PEDIATRICS Vol. 41 No. 2 February 1968, pp. 503-509
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POPLITEAL PTERYGIUM SYNDROME

A Syndrome Comprising Cleft Lip-Palate, Popliteal and Intercrural Pterygia, Digital and Genital Anomalies

Robert J. Gorlin D.D.S., M.S.1, Heddie O. Sedano D.D.S., M.S.1, and J. Cervenka D.D.S., M.D.1

1 Division of Oral Pathology, University of Minnesota School of Dentistry, Minneapolis

The name of popliteal pterygium syndrome is suggested for a well defined complex that consists of popliteal pterygium (usually bilateral), intercrural pterygium, various digital anomalies that include hypoplasia or agenesis of digits, valgus or varus deformities of the feet and syndactyly, genitourinary anomalies (such as cryptorchidism, absent or cleft scrotum, inguinal hernia, hypoplasia or aplasia of the labia majora, and oral anomalies (such as cleft lip-palate, syngnathia and lower labial pits). There may also be filiform adhesion of the eyelids.

This syndrome is but one of perhaps 20 or more that involve clefting of the lip and/or palate. It is inherited as an autosomal dominant trait with variable expressivity. Rarely is it transmitted from affected parent to offspring due to genital hypoplasia. The authors point out the importance of the awareness of the surgeon who corrects the popliteal pterygium since the sciatic nerve runs beneath the fibrous septum that forms the free edge of the pterygium.

Submitted on June 27, 1967
Accepted on September 5, 1967