Denouement and Discussion
Cranioectodermal Dysplasia
Manifestations
Major manifestations consist of abnormalities of the skull, hair, face, teeth, and limbs. The head is relatively large in comparison to stature, which is in the low-normal range. Dolichocephaly, frontal bossing, and sagittal suture synostosis frequently occur. The hair is fine, sparse, and slow growing. Abnormalities of the teeth, which may be widely spaced, include microdontia and hypodontia, fusion, and enamel dysplasia. The limbs are short, with the arms more affected than the legs. Skin dimples may occur at the elbows and knees. The hands are small, with very short fingers, fifth-finger clinodactyly, cutaneous syndactyly, broad short nails, and aberrant palmar creases. Foot abnormalities include short toes, increased space between the first and second toes, cutaneous syndactyly of the second and third toes, bilateral hallucal plantar creases, a single flexion crease on each toe, and hallux valgus. Other skeletal abnormalities consist of a short narrow thorax, pectus excavatum, and hyperextensible