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June 1976

Picture of the Month

Author Affiliations

From the Center for Genetic Counseling and Birth Defect Evaluation, New England Medical Center Hospital, Boston, and the Center for Craniofacial Anomalies, University of Illinois, Chicago.

Am J Dis Child. 1976;130(6):653-654. doi:10.1001/archpedi.1976.02120070079015

Denouement and Discussion 

The EEC Syndrome 

Manifestations  Major manifestations are ectrodactyly (E), ectodermal dysplasia (E), and cleft of the lip and palate (C).Typical facial findings are midfacial hypoplasia, prominent supraorbital ridges and nasal bridge, bilateral complete cleft lip, and complete cleft palate. Associated ocular abnormalities include chronic recurrent blepharitis, photophobia, and decreased tear formation. There is a deficiency of eyelashes, scarring of the lacrimal puncta, cicatricial narrowing of the palpebral fissures, and a deficiency of Meibomian gland orifices. Varying degrees of bilateral superficial corneal vascularization may also be present. Xerostomia and absence of Stensen ducts have been reported. There is high susceptibility to caries and an increased incidence of Candida albicans infection of the oral mucosa. A mild to moderate degree of conduction hearing loss is a common association.The most common type of limb malformations are clawhand and clawfoot deformities associated

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