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July 1968

Picture of the Month

Am J Dis Child. 1968;116(1):81-82. doi:10.1001/archpedi.1968.02100020083012

Denouement and Discussion 

Lip Pits-Cleft Lip and Palate-Popliteal Pterygia Syndrome 

Manifestations  Cleft lip and palate, lip pits, popliteal pterygia, and genital abnormalities are the major manifestations. There are various types and degrees of cleft lip and palate. The lip pits, usually two in number, are circular depressions located near the midline on the vermilion border of the lower lip. A fistulous tract is present in the center of the depression. There may be oral webbing consisting of bands extending from the alveolar ridges. Enamel hypoplasia and a short upper lip may be present. The genitalia may show an enlarged clitoris, absent labia, bidfid scrotum, cryptorchidism, or ambiguous genitalia. The popliteal pterygia, usually bilateral, vary in severity, with the most severe extending from the heel to the upper thigh, causing inability to extend the leg. Dysplasia of the toenails is frequently found. Other abnormalities include syndactyly, hypoplasia, or aplasia