Ayers Kathryn Marina Sherwood, Drummond Bernadette Kathleen
Department of Oral Sciences, Faculty of Dentistry, University of Otago, Dunedin, New Zealand.
Pediatr Dent. 2003 Sep-Oct;25(5):501-4.
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.
先天性挛缩性蜘蛛指（CCA）是一种与马凡综合征类似的遗传性结缔组织疾病。本文描述了一名患CCA的年轻女孩的颅面及口腔特征。该患者具有CCA的典型特征，同时还伴有一些此前未见该综合征报道的额外牙齿异常。这些异常包括带状凹陷性釉质发育不全和矿化不足、细长的锥形根，以及伴有多个牙髓石的牙髓腔闭塞。牙医必须了解患者综合征的临床特征，以确定是否对牙科治疗有影响，例如是否需要预防性使用抗生素。将马凡综合征作为CCA的鉴别诊断予以排除很重要，因为前者有更多相关并发症且预后较差。
