[Significance of the "V" syndrome with double "up shoot". Insufficiency of the two superior oblique muscles in craniofacial malformations].

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.