{"orpha":137932,"name":"Congenital laryngeal palsy","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Congenital vocal cord paralysis"],"definition":"Congenital laryngeal palsy is a rare larynx anomaly characterized by unilateral or bilateral paralysis of the vocal cords as a result of dysfunction of the motor nerve supply to the larynx. Patients typically present at birth (or shortly thereafter) with stridor, weak or breathy cry, dysphonia or aphonia, feeding or aspiration difficulties and, occasionally, respiratory compromise. Neurological disease, masses that cause compression and aberrant vessels are often associated. Most cases resolve spontaneously over 6-12 months.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137932/","orphanet_url":"https://www.orpha.net/en/disease/detail/137932","names":{"fr":{"name":"Paralysie laryngée congénitale","synonyms":["Paralysie congénitale des cordes vocales"],"definition":"La paralysie laryngée congénitale est une anomalie rare du larynx caractérisée par la paralysie unilatérale ou bilatérale des cordes vocales, suite à un défaut de distribution des nerfs moteurs dans le larynx. Habituellement, les patients présentent à la naissance (ou peu de temps après) un stridor, des cris faibles ou haletants, une dysphonie ou une aphonie, des difficultés pour s'alimenter ou aspirer et parfois, une insuffisance respiratoire. Une maladie neurologique est souvent associée, ainsi que des masses causant une compression et des vaisseaux aberrants. La plupart des cas se résolvent de manière spontanée au bout de six à douze mois."},"de":{"name":"Larynxparalyse, kongenitale","definition":"Die kongenitale Larynxparalyse ist eine seltene Kehlkopfanomalie, die durch eine ein- oder beidseitige Lähmung der Stimmbänder als Folge einer Störung der motorischen Nervenversorgung des Kehlkopfes gekennzeichnet ist. Die Patienten präsentieren sich typischerweise bei der Geburt (oder kurz danach) mit Stridor, schwachem oder atemlosem Schrei, Dysphonie oder Aphonie, Fütterungs- oder Aspirationsschwierigkeiten und gelegentlich einer Beeinträchtigung der Atmung. Neurologische Erkrankungen, Massen, die eine Kompression verursachen, und aberrante Gefäße sind oft assoziiert. Die meisten Fälle klingen spontan über 6-12 Monate ab."},"es":{"name":"Parálisis congénita de la laringe","synonyms":["Parálisis congénita de cuerdas vocales","Parálisis congénita de las cuerdas bucales"],"definition":"La parálisis congénita de la laringe es una anomalía poco frecuente de la laringe caracterizada por parálisis uni- o bilateral de las cuerdas vocales como resultado de la disfunción de los nervios motores que inervan la laringe. Típicamente, los afectados presentan al nacimiento (o poco después) estridor, llanto débil o entrecortado, disfonía o afonía, dificultades para alimentarse o aspirar y, ocasionalmente, compromiso respiratorio. A menudo está asociada a enfermedades neurológicas, masas que provocan comprensión y vasos aberrantes. La mayoría de los casos se resuelven espontáneamente en un plazo de 6-12 meses."},"it":{"name":"Paralisi laringea congenita","definition":"La paralisi laringea congenita è un'anomalia rara della laringe, caratterizzata da paralisi mono- o bilaterale delle corde vocali, causata da una disfunzione dei nervi motori a livello della laringe. In genere esordisce alla nascita (o poco dopo) con stridore, pianto debole o ansimante, disfonia o afonia, difficoltà di alimentazione o di aspirazione e, raramente, compromissione respiratoria. Spesso si associano malattie neurologiche, masse che causano compressione e vasi aberranti. La maggior parte dei casi si risolve spontaneamente nell'arco di 6 -12 mesi."},"nl":{"name":"Congenitale laryngeale paralyse","synonyms":["Congenitale laryngeale verlamming","Congenitale stembandverlamming"],"definition":"Congenitale laryngeale paralyse is een zeldzame anomalie van het strottenhoofd dat gekarakteriseerd wordt door unilaterale of bilaterale verlamming van de stembanden ten gevolge van een dysfunctie van de motorische zenuwen die de larynx innerveren. Doorgaans vertonen patiënten bij de geboorte (of kort daarna) stridor, een zwakke of hese schreeuw, dysfonie of afonie, moeilijkheden met voeden en aspireren en, soms, een verstoorde ademhaling. Vaak zijn neurologische ziekten, gezwellen die compressie veroorzaken en afwijkingen aan bloedvaten geassocieerd. De meeste gevallen genezen spontaan na 6-12 maanden."},"pt":{"name":"Paralisia laríngea, congénita"},"pl":{"name":"Wrodzone porażenie krtani","synonyms":["Wrodzony paraliż strun głosowych"]},"cs":{"name":"Vrozená laryngeální paralýza","synonyms":["Vrozená obrna hlasivek"]},"uk":{"name":"Вроджений параліч гортані","synonyms":["Вроджений параліч голосових звязок"],"definition":"Congenital laryngeal palsy is a rare larynx anomaly characterized by unilateral or bilateral paralysis of the vocal cords as a result of dysfunction of the motor nerve supply to the larynx. Patients typically present at birth (or shortly thereafter) with stridor, weak or breathy cry, dysphonia or aphonia, feeding or aspiration difficulties and, occasionally, respiratory compromise. Neurological disease, masses that cause compression and aberrant vessels are often associated. Most cases resolve spontaneously over 6-12 months."},"zh":{"name":"先天性喉麻痹","synonyms":["先天性声带麻痹"],"definition":"先天性喉麻痹是一种罕见的喉部异常，其特征是喉部运动神经功能障碍导致的单侧或双侧声带麻痹。患者通常在出生时（或出生后不久）出现喘鸣、微弱或缺少声量的啼哭、发声困难或失声、进食或吸气困难，偶尔出现呼吸困难。通常与神经系统疾病，可导致压迫的肿块和异常血管相关。大多数病例会在6-12个月内自然消退。"},"tr":{"name":"Doğumsal laringeal felç","synonyms":["Doğumsal vokal kord paralizi"]}},"xrefs":[{"source":"ICD-10","code":"J38.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA71.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1508780420"},{"source":"MONDO","code":"0015316","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0396058","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12713","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10087175","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":182086,"name":"Acquired peripheral neuropathy","classifications":[181]},{"orpha":156249,"name":"Larynx anomaly","classifications":[147,200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Neonatal"],"prevalence":[],"phenotypes":null}