{"orpha":490,"name":"Omphalomesenteric cyst","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare non-syndromic diaphragmatic or abdominal wall malformation, a remnant of omphalomesenteric duct, characterized by cuboidal or columnar epithelium with gastrointestinal differentiation. Patients may be asymptomatic or present with infraumbilical mass, umbilical lesion with secretions, abdominal pain, hernia, abscess, gastrointestinal tract bleeding, intestinal obstruction, and acute abdomen.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-490/","orphanet_url":"https://www.orpha.net/en/disease/detail/490","names":{"fr":{"name":"Kyste omphalo-mésentérique","definition":"Malformation non syndromique rare du diaphragme ou de la paroi abdominale (absence d'involution du canal omphalomésentérique) caractérisée par un épithélium cuboïde ou cylindrique avec différentiation gastro-intestinale. Les patients peuvent être asymptomatiques ou présenter une masse sous-ombilicale, une lésion ombilicale avec présence de sécrétions, des douleurs abdominales, une hernie, un abcès, une hémorragie gastro-intestinale, une occlusion intestinale et un abdomen aigu."},"de":{"name":"Zyste, omphalomesenterische","definition":"Eine seltene nicht-syndromale Zwerchfell- oder Bauchwandfehlbildung, ein Überbleibsel des Ductus omphalomesentericus (Dottergang), gekennzeichnet durch quaderförmiges oder säulenförmiges Epithel mit gastrointestinaler Differenzierung. Die Patienten können asymptomatisch sein oder sich mit einer infraumbilikalen Masse, einer nabelartigen Läsion mit Sekretion, Bauchschmerzen, Hernie, Abszess, Blutungen im Magen-Darm-Trakt, Darmverschluss und akutem Abdomen vorstellen."},"es":{"name":"Quiste onfalo-mesentérico","definition":"Es una malformación no sindrómica y poco frecuente de la pared abdominal o diafragmática, que consiste en un remanente del conducto onfalomesentérico, caracterizado por epitelio cuboidal o columnar con diferenciación gastrointestinal. Los pacientes pueden permanecer asintomáticos o presentarse con una masa infraumbilical, lesión umbilical con secreciones, dolor abdominal, hernia, absceso, hemorragia del tracto gastrointestinal, obstrucción intestinal y abdomen agudo."},"it":{"name":"Cisti onfalo-mesenterica","definition":"Si tratta di una malformazione rara non sindromica della parete diaframmatica o addominale, un residuo del dotto onfalo-mesenterico, caratterizzata da un epitelio cuboidale o colonnare con differenziazione gastrointestinale. I pazienti possono essere asintomatici o presentare una massa intraombelicale, una lesione ombelicale con secrezioni, dolore addominale, ernia, ascessi, emorragie del tratto gastrointestinale, ostruzione intestinale e addome acuto."},"nl":{"name":"Omfalomesenterische cyste","definition":"Een zeldzame, niet-syndromale malformatie van het diafragma of de buikwand, een overblijfsel van de omfalomesenterische ductus, gekarakteriseerd door cuboïdaal of columnair epitheel met gastro-intestinale differentiatie. Patiënten kunnen asymptomatisch zijn of zich presenteren met een infra-umbilicale massa, umbilicaal letsel met secreties, buikpijn, hernia, abces, bloedingen aan het maag-darmstelsel, darmobstructie, en acute buik."},"pt":{"name":"Quísto onfalo-mesentérico"},"pl":{"name":"Torbiel pępkowo-jelitowa"},"cs":{"name":"Omfalomezenterická cysta"},"uk":{"name":"Омфаломезентеріальна кіста","definition":"A rare non-syndromic diaphragmatic or abdominal wall malformation, a remnant of omphalomesenteric duct, characterized by cuboidal or columnar epithelium with gastrointestinal differentiation. Patients may be asymptomatic or present with infraumbilical mass, umbilical lesion with secretions, abdominal pain, hernia, abscess, gastrointestinal tract bleeding, intestinal obstruction, and acute abdomen."},"zh":{"name":"卵黄管囊肿"},"tr":{"name":"Omfalomesenterik kist"}},"xrefs":[{"source":"ICD-10","code":"Q43.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB03.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"670570793"},{"source":"MONDO","code":"0018847","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0266180","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4081","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":108977,"name":"Non-syndromic diaphragmatic or abdominal wall malformation","classifications":[147,182]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":4.0,"area":"Worldwide","status":"Validated","source":"24353646[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"24353646[PMID]_ORPHANET"}],"phenotypes":null}