{"orpha":137926,"name":"Primary laryngeal lymphangioma","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Primary laryngeal lymphangioma is a rare, benign, congenital malformation of the lymphatic system characterized by a polypoidal, variable-sized, soft tissue mass located in the larynx. Most lesions manifest by the 2nd year of life and, depending on the size, patients may present with changes in voice, dysphagia, stridor, airway obstruction and/or respiratory distress. Cystic hygroma of the neck is frequently associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137926/","orphanet_url":"https://www.orpha.net/en/disease/detail/137926","names":{"fr":{"name":"Lymphangiome laryngé primaire","definition":"Le lymphangiome laryngé primaire est une malformation congénitale bénigne et rare du système lymphatique caractérisé par une masse polypoïde de tissu mou, de taille variable, située dans le larynx. La plupart des lésions surviennent au cours de la deuxième année de vie, et en fonction de leur taille, les patients peuvent présenter des changements de voix, une dysphagie, un stridor, une obstruction des voies aériennes et/ou une détresse respiratoire. Un hygrome kystique du cou est fréquemment associé."},"de":{"name":"Lymphangiom, laryngeales, primäres","definition":"Das primäre laryngeale Lymphangiom ist eine seltene, gutartige, angeborene Fehlbildung des lymphatischen Systems, die durch eine polypoide, unterschiedlich große Weichteilmasse im Kehlkopf gekennzeichnet ist. Die meisten Läsionen manifestieren sich bis zum 2. Lebensjahr und je nach Größe können die Patienten mit Stimmveränderungen, Dysphagie, Stridor, Atemwegsobstruktion und/oder Atemnot auftreten. Ein zystisches Hygrom am Hals ist häufig assoziiert."},"es":{"name":"Linfangioma primario de laringe","definition":"El linfangioma primario de laringe es una malformación congénita, benigna y poco frecuente del sistema linfático caracterizada por una masa polipoide de tejido blando y tamaño variable localizada en la laringe. La mayoría de las lesiones se manifiestan antes de los 2 años de vida y, dependiendo del tamaño, los afectados pueden presentar cambios en la voz, disfagia, estridor, obstrucción de las vías aéreas y/o disfunción respiratoria. Generalmente, se asocia con higromas quísticos del cuello."},"it":{"name":"Linfangioma primario della laringe","definition":"Il linfangioma primario della laringe è una malformazione congenita rara benigna del sistema linfatico, caratterizzata dalla presenza di una massa di tessuto molle polipoide di grandezza variabile localizzata nella laringe. La maggior parte delle lesioni si manifesta prima del secondo anno di vita e, a seconda delle loro dimensioni, i pazienti possono presentare alterazioni della voce, disfagia, stridore, ostruzione delle vie aeree e/o distress respiratorio. Spesso si associa a igroma cistico del collo."},"nl":{"name":"Primair laryngeaal lymfangioom","definition":"Primair laryngeaal lymfangioom is een zeldzame, goedaardige, congenitale malformatie van het lymfevatenstelsel en wordt gekarakteriseerd door een polypoïde massa met een variabele omvang in het zachte weefsel van de larynx. De meeste letsels manifesteren zich in het tweede levensjaar en afhankelijk van de omvang van de massa kunnen zich stemveranderingen, dysfagie, stridor, obstructie van de luchtwegen en/of ademnood voordoen bij patiënten. Regelmatig is cystisch hygroom van de nek geassocieerd."},"pt":{"name":"Linfangioma laringeo primário"},"pl":{"name":"Pierwotny naczyniak chłonny krtani"},"cs":{"name":"Primární laryngeální lymfangiom"},"uk":{"name":"Первинна лімфангіома гортані","definition":"Primary laryngeal lymphangioma is a rare, benign, congenital malformation of the lymphatic system characterized by a polypoidal, variable-sized, soft tissue mass located in the larynx. Most lesions manifest by the 2nd year of life and, depending on the size, patients may present with changes in voice, dysphagia, stridor, airway obstruction and/or respiratory distress. Cystic hygroma of the neck is frequently associated."},"zh":{"name":"原发性喉淋巴管瘤","definition":"原发性喉淋巴管瘤是一种罕见的良性先天性淋巴系统畸形，其特征是位于喉部的息肉状、大小不等的软组织肿块。大多数病变在2岁时出现，根据肿块大小，患者可能会出现声音变化、吞咽困难、喘鸣、气道阻塞和/或呼吸窘迫。常伴有颈部囊性淋巴管瘤。"},"tr":{"name":"Primer laringeal lenfanjiyom"}},"xrefs":[{"source":"ICD-10","code":"D18.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0015314","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4706521","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19891","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":156249,"name":"Larynx anomaly","classifications":[147,200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[],"phenotypes":null}