasal tumor and the brain. Surgical resection is the usual method of managing such pathologies. In this case, an open rhinoplasty approach was used to resect this mass after the radiologic evaluation was complete.

External rhinoplasty excision of a nasal tip glioma in a 6-month-old infant: Case report and review of the literature

Summary In this report, we describe an infant with a nasal tip glioma treated by excision via external rhinoplasty. A 3-month-old full-term, otherwise healthy infant presented with a slowly enlarging midline mass of the nasal tip. Clinically the mass appeared cystic without trans-illumination, enlargement with crying, or signs of infection. On successive CT scans at 3 and 5 months old the mass appeared as an enlarging focal prominence to the left of midline with central hypodensity without evidence for intracranial extension. Preoperatively the differential diagnosis included nasal dermoid, glioma, and hemangioma. The mass was removed at 6 months of age via an open rhinoplasty approach with conchal cartilage grafting to reconstruct the left upper lateral cartilage. The final pathological diagnosis was nasal glioma (glial heterotopia) with fibrosis. Pre-operative, intra-operative, and post-operative photos were taken. The patient has been followed up for 6 months without evidence of recurrence or other complications. A review of the literature on nasal glioma and external nasal surgery in infancy is presented.

A 2-month-old Girl with an Enlarging Lesion of the Nasal Root

A 2-month-old Girl with an Enlarging Lesion of the Nasal Root

Glial heterotopia of the face

The authors report a case of combined subcutaneous and intranasal glial heterotopia of the face in a 4-month-old boy. The pathogenesis and differential diagnoses of this rare developmental disorder are discussed as is the importance of careful radiologic findings for appropriate surgical decision.

An unusual midline nasal mass in a newborn

We report a rare case of glial heterotopia that presented as an unusual mass over the bridge of an infant's nose. Nasal gliomas arise due to defective closure of the anterior neuropore during embryological development and represent encephaloceles that have lost their intracranial connection. They are treated by excision, which also allows definitive histological diagnosis.

Respiratory Distress Secondary to Nasopharyngeal Glial Heterotopia

Nasal glial heterotopia (nasal glioma) is a rare congenital malformation of neural origin. We present a newborn baby with life-threatening respiratory distress secondary to nasopharyngeal glial heterotopia that obstructed the nasopharyngeal or nasal airway. A high degree of suspicion, early diagnosis and surgical management are essential to cure this rare and potentially life-threatening disorder.

Report of a case of sinonasal undifferentiated carcinoma arising in a background of extensive nasal gliomatosis

Sinonasal undifferentiated carcinoma (SNUC) is a relatively rare, aggressive malignancy of adulthood. Nasal glial heterotopia is a benign congenital condition in which mature benign brain tissue develops outside the central nervous system. A 37-year-old man was seen with right nasal obstruction, epistaxis, and headache. Imaging showed a radiologically malignant mass lesion. Biopsy and subsequent resection were performed in the evaluation and treatment of this lesion. Histologic and immunohistochemical assessments of the biopsy and resection specimens on the pathology material classified the lesional tissue. Histology and immunohistochemistry of the biopsy specimen demonstrated an SNUC that was subsequently resected following chemotherapy. In addition to SNUC, the resection specimen revealed extensive, multifocal nasal glial heterotopia (so called "nasal glioma"). This is the first known case in the literature in which these 2 relatively rare entities coincide.

Bilateral Macrostomia Associated with Aqueductal Stenosis and Glial Heterotopias

We report on an Italian boy, born to normal and nonconsanguineous parents with a prenatal diagnosis of ventriculomegaly and subependymal glial heterotopias. At birth bilateral macrostomia was diagnosed without other evident facial anomalies. Magnetic resonance imaging (MRI) showed triventricular hydrocephalus and aqueductal stenosis and confirmed the nodules of glial heterotopia. The bilateral macrostomia was surgically corrected with the vermilion square flap method and W-plasty technique and follow up MRI at 6 months showed mild increase of ventricular dilatation without signs of active hydrocephalus. The association between macrostomia and hydrocephalus has been reported only in rare cases of complex malformative syndromes but never with isolated macrostomia.

Nasal Glial Heterotopia—A Case Report

Introduction: Congenital nasal masses occur in about 1 in 20,000 to 40,000 live births. Usually, these are dermoid cysts, encephaloceles, or nasal gliomas (nasal glial heterotopia). They form during embryogenesis due to incomplete separation of ectodermal and neuroectodermal tissue. Nasal gliomas may occur extranasally, intranasally, or as a combination of both. They manifest usually at birth or during early childhood and can cause a visible deformation, a nasal obstruction, or chronic otitis media.

Ectopic Cerebellum in Anterior Cranial Fossa: Report of a Unique Case Associated With Skull Congenital Malformations and Epilepsy

An isolated, well-differentiated ectopic cerebellum arising outside the posterior fossa is extremely rare. We present a unique case of ectopic cerebellum in 25-year-old woman with hypertelorism, skull deformation, and a longstanding history of epileptic seizures. Magnetic resonance imaging revealed a mass lesion at the base of the frontal lobes with no apparent connections to the adjacent brain structures. Total resection of the lesion was performed. On gross inspection, its surface resembled cerebellar cortex with densely packed folia. Histologically, the cerebellar cortex was composed of well-differentiated external molecular, Purkinje cell, and internal granular cell layers. The deeper part of white matter displayed the features of neuroglial, hamartomatouslike abnormalities. There were numerous neuronal and/or glial heterotopias ranging from single dysplastic neurons to well-circumscribed clusters of neuronal and/or glial cells surrounded by neuropil. Some large neurons looked like mature ganglion cells, Purkinje cells, or dentate neurons. Large irregular islands of heterotopic tissue displaying well-differentiated cerebellar cortex could also be seen. Other parts of the ectopic cerebellum revealed loosening of tissue with dispersed glioneuronal elements. The ectopic brain tissue may arise from disturbed migration of primitive pluripotent stem cells during embryogenesis. The presented case of cerebellar ectopia associated with skull congenital malformations exhibited multiple dysontogenetic abnormalities. To our knowledge, this is the first report of totally isolated, well-differentiated ectopic cerebellum in the anterior cranial fossa accompanied by skull deformation and epilepsy.

A midline nasopharyngeal heterotopic neuroglial tissue

Glial heterotopia is a rare disorder. However, it must be considered in the differential diagnosis of airway obstruction in the newborn. Differentiation of this lesion from nasal glioma and encephalocele is important. In this paper we report a midline glial heterotopia, which presented with upper airway obstruction in a newborn. The origin of a glial heterotopia from the midline of the nasopharynx has not been reported before. We present a review of the literature and discuss the clinical, radiological and pathological features of nasopharyngeal brain heterotopia in an infant.