June 1968

Picture of the Month

Author Affiliations

Boston; Los Angeles
From the Childrens Hospital of Los Angeles.

Am J Dis Child. 1968;115(6):719-720. doi:10.1001/archpedi.1968.02100010721014

Denouement and Discussion 

Apert's Syndrome  (Acrocephalosyndactyly)

Manifestations  The major manifestations include premature closure of cranial sutures and syndactyly of the hands and feet. Because of closure of the coronal sutures the calvarium is lengthened vertically and decreased in the anteroposterior diameter, resulting in a flattened occiput and a prominent frontal area. There is hypertelorism, an antimongoloid slant, and bulging of the eyes secondary to the shallow orbits. Facial dysostosis consisting of hypoplasia of the maxillae, a prominent mandible, high-arched narrow palate and crowded teeth, an open mouth secondary to nasal obstruction, and occasionally a cleft palate are present.The syndactyly is marked and resembles a "mitten hand" or "sock foot." There is usually a complete fusion of the distal soft tissues and occasionally of the bones. The thumbs and big toes may or may not be involved in the fusion. Other skeletal anomalies include hallux varus, ankylosis of major joints,

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