{"orpha":620102,"name":"Non-syndromic unicoronal craniosynostosis","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Isolated frontal plagiocephaly","Isolated unicoronal craniosynostosis","Non-syndromic anterior synostotic plagiocephaly","Non-syndromic frontoparietal craniosynostosis","Non-syndromic hemicoronal craniosynostosis","Non-syndromic unilateral coronal synostosis"],"definition":"A form of non-syndromic unisutural craniosynostosis characterized by the premature fusion of one of the two coronal sutures of the newborn, leading to plagiocephaly with flattening of the ipsilateral side of the forehead and a compensatory bulging of the contralateral side. Ocular anomalies (strabismus, hypermetropia, astigmatism) due to orbital dystopia, and ocular torticollis, are commonly associated with in unicoronal craniosynostosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-620102/","orphanet_url":"https://www.orpha.net/en/disease/detail/620102","names":{"fr":{"name":"Craniosynostose coronale unilatérale non syndromique","synonyms":["Craniosynostose coronale unilatérale isolée","Craniosynostose unicoronale non syndromique"],"definition":"Forme de craniosynostose unisuturaire non syndromique caractérisée par une fusion prématurée de l'une des deux sutures coronales du nouveau-né, entraînant une plagiocéphalie avec aplatissement ipsilatéral du front et bombement compensatoire controlatéral. Des anomalies oculaires (strabisme, hypermétropie, astigmatisme) dues à une dystopie orbitaire et un torticolis d'origine oculaire sont fréquemment associés."},"de":{"name":"Kraniosynostose, unikoronale, nicht-syndromale","synonyms":["Anteriore synostotische Plagiozephalie, nicht-syndromale","Frontoparietale Kraniosynostose, nicht-syndromale","Hemikoronale Kraniosynostose, nicht-syndromale","Isolierte frontale Plagiozephalie","Isolierte unikoronale Kraniosynostose","Unilaterale koronale Synostose, nicht-syndromale"],"definition":"Eine Form der nicht-syndromalen unisuturalen Kraniosynostose, die durch die vorzeitige Fusion einer der beiden Koronalnähte des Neugeborenen gekennzeichnet ist und zu einer Plagiozephalie mit Abflachung der ipsilateralen Seite der Stirn und einer kompensatorischen Wölbung der kontralateralen Seite führt. Augenanomalien (Strabismus, Hypermetropie, Astigmatismus) aufgrund orbitaler Dystopie und okulärer Torticollis sind häufig mit einer unikoronalen Kraniosynostose verbunden."},"es":{"name":"Craneosinostosis unicoronal no sindrómica","synonyms":["Craneosinostosis frontoparietal no sindrómica","Craneosinostosis hemicoronal no sindrómica","Craneosinostosis unicoronal aislada","Plagiocefalia frontal aislada","Plagiocefalia sinostótica anterior no sindrómica","Sinostosis coronal unilateral no sindrómica"],"definition":"Es una forma de craneosinostosis unisutural no sindrómica caracterizada por la fusión prematura de una de las dos suturas coronales del recién nacido, lo que resulta en plagiocefalia con aplanamiento del lado ipsilateral de la frente y un abultamiento compensatorio del lado contralateral. Las anomalías oculares (estrabismo, hipermetropía, astigmatismo) debidas a distopía orbitaria y tortícolis ocular se asocian comúnmente con la craneosinostosis unicoronal."},"it":{"name":"Craniosinostosi monocoronale non sindromica","synonyms":["Craniosinostosi emicoronale non sindromica","Craniosinostosi frontoparietale non sindromica","Craniosinostosi monocoronale isolata","Plagiocefalia frontale isolata","Plagiocefalia sinostotica anteriore non sindromica","Sinostosi coronale monolaterale non sindromica"]},"nl":{"name":"Niet-syndromale unicoronale craniosynostose","synonyms":["Geïsoleerde frontale plagiocefalie","Geïsoleerde unicoronale craniosynostose","Niet-syndromale anterieure synostotische plagiocefalie","Niet-syndromale frontopariëtale craniosynostose","Niet-syndromale hemicoronale craniosynostose","Niet-syndromale unilaterale coronale synostose","Niet-syndromale unilaterale synostose van sutura coronalis"],"definition":"Een vorm van niet-syndromale unisuturale craniosynostose, gekenmerkt door premature fusie van een van de twee kroonnaden (sutura coronalis) van een neonaat, wat leidt tot plagiocefalie met afplatting van de ipsilaterale zijde van het voorhoofd en een compenserende uitpuiling van de contralaterale zijde. Ooganomalieën (strabisme, hypermetropie, astigmatisme) door dystopie van oogkas, en oculaire torticollis, zijn doorgaans geassocieerd met unicoronale craniosynostose."},"pt":{"name":"Craniossinostose unicoronal não-sindromática","synonyms":["Craniossinostose frontoparietal não-sindromática","Craniossinostose hemicoronal não-sindromática","Craniossinostose unicoronal isolada","Plagiocefalia frontal isolada","Plagiocefalia sinostótica anterior não-sindromática","Sinostose coronal unilateral não-sindromática"]},"pl":{"name":"Niesyndromiczna kraniosynostoza obejmująca szew wieńcowy","synonyms":["Isolated frontal plagiocephaly","Isolated unicoronal craniosynostosis","Non-syndromic anterior synostotic plagiocephaly","Non-syndromic frontoparietal craniosynostosis","Non-syndromic hemicoronal craniosynostosis","Non-syndromic unilateral coronal synostosis"],"definition":"Forma niesyndromicznej kraniosynostozy charakteryzująca się przedwczesnym zrośnięciem jednego z dwóch szwów wieńcowych u noworodka, co prowadzi do plagiocefalii ze spłaszczeniem ipsilateralnej strony czoła i kompensacyjnym wybrzuszeniem strony przeciwległej. Często występują wady narządu wzroku (zez, nadwzroczność, astygmatyzm) spowodowane dystopią oczodołową oraz kompensacyjny kręcz szyi."},"cs":{"name":"Nesyndromická unikoronární kraniosynostóza","synonyms":["Isolated frontal plagiocephaly","Isolated unicoronal craniosynostosis","Non-syndromic anterior synostotic plagiocephaly","Non-syndromic frontoparietal craniosynostosis","Non-syndromic hemicoronal craniosynostosis","Non-syndromic unilateral coronal synostosis"]},"uk":{"name":"Несиндромальний унікоронарний краніосиностоз","synonyms":["Ізольована лобова плагіоцефалія","Ізольований однокоронний краніосиностоз","Несиндромна передня синостотична плагіоцефалія","Несиндромний лобно-тімяний краніосиностоз","Несиндромний гемікоронарний краніосиностоз","Несиндромний односторонній коронарний синостоз"]}},"xrefs":[{"source":"ICD-10","code":"Q75.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB70.00","qualifier":"NTBT","icd_relation":"specific","validated":true,"icd11_uri":"1652888053"},{"source":"MONDO","code":"0850073","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680401","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":620096,"name":"Non-syndromic unisutural craniosynostosis","classifications":[147,156,199]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal recessive"],"onset":["Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1049,"area":"Worldwide","status":"Validated","source":"35636659[PMID]_38348760[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-10-29","source":"35207332[PMID]_37762999[PMID]_37834867[PMID]","terms":[{"hpo":"HP:3000030","label":"Abnormal morphology of bony orbit of skull","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000290","label":"Abnormality of the forehead","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000646","label":"Amblyopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000483","label":"Astigmatism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004484","label":"Craniofacial asymmetry","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011225","label":"Epiblepharon","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002007","label":"Frontal bossing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000486","label":"Strabismus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000473","label":"Torticollis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012803","label":"Anisometropia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000565","label":"Esotropia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000577","label":"Exotropia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030001","label":"Lagophthalmos","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001085","label":"Papilledema","frequency":"Very rare (<4-1%)","criteria":null}]}}