title: [Significance of the "V" syndrome with double "up shoot". Insufficiency of the two superior oblique muscles in craniofacial malformations].
abstract: Craniofacial malformations (telorbitism, craniofaciostenosis, plagiocephalia, Franceschetti's syndrome) are frequently associated with oculomotor anomalies. The most common vertical anomaly is the existence of a "V" syndrome with double "up shoot", with deficiencies in the two superior oblique muscles. Several mechanisms may be involved in the origin of this anomaly, related to the anatomical orbital malformation: orbital torsion with antimongoloid clefts responsible for a syndrome of macular pseudo-ectopia with extorsion of the 4 rectus muscles; plagiocephalia responsible for sagitallization of the inferior obliques; retromaxillia with exorbitism responsible for modification of muscle contact arches. Muscle agenesis is very rarely observed. The pathogenesis of these different mechanisms is discussed based on a study of 64 cases.
mesh_terms: D003394:Craniofacial Dysostosis / Q000150:complications*; D005133:Eye Movements; D006801:Humans; D009801:Oculomotor Muscles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery; D009915:Orbit / Q000002:abnormalities; D013285:Strabismus / Q000150:complications*; D013577:Syndrome
vernacular_title: Signification du syndrome V avec double "up shoot". Insuffisance des deux grands obliques au cours des malformations cranio-faciales.