Subependymal grey matter heterotopia, also known as periventricular heterotopia, is the most common form of grey matter heterotopia and is characterized by nodules of grey matter located immediately beneath the ependyma of the lateral ventricles. The radial glial processes form delicate, parallel arrays extending from the vz bottom to the cortical plate top, not in view. Glial heterotopia is an extremely rare clinical entity mostly presenting in head and neck region secondary to an abnormal development of an isolated mature central nervous system cns tissue at an abnormal location without any continuity with the brain, spinal cord, or meninges. Case report a case of nasal glial heterotopia in an adult. Nasal glial heterotopia refers to congenital malformations of displaced normal, mature glial tissue, which are no longer in continuity with an intracranial component.
Bicklerb,g, a department of surgery, naval medical center san diego, san diego, ca 924, usa b department of surgery, university of california san diego, san diego, ca 92103, usa c department of pediatrics, tamale teaching. Heterotopia article about heterotopia by the free dictionary. Glial heterotopia is a rare congenital often midline developmental, nonneoplastic displacement of neuroglial tissue in extracranial sites without connection to the cranial cavity. A moderately retarded japanese boy, with a normal male karyotype 46,xy, was diagnosed to have a subcortical band heterotopia or double cortex syndrome. On t2weighted coronal mr sections, there were numerous radial linear structures between the cortex and the band, probably representing the. A rare presentation ranju sitaula, gulshan b shrestha, nabin paudel, sneha shrestha and dev n shah abstract background. Thompson, armed forces institute of pathology, washington, dc. We herein present the mr imaging findings of a parapharyngeal neuroglial heterotopia presenting as a giant, growing, single locular cyst in a monthold girl. Teerthanath srinivas, kailkere padma shetty, hariprasad sinivas department of pathology and surgery, k. Lizardoa,g, simone langnessb,g, hassan mumunc, james murphyd, robert newburye, patricia brombergerf, stephen w.
Mixed heterotopic gastrointestinal cyst and extranasal glial tissue of oral cavity with cleft palate. This paper reports a rare case of nasal glial heterotopia presenting as sphenochoanal polyp. However, from ct findings, 15%20% of patients have a defect in the cribriform plate 4, 5. Periventricular heterotopia genetic and rare diseases. A 6 yearoldboy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed. Band heterotopia or double cortex in a male neurology.
Nasal glial heterotopia is a rare type of neoplasm consisting of meningothelial andor neuroglial elements. Glial heterotopia or ectopic brain masquerading as a. A case of nasal glial heterotopia in an adult ncbi. Health, general excision surgery usage genetic disorders diagnosis research medical care quality management medical care quality nasopharynx neuroglia. Our data suggest that disruption of both polarized radial glial scaffold and neuroepithelial lining was the likely cause of the failure of some neurons to migrate away from the vz and forming ph. Complete surgical excision of nasal glial heterotopias is curative. Like focal subcortical heterotopia, band heterotopia form in the white matter beneath the cortex, but the gray matter is more diffuse and is symmetric between the hemispheres. The potential for this type of ngh to have an intracranial connection must always be considered when planning surgery for a congenital midline mass to prevent the risk. A case of nasal glial heterotopia in an adult europe pmc.
Unlike midline glial heterotopias, nonmidline glial heterotopias are seen in adults. She was born at 40 weeks gestation via normal spontaneous vaginal delivery. Mixed heterotopic gastrointestinal cyst and extranasal. Case report open access glial heterotopia of the orbit. Glial heterotopia of the middle ear volume 121 issue 4 m z uguz, s arslanoglu, s terzi, d etit. Heterotopic definition of heterotopic by medical dictionary. Glial heterotopia is a rare nonhereditary, benign congenital malformation, which is composed of normal glial tissue isolated from the central nervous system. Nasal glial heterotopias have no communication with the central nervous system. For 2 years after surgery, the patient has been free from nasal. The presence of glial tissue can be confirmed by ihc for. Subependymal grey matter heterotopia radiology reference. Pdf nasal glial heterotopia also known as nasal glioma, is a rare developmental. Case report nasal glial heterotopia in a newborn infant catarina vilarinho, md, filipa ventura, md, ana paula vieira, md, maria joa.
The marked weight loss of this patient could be attributed to dysphagia from oropharyngeal obstruction, decreased appetite from. Considers the uses and dangers of utopian thinking in the postmodern world. Introduction nasal glial heterotopias ngh are congenital tumours of the midline frontonasal space arising from a normal neurectodermal tissue entrapped during the closure of the anterior neuropore. Nasal gliomas are nonneoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. Nasal glial heterotopia also known as nasal glioma is a rare developmental abnormality typically seen at birth or in early childhood. To our knowledge this is the first reported case of glial heterotopia of orbit. Nasal glial heterotopia is a rare condition, thought to be derived from either entrapped neuroectodermal tissue during the closure of the covering of the brain, or a nasal encephalocele which is covered by dura, pia, and arachnoid and later disconnected from the intracranial cavity during subsequent development. Nasal glial heterotopia refers to congenital malformations of displaced normal, mature glial.
They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Parapharyngeal neuroglial heterotopia presenting as a. Scattered pigmentary and atrophic changes were present in. Nasal glial heterotopia develops from normal glial tissue, but is located in an abnormal location, and is isolated from the nervous system without intracranial connection. Glial heterotopia of the scalp inchara y k, rajalakshmi t. Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Failure to recognize the entity is the principal difficulty in diagnosis. Dec 01, 2008 the authors report a case of combined subcutaneous and intranasal glial heterotopia of the face in a 4monthold boy. Subsequently, ct scan of the sinuses revealed sphenochoanal polyp filling. Previous reports of these cases have shown only ct images of neuroglial heterotopia presenting as a lowattenuation mass with some area of focal cystic formation 35. Theright fundus showedadensewhitefibrous or glial tissue mass extending back into midvitreous fromthe upper temporal ora serrata with aslightly bulbous termination and a finer band extending from its tip to the upper part ofthe optic disc fig. All texts by michel foucault for educational purpose, under fair use of the berne convention. Nasal glial heterotopia nasal glioma the journal of pediatrics. They, are particularly dangerous because of the possibility of intracranial extension.
Hegde medical academy, nithyanand nagar, derelakatte, mangalore 575 018, karnataka, india click here for correspondence address and email. Aim and objective of the study was to study clinicopathological aspects of four cases of nasal glial heterotopia and a rare case of glial heterotopia involving the middle ear. Heterotopia space wikipedia, the free encyclopedia. We report a rare case of nasal glial heterotopia in an adult. The term heterotopia was introduced by the german naturalist e. The radial glial architecture is disrupted in the vz around the pvh. Background glial heterotopia is defined as presence of normal glial tissue. For years a er surgery, the patient has been free from nasal symptoms, signs of recurrence, and dyskinesia. The patient was free of disease 18 months following surgery. We report a neonate with extensive extranasal glial heterotopia involving the left. This congenital lesion is found mainly in the nose and. Johns medical college and hospital, bangalore 560 034, india 2 department of paediatric surgery, st.
Nasal glial heterotopia, also known as nasal glioma, is a rare developmental anomaly of the nasal cavity occurring in patients over a wide range of ages, with a predilection for presentation at birth or in early childhood. Heterotopia definition of heterotopia by the free dictionary. Histologically they comprise glial elements within interspersed fibrous tissue. As it occurs commonly in or around the nose, it is often referred to as nasal glial heterotopia. Haeckel in 1874 to designate disturbances of the phylogenetically determined spatial sequence of the stages of ontogeny. Histologic examination revealed a prominent dermal and subcutaneous neural proliferation composed of astrocytes, neurons, and neuroglial. Nasal glial heterotopia with cleft palate sudhir chandna 1, milind a mehta 2, abhishek kishore kulkarni 2 1 department of pediatric surgery, smt. Neuroglial heterotopia, heterotopic brain tissue, or differentiated neural tissue outside the cranial vault is uncommon, and these anomalies most commonly occur in the nasal cavity. Nasal glial heterotopia is a rare developmental abnormality, particularly rare in. The incidence of congenital midline nasal masses is rare and reported to be 1 in 20,00040,000 live births with.
Interpretations of heterotopia foucaults accounts of heterotopia remain briefly sketched, provisional and at times confusing. It must be distinguished from meningeal heterotopias that are more frequent and derived from the brain and the spinal cord meninges 1. Disruption of the neuroepithelial lining was even more marked below the heterotopia in the majority of e20 flnaknockdown brains fig. Importance heterotopic glial tissue is a rare, benign congenital anomaly that can present as a fibrous mass with or without overt signs of neural tissue origin, such as overlying alopecia, pigmentation, or a hair collar sign. We could not find any report of adult nasal glial heterotopias that developed cerebral. It was subsequently shown that heterotopia does not fit into haeckels interpretation of cenogenesis. Pdf glial heterotopias are rare, benign, congenital, midline. Glial heterotopia of the middle ear the journal of. Since 1998, has been providing free access to a large selection of texts. Nasal glial heterotopia develops from normal glial tissue, but is located in an abnormal. A rare case of nasal glial heterotopia presenting as. We report a neonate with extensive extranasal glial heterotopia involving the left buccopharyngeal region, palate and base of the skull and presenting with respiratory distress and a bleeding oral mass.
Not a true neoplasm but a malformation that cause tumorlike conditions in newborns and older infants. This is distinctly different from an encephalocele, which is a herniation of brain tissue andor leptomeninges, that develops through a defect in the skull, where there is a continuity with the cranial cavity. A case of nasal glial heterotopia in an adult hindawi. We report a rare case of extranasal glial heterotopia identified at birth. Discussion nasal glial heterotopia also known as nasal glioma is a rare developmental abnormality typically seen at birth or in early childhood. The rarity of nasal glial heterotopia as an embryologic anomaly is interesting but the twists and turns that followed the several changing impressions in managing this case made it even more interesting. For 2 years after surgery, the patient has been free from nasal symptoms. Most of these lesions develop in the head and neck area near the brain, in particular at the root of the nose nasal glioma.
Patients with focal subcortical heterotopia have a variable motor and intellectual disturbance depending on the size and site of the heterotopion. The heterotopia is capable of juxtaposing in a single real place several spaces, several sites that are in themselves incompatible. Esthesioneuroblastoma arises from the olfactory epithelium of the superior nasal cavity 3,24 and is most commonly a rapidly growing, aggressive neoplasm fig. Cases of this are rest homes and psychiatric hospitals, and of course prisons, and one should perhaps add retirement homes that are, as it were, on the borderline between the heterotopia of crisis and the heterotopia of deviation since, after all, old age is a crisis, but is also a deviation since in our society where leisure is the rule. Neuroglial heterotopia is a rare, nonhereditary malformative lesion, defined by the presence of an ectopic glial or neuroglial tissue outside the brain. Nasal glial heterotopia in a 1 year old child a case. Periventricular heterotopia may result from radial glial. The band heterotopia was relatively thick compared with that of other patients reported. Nasal glial heterotopia nasal glioma, the journal of. Nasal glial heterotopia ngh is a benign congenital malformation wherein abnormally located mature brain glial tissue presents as a mass on the forehead or nasal root area. The pathogenesis and differential diagnoses of this rare developmental disorder are discussed as is the importance of careful radiologic findings for appropriate surgical decision.
Nasopharyngeal glial heterotopia with intracranial. A 58yearold man had unilateral nasal obstruction that progressed for. Nov 16, 2011 glial heterotopias are rare, benign, congenital, midline, nonteratomatous extracranial glial tissue. Case report, clinical report by journal of evolution of medical and dental sciences.
Nasopharyngeal glial heterotopia with intracranial extension. The authors report a case of combined subcutaneous and intranasal glial heterotopia of the face in a 4monthold boy. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. These lesions are typically found in the midline area, though they have been found laterally involving the mastoid and middle ear regions. A rare case of nasal glial heterotopia presenting as sphenochoanal polyp. Radial glia in a 21wk fetus without pathological anomalies. Glial heterotopia of the face, journal of pediatric surgery.
Health, general birth defects care and treatment diagnosis encephalocele glia abnormalities neuroglia. The neurons in heterotopia appear to be normal, except for their mislocation. Mixed heterotopic gastrointestinal cyst and extranasal glial. Nasal glial heterotopia, also called nasal glioma, is a rare developmental abnormality, which is probably related to congenital encephalocele. Gray matter heterotopias are neurological disorders caused by clumps of gray matter nodules of neurons located in the wrong part of the brain. A grey matter heterotopia is characterized as a type of focal cortical dysplasia. Glial choristoma of the temporal bone in a 7monthold. Glial heterotopia or the occurrence of isolated nonteratomatous extracranial glial tissue is rare.
Glial heterotopias are rare, benign, congenital, midline, nonteratomatous extracranial glial tissue. Nhl medical college and vadilal sarabhai general hospital, ahmedabad, gujarat, india 2 department of plastic surgery, smt. Nasal gliomas, also know as nasal glial heterotopia, are a rare congenital lesion composed of dysplastic glial cells which have lost their intracranial connections and present as an extranasal or intranasal mass. Nasal glial heterotopia is a midline nasofrontal mass composed of mature brain tissue isolated from the cranial cavity by closure of the cranial sutures during embryonic development.
Oct 06, 2015 periventricular heterotopia is a condition in which the nerve cells neurons do not migrate properly during early development of the fetal brain. People with this condition typically develop recurrent seizures epilepsy beginning in midadolescence. Heterotopic neural nodules is a type of neuroectodermal malformation. A 55yearold woman presented with a 2month history of left sided nasal obstruction. Pdf glial heterotopia of the middle ear researchgate. Johns medical college and hospital, bangalore 560 034, india click here for correspondence address and email. Rarity of this condition makes clinical level diagnosis a challenge. Cases of neuroglial heterotopia have been described in various sites with nose and nasopharynx being the commonest. We report a case of nasal glial heterotopia in an adult man. Vimentin immunolabeling highlights the radial glial architecture. Extranasal glial heterotopia is a type of congenital lesion that is rarely reported in the literature. Nhl medical college and vadilal sarabhai general hospital, ahmedabad, gujarat, india.
Rigid endoscopy showed greyish left nasal polyp and anterior discharge. Ballard christopher james duffy submitted in accordance with the requirements for a degree of doctor of philosophy the university of leeds school of english november 2015. Nasal glial heterotopia with cleft palate chandna s, mehta ma. It can be divided according to morphology into 2 unilateral focal. Heterotopic glial heterotopia, sphenoid sinus, nasal cavity, sphenochoanal polyp 1. Heterotopia definition of heterotopia by medical dictionary. Case report, case study by turkish archives of otorhinolaryngology. After the surgery, frontal lobe cerebral hemorrhage developed. We firstly removed the nasal portion and subsequently opened sphenoid sinus and completely removed the rest of the polyp. Over time the tumor involves the nasal cavity more extensively and most commonly spreads locally into the paranasal sinuses, orbit, and anterior cranial fossa via the cribriform plate. Glial heterotopia, nasal glioma introduction glial heterotopia is a benign, rare and congenital condition characterized by the presence of central nervous system tissue at places other than the cranial cavity bhadani et al.
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