{"orpha":645310,"name":"Fibroneural non-saccular limited dorsal myeloschisis","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Fibroneural flat LDM","Fibroneural flat limited dorsal myeloschisis","Fibroneural non-saccular LDM"],"definition":"A rare non-saccular limited dorsal myeloschisis, in which the stalk histology is characterized by skin tissue with dermo-epidermal epithelium in continuity with a subcutaneous fibrocollagenous tract containing various ectopic tissues.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-645310/","orphanet_url":"https://www.orpha.net/en/disease/detail/645310","names":{"fr":{"name":"Myéloschisis dorsal limité non-sacculaire de type fibroneural","synonyms":["LDM non-sacculaire de type fibroneural","LDM plat de type fibroneural","Myéloschisis dorsal limité plat de type fibroneural"],"definition":"Forme rare de myéloschisis dorsal limité (LDM) non sacculaire dont la tige est caractérisée histologiquement par du tissu cutané défini par un épithélium dermo-épidermique en continuité avec un tractus fibrocollagène contenant divers tissus ectopiques."},"de":{"name":"Fibroneurale nicht-sakkuläre begrenzte dorsale Myeloschisis","synonyms":["Fibroneurale flache LDM","Fibroneurale flache begrenzte dorsale Myeloschisis","Fibroneurale nicht-sakkuläre LDM"],"definition":"Eine seltene nicht-sakkuläre begrenzte dorsale Myeloschisis, bei der die Stielhistologie durch Hautgewebe mit dermo-epidermalem Epithel in Kontinuität mit einem subkutanen fibro-kollagenen Trakt gekennzeichnet ist, der verschiedene ektopische Gewebe enthält."},"es":{"name":"Mielosquisis dorsal limitada no sacular fibroneural","synonyms":["MDL no sacular fibroneuronal","MDL plana fibroneural","Mielosquisis dorsal limitada plana fibroneuronal"],"definition":"Es una mielosquisis dorsal limitada no sacular, poco frecuente, en la que la histología del tallo se caracteriza por tejido cutáneo con epitelio dermoepidérmico en continuidad con un tracto fibrocolagenoso subcutáneo que contiene varios tejidos ectópicos."},"it":{"name":"Mieloschisi fibroneurale non sacculare limitata al dorso","synonyms":["LDM non sacculare fibroneurale","LDM piatta fibroneurale","Mieloschisi fibroneurale piatta limitata al dorso"]},"nl":{"name":"Fibroneurale niet-sacculaire beperkte dorsale myeloschisis","synonyms":["Fibroneurale niet-sacculaire LDM","Fibroneurale platte LDM","Fibroneurale platte beperkte dorsale myeloschisis"],"definition":"Een zeldzame niet-sacculaire beperkte dorsale myeloschisis, met een steel histologisch gekenmerkt door huidweefsel met dermo-epidermaal epitheel dat overgaat in een subcutaan fibrocollageneus stelsel dat verschillende ectopische weefsels bevat."},"pt":{"name":"Mielosquisis fibroneural não-sacular dorsal limitada","synonyms":["LDM fibroneural não-sacular","LDM fibroneural plana","Mielosquisis fibroneural plana dorsal limitada"],"definition":"Mielosquise dorsal limitada não sacular rara, na qual a histologia do pedúnculo é caracterizada por tecido cutâneo com epitélio dermoepidérmico em continuidade com um trato fibrocolagenoso subcutâneo contendo vários tecidos ectópicos."},"pl":{"name":"Włóknisto-nerwowy nieworkowaty ograniczony rozszczep grzbietowej części rdzenia kręgowego","synonyms":["Fibroneural flat LDM","Fibroneural flat limited dorsal myeloschisis","Fibroneural non-saccular LDM"],"definition":"Rzadki, nieworkowaty, ograniczony rozszczep rdzenia kręgowego, w którym budowa histologiczna szypuły charakteryzuje się obecnością tkanek skóry z nabłonkiem skórno-naskórkowym połączonym z podskórnym pasmem włóknisto-kolagenowym zawierającym różne tkanki ektopowe."},"cs":{"name":"Fibroneurální ohraničená dorzální myeloschíza bez vaku","synonyms":["Fibroneural flat LDM","Fibroneural flat limited dorsal myeloschisis","Fibroneural non-saccular LDM"]},"uk":{"name":"Фіброневрологічний несакральний обмежений дорсальний мієлошизис","synonyms":["Фіброневрологічний плоский LDM","Фіброневральний плоский обмежений дорзальний мієлошизис","Фіброневротична несакральна LDM"],"definition":"A rare non-saccular limited dorsal myeloschisis, in which the stalk histology is characterized by skin tissue with dermo-epidermal epithelium in continuity with a subcutaneous fibrocollagenous tract containing various ectopic tissues."}},"xrefs":[{"source":"MONDO","code":"0970961","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5816724","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":645343,"name":"Non-saccular limited dorsal myeloschisis","classifications":[147,156,181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":[],"prevalence":[],"phenotypes":null}