Chondroectodermal dysplasia (Ellis van Creveld syndrome): a report of three cases with review of literature.

Chondroectodermal dysplasia is a rare mesenchymal - ectodermal dysplasia first described in 1940 by Richard W.B. Ellis and Simon van Creveld now known as Ellis van Creveld syndrome. It is also known as Mesvectodermal dysplasia. Majority of cases were characteristically seen in one particular inbred population from the Amish community of Lancaster County, Pennsylvania, U.S.A. The syndrome manifests with several skeletal anomalies, oral mucosal and dental anomalies, congenital cardiac defects and nail dysplasia. Ellis van Creveld syndrome may be differentiated from other chondrodystrophies like achondroplasia, chondroplasia punctata, asphyxiating thorasic dystrophy and Morquio's syndrome. The presence of oral mucosal and dental alterations like notching of the lower alveolar process, fusion of the upper lip with gingival mucosal margin, occasional presence of neonatal teeth, oligodontia and conical shape of anterior teeth will confirm the diagnosis of Ellis van Creveld syndrome and hence its importance to dentists.

Facial hemiatrophy--a report of 5 cases.

Progassive hemifacial atrophy is an uncommon and poorly understood degenerative condition characterized by atrophic changes involving the entire one side of the face, head and associated structures and typically appearing during the growth spurts to young adulthood. The condition involves both soft tissues and bone of the affected side. Intraorally the tongue, lips and salivary glands may be involved. Developing teeth may also show incomplete root development deleyed eruption. The couse of the condition is obscure or unknown although speculation has considered trophic malfunction of the cervical sympathetic nervous system, a history of prior trauma has been documented in some cases, although other reports have considered a viral or borrelial infection. It is usualy sporadic but a few familial cases have been reported suggetsting a possible hereditary influence and many investigators believe it to be a localized from of scleroderma. Five cases of Hemifacial Atrophy (Parry Romberg Syndrom) has been reported and discused.