Hypertelorism is defined clinically as increased distance between the pupils. It is detected sonographically by maeasuring the distance between orbits. A multitude of syndromes are associated with hypertelorism, and most have in common cranial contour abnormalities, such as those caused by craniosynostosis. Most of these syndromes are also associated with limb and digit anomalsies. Hypoplastic greater wings of the sphenoid and overgrowth of the lesser wings of the sphenoid are said to be responsible for the defect. Overgrowth of the nasofrontal process of the frontal bone may also be responsible. A mass lesion such as frontal encephalocele may space the orbits wide apart and prevent them from reaching their normal anatomical position.
· Frontal bossing of skull-
Gorlin’s syndrome (basal nevus
Otopalatodigital syndrome type I
Cleidocranial dysostosis Acrocephalosyndactyly Apert type)
Noonan’s syndrome (also macrocephaly)
Marden’s syndrome (also cleft lip).
· Broad/square forhead-
Treacher Collins syndrome
Larsen’s syndrome (also cleft palate and uvula)
Chromosome i8p syndrome (broad nose, flat face)
· Mass lesion forhead/midupper face-
Median cleft face syndrome
Glioma of nose
Nose and nasal septum defect
Fetal hydantoin syndrome.
· Delayed cranial ossifaction-
Ehlers—Danlos syndrome (flat orbit).
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