{"orpha":141112,"name":"Nasal glial heterotopia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Nasal glioma"],"definition":"Nasal glial heterotopia is a rare developmental abnormality presenting usually at birth or in early childhood (rarely in adulthood) as a benign, non-pulsatile mass that can lead to nasal obstruction, deformation of the septum and nasal bone, and respiratory distress if untreated. Nasal glial heterotopias have no communication with the central nervous system; however an associated defect in the cribriform plate is sometimes reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141112/","orphanet_url":"https://www.orpha.net/en/disease/detail/141112","names":{"fr":{"name":"Hétérotopie gliale nasale","synonyms":["Gliome nasal"],"definition":"L'hétérotopie gliale nasale est une anomalie rare du développement qui se manifeste habituellement à la naissance ou en début d'enfance (rarement à l'âge adulte). Elle se caractérise par une masse bénigne non pulsatile pouvant conduire à une obstruction nasale, une déformation du septum nasal et de l'os nasal, ainsi qu'à une détresse respiratoire en l'absence de traitement. L'hétérotopie gliale nasale ne communique pas avec le système nerveux central, cependant, une anomalie de la plaque cribriforme est parfois associée."},"de":{"name":"Gliom, nasales","synonyms":["Nasengliom"]},"es":{"name":"Heterotopia glial nasal","synonyms":["Glioma nasal"],"definition":"Es una anomalía poco frecuente del desarrollo que, por lo general, se presenta al nacimiento o en la infancia temprana (excepcionalmente en la edad adulta) con una masa benigna no pulsátil que puede derivar en obstrucción nasal, deformación del tabique y del hueso nasal, y dificultad respiratoria, en ausencia de tratamiento. Estas formaciones no tienen comunicación con el sistema nervioso central; sin embargo, se ha descrito en ocasiones un defecto asociado en la placa cribriforme."},"it":{"name":"Eterotopia gliale nasale","synonyms":["Glioma nasale"],"definition":"L'eterotopia gliale nasale è un'anomalia rara dello sviluppo, che di solito esordisce alla nascita o nella prima infanzia (raramente nell'età adulta), sotto forma di una massa benigna non pulsante che, se non trattata, può causare ostruzione nasale, deformazione del setto e dell'osso nasale e distress respiratorio. Le eterotopie gliali nasali non comunicano con il sistema nervoso centrale; tuttavia in alcuni casi è stato segnalato un concomitante difetto della lamina cribriforme."},"nl":{"name":"Nasale gliale heterotopie","synonyms":["Nasaal glioom"],"definition":"Nasale gliale heterotopie is een zeldzame ontwikkelingsanomalie en komt doorgaans tot uiting bij de geboorte of in de vroege kindertijd (zelden in de volwassenheid) als een goedaardige, niet-pulserende massa die kan leiden tot nasale obstructie, misvorming van het neustussenschot en het neusbeen, en ademnood wanneer het onbehandeld blijft. Nasale gliale heterotopieën staan niet in verbinding met het centrale zenuwstelsel, hoewel soms een geassocieerd defect aan de zeefplaat van het zeefbeen wordt gerapporteerd."},"pt":{"name":"Glioma nasal"},"pl":{"name":"Nosowa heterotopia glejowa","synonyms":["Glejak nosa"]},"cs":{"name":"Nosní gliální heterotopie","synonyms":["Nosní gliom"]},"uk":{"name":"Носова гліальна гетеротопія","synonyms":["Гліома носа"],"definition":"Nasal glial heterotopia is a rare developmental abnormality presenting usually at birth or in early childhood (rarely in adulthood) as a benign, non-pulsatile mass that can lead to nasal obstruction, deformation of the septum and nasal bone, and respiratory distress if untreated. Nasal glial heterotopias have no communication with the central nervous system; however an associated defect in the cribriform plate is sometimes reported."},"zh":{"name":"鼻部神经胶质异位","synonyms":["鼻胶质瘤"],"definition":"鼻部神经胶质异位是一种罕见的发育异常，通常在出生时或儿童早期（成人期少见）表现为良性、非搏动性肿块，若不治疗可导致鼻塞、鼻中隔和鼻骨变形和呼吸窘迫。鼻部神经胶质异位与中枢神经系统无联系；但有时会出现筛板内相关缺陷。"},"tr":{"name":"Nazal glial heterotopi","synonyms":["Burun gliomu"]}},"xrefs":[{"source":"ICD-10","code":"Q30.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA70.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1449384774"},{"source":"MONDO","code":"0015392","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0266490","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19949","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":156246,"name":"Nose and cavum anomaly","classifications":[147,200]},{"orpha":98061,"name":"Rare otorhinolaryngologic tumor","classifications":[200,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}