Novel dental anomalies associated with congenital contractural arachnodactyly: a case report.Pediatr Dent. 2003 Sep-Oct; 25(5):501-4.PD
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 hypomineralization, long, spindly tapered roots, and pulp canal obliteration with multiple pulp stones. Dentists must be aware of the clinical features of a patient's syndrome to determine whether there are implications for dental treatment such as a need for antibiotic prophylaxis. It is important to exclude Marfan's syndrome as a differential diagnosis for CCA because the former has more associated complications and a less favorable prognosis.