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January 1974

Picture of the Month

Am J Dis Child. 1974;127(1):85-86. doi:10.1001/archpedi.1974.02110200087012

Denouement and Discussion 

Median Cleft Face Syndrome (Frontonasal Dysplasia) 

Manifestations  Major manifestations include ocular hypertelorism, broad bridge of the nose, widow's peak, anterior cranium bifidum occultum, and clefts involving the nose, lip, or palate.The most consistent finding is ocular hypertelorism, which can be determined clinically by an increased interpupillary distance or increased interorbital distance on roentgenographic examination. Microphthalmia, epibulbar dermoids, colobomas of the upper lid, and congenital cataracts have also been reported. Abnormalities of the nose include a broad bridge, flattened nose, lateral notching of the ala nasi, absence of the nasal tip, and asymmetry of the nostrils. If the median clefting is severe, the nose may be separated into two parts. The widow's peak, which may be present in infancy, is a V-shaped protrusion of hair extending to the center of the forehead. The anterior cranium bifidum occultum is manifested by a bony indentation of the forehead. Cleft lip, which is found more frequently than cleft palate,

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