Cardiac anomalies complicating congenital contractural arachnodactyly.

Cardiac anomalies complicating congenital contractural arachnodactyly.

A newborn boy with congenital contractural arachnodactyly (CCA) was found to have an atrial septal defect, ventricular septal defect, patent ductus arteriosus, and aortic arch anomalies. These resulted in congestive failure but subsequent progressive dilatation of both great arteries and development of aortic regurgitation were associated with eventual cardiorespiratory failure and death at 11....

Congenital contractural arachnodactyly.

arachnodactyly for this condition(l). CCA is characterized by multiple contractures, dolichostenomelia, scoliosis, arachnodactyly and external ear anomalies(l,2). This new syndrome contrasted with Marfan by the absence of eye and heart anomalies(l,2) but with increasing number of cases reported, occasional association of cardiovascular and ocular anomalies have been reported in patients with CC...

Congenital contractural arachnodactyly (Beals syndrome)

Congenital contractural arachnodactyly (Beals syndrome) is an autosomal dominantly inherited connective tissue disorder characterized by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. It is caused by a mutation in FBN2 gene on chromosome 5q23. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint co...

Novel dental anomalies associated with congenital contractural arachnodactyly: a case report.

Congenital contractural arachnodactyly (CCA) is an inherited disorder of connective tissue similar to Marfan's syndrome. The craniofacial and oral features of a young girl with CCA are described. The patient has the typical features of CCA as well as some additional dental anomalies which have not previously been reported with this syndrome. These include banded pitted enamel hypoplasia and hyp...

Contractural arachnodactyly versus Marfan's syndrome.